Scheme Rheumatoid arthritis .... Sero-negative arthropathy Juvenile idiopathic arthritis... ese eseee ccccccesesees 24 Vasculitis ...... Systemic SclerOsis..........:::sccsscscesseesseeetssnseessseeesssevaeennen 50 Osteoarthritis Polymyositis ..... Familial Mediterranean Fever .. Sarcoidosis i CCamSeanner ss teesRheumatology CHEME Mowafy medical symphony #7 ( of any rheumatic disease ) It's a connective tissue disorder characterized by articular & extra-articular | manifestations, most probably autoimmune. (of any rheumatic disease ) > Still questionable. > But, most probably autoimmune. > Genetic factors may play a role (+ve family history & HLA association). > Environmental factors play some role. | > In SLE, add: © Hormonal factor also may play an important role. - ere ca oe ©_Dmug induced lupus : Hydralazine, INH, penicillamine, phenytoi ( of any rhewmatic disease ) -9>3 _© . . ~ . a ( ceptions out, Polyarteritis nodosa, Ankylosing spondyloitis ... ) Ritey— gout > Rheumatoid Arthritis: 9:3 > SLE :b=9: © I. General manifestations, IL. Articular manifestations. | MI. Extra-articular manifestations. LGenieral manifestations; = & > Fever, > Fatigue. > Weight loss. \CamSeanner + tpsMowafy medical symphony 2 2 I Articular manifestations: & (3D) > Distribution . > Description . > Deformity istribution:] t 1. Number of the affected Joints : mono or poly arthritis e.g. © RA, SLE, chronic gout © polyarthropathy. © Acute gout, septic arthritis & monoarthropathy. © RA may be monoarthropathy in typical form. | 2. Symmetrical or asymmetrical : Allare asy! mmetrical except Rheumatoid Arthritis & SLE — both are symmetrical. 3- Small Joints or large Joints: RA & SLED small Joints. 4- Peripheral (upper & lower limbs) or central (vertebral column & sacroiliac) : RA & SLE : peripheral > central 5-Erosive or nonerosive: = * No * Erosive : erode & destroy the cartilage e.g, RA, chronic gout, septic ive : inflammation of synovial membrane only. arthritis. £ Description:| v 1- Hotness 2- redness 3- Tenderness 4- Swollen 5- Limitation of movement. [Deformity:] If the lesion is erosive only. e.g.: Rheumatoid arthritis > © (see later) CCamSeanner se tees“Mowafy medical symphony 2 2 Rheumatology ‘The same in any Rheumatologic disease, as regard to skin & Renal manifestations. [i- Skin manifestations} © RA: Palmer erythema, SC nodule. - Yascuti bis wiley - Paltoj- S SLE: 6 (see later) 6 Scleroderma : edema, Skin induraion then pigmentation. $ Inall diseases : pallor, vasculitis , Raynaud's phenomenon. 2- Renal manifestations; SRA: Amyloidosis. © SLE: Glomerulonephritis ( Lupus nephritis ) $ Scleroderma : Scleroderma renal crisis (6c later) O-> ayritis muelans Ce fosive) 6~ B. Cardiac manifestation: § Remateid ayt hyiths © Pericarditis. @ Ps ovratic ath itis Myocarditis. [3 Scuty ofthitis 6 Endocarditis (?) a Severe aestvuctive Frama : : SS septic arthyitts Systemic Hypertension © Ischemic heart diseases. A Chest manifestations: © Pleurisy. © Interstitial pulmonary fibrosis. Pulmonary infarction occusun of artery 6 fon, 2 VOSCULT 1 Pulmonary Hypertension < eer > $ Caplan's syndrome in RA. _ © ARDS in SLE. —_ _—— CCamSeanner se taps!Mowafy medical symphony 4 # G= CNS manifestations] © Psychosis. © Depression, & Chorea, © Epilepsy. 4 4 Neuropathy. & Myopathy. | S Cerebral vasculitis + stroke. In RA, add_: Carpal tunnel syndrome & paraplegia. 6 Eye manifestations:| © Conjunctivitis, © Scleritis. © Anterior uveitis. (NOT in rheumatoid arthritis) MCQ (7- GIT manifestations: © Nausea & Vomiting Esophagitis, % Abdominal pain due to vasculitis, % Gastritis & peptic ulcer © Pancreatitis . © Hepatosplenomegaly. © Dysphagia, malabsorption syndrome & constipation in scleroderma. b Blood manifestations: < Anemia : > Normocytic normochromic ; autoimmune hemolytic anemia . > Microcytic hypochromic : o Anemia of chronic disease. © Iron deficiency anemia : due to chronic blood loss. CCamSeanner se tessaRheumatology Mowafy medical symphony & F & Osteoporosis. 4 Inerosive diseases: > Narrow joint space . > Deformity. ( mention) 6 x-ray chest: Pleural effusion. 2- Aspiration of synovial fluid : Antibodies. [Disease ~—||WBCs_/' mm? Crystals / polarized light microscopy Normal < 200 ve {Non- inflammatory “< 2000 ae (/ Rheumatoid arthritis 5000 - 50000 ve AGout 5000 - 50000 © Needle shaped crystals | of Na urate. © -ve birefringent. //®seudogout 5000 - 50000 Rhomboid, +ve birefringent crystals of Ca pyrophosphate. {Septic arthritis > 50000 ~ve eet i pa oUDOes we 3- Blood picture : © Anemia: 11 RBCs : normocytic or microcytic. NSAID Ef sis 4 blood loss o WBCs: 1 except in SLE & Felty's syndrome. MCQ © ESR: 1 vrarictd wevease (TB-mal9- tumey-Calagen Atsease) Felied © CRP: 1 except in SLE. (CRP may tt in SLE with infection ) -' “ptemetun op of © SGOT & SGPT: this is more likely to be drug induced rather than arena ne I i fumes of the Se a celts oF cRP-9enetic nonfgenegin CT Amie. ‘camseannerMowafy medical symphony #2 A Serological tests:| » Non specific Antibodies: & (may be +ve in all rheumatologic diseases , inflammation or even in normal Population) I- Rheumatoid Factor (RF): o +ve in 70% of RA © 4ve in 25% of SLE & scleroderma. © Highest incidence of rheumatoid factor is found in : Sjégren's syndrome. ( 90% ) MCQ U- Lupus Erythematosis (LE): eu YP whch eng we mmune © +ve in 80% of SLE. Cornflex. © +ve in 20% of RA. IIl- Antinuclear antibody (ANA): © vein 95-100% of SLE ( most sensitive ) © +ve in 90% of scleroderma. © +ve in 30% of RA. » Specific Antibodies : * Anti CCP (Cyclic Citrullinated Peptide, NOT ContraCeptive Pills ) & Rheumatoid arthritis. © ° Anti double stranded DNA( dsDNA) © SLE. (80%) 5 Peavpte sug ¢ Anti-sm (Anti-smith Ab, NOT anti smooth muscle Ab) > specific to SLE. ¢ Anti Ro AntiLa © specific to SLE , Sjogren's syndrome. 4 4 C% Anti histone antibody: © drug induced SLE e.g. hydralazine. 2 p/H ¢ SCL 70 antibody > Scleroderma. ¢ Anti- centromere > CREST syndrome. sPecific j e Anti-RNP (ribonucleoprotein) : associated with mixed connective tissue disease. _ ¢ cANCA ( Anti-Neutrophil Cytoplasmic Ab ) > Wegener granulomatosis ( 90% ) > Complement : Low complement levels indicate consumption as in active SLE, “6< CCamSeanner se tees—_— Rheumatol ‘Mowafy medical symphony # # — zs DD of Polyarthropathy. See later See later 2s. DD of monoarthropathy. I. General : Rest, Physiotherapy. II. Medical : = Cortisone. ws NSAIDs . v3 DMARDs : Gold. Penicillamine v= Immunosuppressive drugs : (methotrexate , cyclophosphamide ) II. Treatment of complications : e.g. © End stage kidney damage in a cases of SLE : dialysis or kidney transplant. o Renal crisis in a cases of scleroderma : ACE inhibitor is the drug of choice " Life saving " “SPECIFIC IMEANS dood 4ue Fest SEnstVY ~ 9604 -Ve Yesult — F Ar seage. of Sh Ing -Te€Xchude The best advice I ever heard is, don’t take anyone else's advice CCamSeanner se taps!be Ap UaUenIa ES Definition J ' ir i acterized _ by articular & ext = It's a chronic systemic inflammatory disease chai Dy articular & exty une, articular_manifestations, most probably autoimmi RA isthe most common form of inflammatory arthritis. (1-3% of the population) is the most common ———$<$<$<$— Still questionable, but many theories are suggested : ~ Autoimmunity : Infiltration of synovial membrane with lymphocytes. meo ~ Genetic factor may play a role : +ve family history, HLA association ( HLADr4 ) ~ Environmental factor : e.g. infection mostly viruses. EBv-affPical, bacterig, zt Sabu event is unknown (vital) StimalareT: COS» Ly mp fe Knes (315 - ‘athology ; FI O-TWE Jo stimnake B- TCLS, B CLUS dis EQUYCLIE - F Articular :mmwnl system ye ease 79> IM mune ComPle Sen Gul Fed by Pt Hg MWh 19Sc24mes Wich inhale syreuts '- Synovitis: Thick, inflamed & infiltrated with Iymphocytes & plasma cells. ; au - ‘cartilage. 72 “M9Teqne5 "3 2. Pannus formation: erode & destroy the articular cartilage. annus formation: 3. Cartilage is eroded & destroyed > sublaxation & deformity.e/ede bone First > RV GY HCey ase va) So, disease like HIV, in which T cells are decreased, will improve Pre-existing RA. This is the reason pee why RA is very rare in patients with HIV. II- Extra-articular ; The predominant infiltrating cell is T Iymphocyte central joints 4-Affects mainly small Joints e.g: hands, feet (proximal interphalangeal, — er metacarpophalangeal & wrist Joints). N.B: The distal interphalangeal Joints are usually spared. e .cePF IZA 5- The arthropathy is erosive ¢ deformity. Description; v 1-Redness 2- Hotness 3- Tenderness 4- Swollen _5- Limitation of movement. Deformity; v 1-Hand deformity: i |- Boutonniere deformity: extended distal & flexed proximal interphalangeal Joint. rl wy 2. Swan-neck: flexed distal & extended proximal interphalangeal Joint. @ ~ ocsefad LITHE Finger halangeal & extended interphalangeal 3- Ulnar deviation of metacarpophalangeal Joint, dewaret 4- Z-shaped thumb: flexed metacarpo-pl joints. — 5- Carpal tunnel syndrome. 6- Telescope deformity: due to substantial absorption of metacarpal and Phalangeal bone ends. Involved fingers may be extended or shortened as the . telescope, CCamSeanner ss tees!f voice. i d: stric wr & hoarseness ol voice. . 2 Ceeonronei Soe mn: Spinal cord compression > paraplegia, 3- Atlanto-axial subluxati |- Tendon rupture: (extensor more than flexor) e.g.: flexion of hip & knee. ( ) 4- Tendo hi fl f hi it st). 5- Synovial membrane rupture : Baker cyst ( popliteal cyst ) r Synovis. membrane rupture : Baker cyst ( poplbea’ cyst i St Tn Oe fe mmiocm indismetey D's!Mtbution tendon ¢ heathey exons surf toid nodules” “**2l3 over peny PYominants Lmeiacey Rigdon 0 S.C. rheumatoi 5 : olec4e non ae ) © Palmer erythema. 20% op Pr. RE ave - active Alsease © Vasculitis.» wicers © Pallors Auots anemta © Amyloidosis. ] © Glomerulonephritis: drug induced ©8.: NSAIDs, Gold & Penicillamine. | © Pericarditis (30%), © Myocarditis. - engocariitt’s Cirmnge) YAR occurs due to vasculitis, Causes of edema LL in RA: * Nephrotic syndrome (due to amyloidosis or drug induced GN), * Pulmonary Hypertension or IPF. cor pulmonale * Myocarditis He, © Systemic Hypertension, © Ischemic heart disease, . HTN ic Lvascuts in coPmary ortieras © Pleurisy & pleural effusion, 2 Rheumatoid pleural effusion : © Interstitial Pulmonary Fibrosis, © Pleural fluid glucose < 30 mg% . © Cholesterol crystals, Memmerages Polmonary Infarction. arty ec Secondary ene canine © Pulmonary Hypertension © vet : ‘ IMerentiret PP shyPatig >PH lO o Caplan's syndrome : theumatoid Nodules with Pneumoconiosis, ( Tare)... RA Methotrexate =. Pneumonitis, - CamScanner + Lise i sual!In Capsule Series mp . o Carpal tunnel syndrome: compression of the median nerve due to hypertrophied synovium, it may be the first symptom of R.A o Atlanto-axial subluxation > spinal cord compression > paraplegia. © Psychosis. o Depression. 7 vosculltt’s o tnfarcten o Neuropathy. © Myopathy. © Chorea. 0 Epilepsy. © Cerebral vasculitis > stroke . © Conjunctivitis 0 Scleritis © repeated attack @scleromalacia ( blue sclera ). © Iritis is not common. o Nausea & vomiting.» Sf te dYug (wsAtO) o Abdominal Pain due to vasculitis. © Hepatosplenomegaly. o Pancreatitis. © Gastritis & peptic ulcer. {vili- Blood} © Anemia : macvecgt > Normocytic normochromic anemia. (Autoimmune hemolytic anemia). ic anemic» Meitst Yexete > Microcytic hypochromic anemia (Iron deficiency, anemia of chronic disease ) - RENAL oP Pectien + Vatrodec CSE oF dyuQs LT ea ae heumatoid arthritis - <6 weeks - Chronic disease > 6 weeks ~ erosive CCamSeanner si eslJust scheme I-X-ray : a Jone of the X-ray finding is diagnost © None of the } ing i © Osteoporosis. Narrow Joint space. Deformity (mention) f Jar cartilage and bone erosions develop after months of SUstg Loss of articu ° activity. X-ray chest: Pleural effusion. 2- Aspiration of synovial fluid : Antibodies 3- Blood: ° ~ 4. RBCs: Anemia (normochromic — hypochromic) ~ WBCs: 11 ( LL in felty’s syndrome ) ~ ESR: 117 may > 100 in severe cases = CRP: 14 4- Serological tests: “RF: +ve in 70% of cases, p RECent Se Ye “LE: vein 20% ofcases, | CPST) For Cari de} NoF RA Nomentin) 32.7% NOT contraceptive pills). © RA tanti mov ( ~ ANA : +ve in 209% Senstive 7 AStIV #2 % of cases, fect. Recently Anti er. _steotte: Recently :AntiCcp (Onli citrullinated pe 7 Jeg metheds: Rose Uy eur ( P Ws an Ign Rains chang, pide ~Hemay be +e in : CCamSeanner so eslPatients can b 2- Arthritis of 3 or more joints. 3- Arthritis of hand joints. 4- Symmetrical arthritis. Criteria 1, 2, 3 & 4 must be present for at least 6 weeks 5- SC Rheumatoid nodules. ¢ said to have RA sil at least 4 of the following 7 criteria : 6- Characteristic x-ray finding e.g. erosions. 7- +ve Rheumatoid factor. [2010 ACR/EULAR Classification Criteria for Rheumatoid Arthri \ re > Definite RA= 26 > Patients with a score <6 should be assessed over time [ Score Joint involvement : 0-5 (Any swollen or tender joint excluding DIP, 1° MTP, 1°CMC) © _Llarge joint (shoulders, elbows, hips, knees, ankles) 0 © 2-10largejoints 1 01-3 small joints e.g. Mcp, Pip, MTP 2-5, thumb IP, wrist (large joints not counted) 2 © 4-10 small joints (large joints not counted) 3 © >10 joints (at least one small joint) e.g. temporomandibular, sternoclavicular. & Serology : 0-3 © Negative RF AND negative ACPA 0 © Low positive RF OR low positive ACCP 2 © High positive RF OR high positive ACCP (> 3 times the upper limit of normal) 3 Acute phase reactants : 0-1 © Normal CRP AND normal ESR 0 © Abnormal CRP OR abnormal ESR 1 Symptom duration : O-1 CCamSeanner se tealTRA onal faa i: sis} Factors associated with poorer progno: ° Male Patients © Insidious polyarticular onset * Extra articular manifestations . Functional disability at one year after start of the disease ¢ High value of RF © Presence of HLADR4 ¢ Radiographic evidence of erosions within 3 years of onset of disease. The cornerstone strategy for management of RA is induction of remission early before structural damage. (A) General treatment | 1. Rest : © Complete bed rest is advisable in active disease, © Articular rest by the use of splints will hel P to suppress synovitis in isolated joint inflammation, Us CCamSeanner se taps!echt Rheumatology [NSAIDs : for relieve of pain & inflammation (ymptom-modifing) irin 500 me/ th has no Ful@in Rathogenst’s -Can't Prevent eyes!h ~ ss ia defarmily - destruction © Diclofenac 50mg t.d.s. © Paracetamol 500mg t.ds. o Indomethacin 50 mg t.d.s. o Ketoprufen 100mg t.d.s. © Celecoxib (Celebrex ), Lornoxicam (Xefo ) : COX-2 inhibitors. Mechanism : cyclooxygenase inhibitor (COX-1 & COX-2 inhibitors ) | Side effects : mnemonic : ASPRIN o Aplastic anemia. o Salt & water retention leading to edema & worsening of hypertension & heart failure. o Peptic ulcer : specially with COX-1 inhibitors. o Rash (I mean skin rash ) o Induce bronchial asthma. © o Nephrotoxicity : Interstitial nephritis and rarely acute renal failure. Precautions : © Never exceed therapeutic doses & Never combine more than one NSAID. © Avoid long term use if possible. 2 Cortisone : 4yit ) -Co te Prevent oStee Plosis-osteemalegia ~ Small dose of cortisone may be given (7.5 mg/d). H0F ne ule (narticlley” mane ~ Systemic steroids uncommonly needed nowadays. PM use th ext Ye ot bi cular ~ May be used in high dose in treatment of extra-articular manifestations, SE of cortisone : see Endocrinology book page 62 CCamSeanner se taps!= Group of drugs used in system activity & progression. i |< 's to appear ), ‘These drugs are slow acting drugs (their effect takes 4-8 weeks ). Id be used as early as possible to induce remission & avoid erosive changes, ” = Should be us — Mechanism of action : exact mechanism is a. Inhibition of lysosomal enzymes, phagocytosis & prostaglandins. unknown b. Inhibitory effects on the immune system. ¢. Some has antimicrobial activity (e.g. sulfasalazine ) i £ (Na aurothiomalate ) * Dose : 50 mg/week IM for about 20 weeks ( Total dose 1000 mg ) 1 SE: DHP @ FV inhuven © Depression of bone marrow, Dermatitis, © Hypersensitivity. © Proteinuria, * Precaution : © Stop if proteinuria > 2g /24h ° Stop if WBCs < 3000 or platelets < 100000 i ee * Dose : 250 me/d * Response : within 4-6 months. * SE: As Gold + SLE. ‘i oe least SE _ butit weak " Dose : 259 mg/d . Response : Within 4.6 months, E *® Retinopathy,Mode of action : antifolate. » Dose: 0.5 - 1 gm/d then increase to 2-3 gm/d over few weeks. + Side effects : headache, skin rash, GIT irritation, leucopenia, reversible depression of sperm count. om we folic at rcareplom send 7 2 methotYaxare ] Series In “Aware. (40- 2) ig with rather rapid on’ nce 20me/d. rarely bone marrow failure. set of action. oo Anew effective dru © Dose: 100me/d for 3 days 1 hepatotoxicity & oS hen maintenat diarthea, skin rash, iii. 0 Dos o S/E : nausea, ach 2-2.5 mg/kg/d. to re - start with Img/kg/d then increase * na nged use increases the risk of leucopenia, hepatotoxicity. Prolo lymphoma. BIOLOGIC DISEASE-MODIFYING AGENTS Tumor necrosis factor a (TNF «) blockers : Infliximab, Adalimumab These agents are used in severe active rheumatoid arthritis when ° standard disease- modifying therapy has failed ( usually given in combination with methotrexate ) ° These drugs block the action of cytokines ( chemical mediators ) ° The onset of action is rapid compared with traditional disease — modifying drugs (2 weeks ), © S/E : Expensive, Infections e.g. TB Interleukin-1 blocker (anakinra ) © It blocks the biologic activity of IL-1 inch i ‘ , Ceradation associated with rheumatoid arthriti is. (ines rear age ARREST ion of cortisone ] intra articular in jection of | cortisone || : ] Indication: CCamSeanner soe tesa!In Capsule Series Rheumatology MARIANTS OF RHEUMATOID ARTHRITIS 1. Sjdgren's syndrome : ©. It is classified as primary when it occurs in the absence of another connective tissue disease, and as secondary when found in association with another connective tissue disease such as RA, SLE, MCTD. © There is an association with HLA-B8, HLA-DR3 Clinical picture : © Arthritis : usually non erosive polyarthritis, ° © Dry eye (xerophthalmia), dry mouth (xerostomia), dry skin & dry vagina, ° Lymphadenopathy: there is increased incidence of lymphoma. ¢ yon hod") © Renal tubular acidosis & Raynaud's phenomenon. S Associated autoimmune discases : myasthenia gravis, autoimmune hepatitis, thyroid diseases. (ymPhemma © Investigations : © Highest incidence of rheumatoid factor is found in : Sjogren's syndrome. ( 90% ) McQ Anti Ro, anti La antibodies. ( Anti Ro is of particular interest because it can cross the placenta and cause congenital heart block ). © +veANA — -schifmer test 5 NoY May Loml 49 ML o Treatment : “SWUVALY gland blo Pasy—yT |ymphecpfes Hydroxychloroquine, low dose cortisone or methotrexate. Xerophthalmia : treated by artificial teardrops & corneal protection by soft contact lenses. © Xerostomia : may be managed by parasympathomimetic. Dry vagina is treated with lubricants e.g, K-Y jelly. IL Felty’s syndrome ; Spe! Yhenaiol © Arthritis (Like RA). Severe © Splenomegaly with hypersplenism. ( No hepatomegaly ) © Pancytopenia. (neat ¥ Pena) mea | © Cortisone isa drug of choice. ‘camSeanner7 Sero -ve spondylopathy) General features: 8 features «factor: negative (the reason for the name). 0 Rheumatoi arthritis. Central Gpondylits and /or sacroilits) > peripheral 2 A 6 Asymmetrical peripheral arthritis. co o HLA-B27 (EXCEPT in Behcet's syndrome ) 0. tve family history. ‘ o Redeye. o Erythema. ~ © Enthesitis (inflammation at sites of tendon insertion). 1- Reactive arthritis : © Itis a sterile arthritis which occurs following an infection. me a cBcs nel 0 Causati rca =feh ‘ SAS * & Chlamydia trachomatis ; urogenital infection. | ~ Fe aes. : £ Salmonella, Shigella, Yersinia: GIT infection (bacillary dysentery) © Reactive arthritis may be just arthritis, but sometimes the clinical triad of Reiter's disease may occur, Reiter's syndrome: 3'> 9 (10: 1) 20-40 Itis a form of reactive arthritis characterized by a L. Arthritis jnvestigetians & ime yic ne a jocures!S sme organisms : Clinical iad of: + Asymmetrical, oligoarticular, usuall (knee, ankles, fet). Sacroilitis & spondylitis 'y involves big joints of LL may occur, ~ 2 Conjunctivitis (ed eye), “EM thesitis of LLLTEnben a chiligc - 3. Urethritis, cystitis mt see, can't pee, on climb the tree ) ae ne lantey Fas citis T :AGCIT:. asc ‘reatment : cs ofa Ulery dlarheg, é = Balamtis +5 _ © Nonpharmacologca The sme NMa PAIMMESS Ubee pop Sens Penk Pharmacological : kelatedeyma ‘ lennery ‘ Me ther 4) 5 * Antibiotics for inf. SKin Wong n panes ii, fections, $s: * NSAIDs for arthritis, CCamSeanner se tealIn Capsule Series Rfieumatology Gere disease! BDO o Ihis o systemic vasculitis of unknown etiology which can involve large, medium and small arteries and veins. ° ‘There is an association with HLA-BS -HLA BIZ o Clinical picture : highly variable with recurrence and remissions > Diagnostic eriteria: eg ssentich aad Far diagnasi 5 yX AM Recurrent painful oral ulcer (100%) : Initial criteria for diagnosis require ©2*~ tne occurrence of at least 3 episodes of painful oral aphthous ulcers/year. To confirm the diagnosis, at least two of the following must also be demonstrated: J +2 of the Following i. Recurrent painful genital ulcers that heal with scarring. (80%) ii, Recurrent anterior uveitis or retinal vasculitis (60%) : may lead to blindness. iii, Skin lesions (60%) : including erythema nodosum or acneiform lesions. iv. Positive pathergy skin test : defined as the formation of a sterile erythematous papule 2 mm in diameter ot larger that appears 48 hours following a skin prick with a sharp sterile needle. ee vnoniive asym ica tise pathy, involves a | na-trosive, asymmetrical oligoarthropathy, {ze Vasculopathy : ~ Unlike most forms of vasculitis, both arteries and veins, of all sizes, are. involved. ~ Venous thrombosis, Budd-chiari syndrome, IVC & SVC clot, ~ Aneurysms of pulmonary arteries may lead to massive lung hemorrhage (3) GI: Ureerations may occur anywhere in the GIT. The terminal ileum and cecum are common sites. It may be difficult to distinguish fom inflammatory bowel disease (such as Crohn’s disease). ey CNS : aseptic meningitis, strokes, cerebellar ataxia, cranial nerve palsi CCamSeanner se tealIn Capsule Series ©. Investigations : Non speci fic, reflect inflammatory state (t ESR, CRP) Sys beets Ms © Treatment : symptomatic treatment Ceeeede inva Wvemene z= NSAIDs for arthritis and thrombophlebitis. / vs. Systemic anticoagulant is often used in patients with thrombosis. v= Mouth ulcers : oral washes, topical steroids. vs, Genital ulcers : topical steroids, colchicine. zs Anterior uveitis : systemic steroids, colchicine, cyclosporine. % Anti-TNF therapy in severe cases. = Ankylosing spondylitis] of UW0123)) v Leal ~ It is a chronic systemic inflammatory disorder of the axial skeleton. The inflammatory process frequently results in gradual fibrous & bony ankylosis. ammatory process Gradhal Ubrous & bony ankylosis. ~ 0.1% of population. ~S:2=3:1 @-HLA B27 in 4% of Ptns associated with Postatt & eae i ~ Age: <40 years (Onset after age of 40 is uncommon) 6'%2%o) * ocular ated! ~ Clinical picture: 74 ynaetemte Arthritis : mainly Sacroiliac joints and the spines, tyoeer oy Back pain} with prolonged moming stiffnes ~ movement a i i Vest ind exercise. The earlies| most This stiffness improves with ic retain” Sacroiliac joints tenderness & decreased spinal motility, ~ —Siked spinal : oprecran ” Sometimes reduced chest expansion due to costovert ° " i tebral joint involvement. Peripheral joints (30%), ., + Patho gensis ink lamarion o¢ CYC + "Anterior wits, (25%, Tergegmr| UBemenh af sine ofattech ment Accs endl | CORE itis) acemlation of Aortic regurage (10%), inClamaterg cols then ostoePenta. LLe CVS ¢ CIS + Amen sinaton-cutti] MEP SHon shen essen Ren © Amyloidosis, “19. nePhlePotiyg ~Oieteas @ ky PhoSt's- Yor dosts j RES © Apical pulmonary fibrosis in Clecse occiPut Fowait distal LL» Chonicpraatta oe Plumber of pectin :- (4 Latdess) -m. ~IBD Hb schroebey test Cdec(enso of Ford ofa lO RYun of (umn ers Pl ‘CamSeanner se tees!- -Patteren oF artiitt's in Ents a ae Pet axthvitis,. BB 1- PeriPhevat artaritis (jarge faint-assym merical | : yifendsuitis Wy Pertrepyic estocaigh Pat! MeCVESIS BF bono see to IMecocortcaid Hr ok b oo Xray: wel Inf fammasin © Sacro-iliae joints: Bilateral symmetrical erosions, irregular joint ons aE the forur\ taste ddint margins. (The cartest finding) PSudo uidenind o& the J (ar) Lad ‘seltfosis © Spine erosions, calification ofthe spinal ligaments (Bamboo spine). Lab; Falld~“S4uate shaPed vertebrea due to efesbns cmd eclersis & : © Fits corneys © HLA-B27 : in 95% oF cases. (not used for the dag FCatien of anuhug © RE: -ve OESR,CRP: 1 Fibyesis (bids Ing © Alkaline phosphatase oa Syndesms Phytes) Treatment : The same as rheumatoid arthritis, (2\o SIF Cetin of Long-Lyeaments by BambeosPingayOagg ey 4 Enteropathic arthritis: (coltic arthritis) ° Arthritis with inflammatory bowel disease (ulcerative colitis & Crohn's disease). © The patients sometimes develop characteristic skin lesions - pyoderma gangerenosum & erythema nodosum. 1 skin Lesion S* - 5- Psoriasis arthritis: 22) he 1) wei demercted Rie eV 4thamtous Plats ~ Patterns of psoriatic arthritis: (5\ ee ~ Arthritis occurs in about 8% of patient Wales pitting . Mei (ilo Asymmetrical oligoarthitis (The most common, > 50%). scolar pay) SAY Bo Distal intephalangeal joints involvement. (5-10% ), | Rae Ketebesis IC Bo Sacroititis & spondylitis (20-40% y acter eee 5 ©. Polyarthrtis" sheumatoid like ": (15-25% ) By oe Conganriits-l¥th/s ~ Desiyvehve atrh ntl < D cus:-aaric ~ Treatment : Like RA but the role of anti-malarial : isswbueney 1s controversial as they may lung, 7 induce exacerbation of psoriasis, | 21 M!"9:- apical Fibrosis Mews pry. Cuada epuing 6- Wipple syndrome: more ing 2% Radi (yr loating SY ndesmoPhytes HEE Pincel and cup apPeotance nip aint oM: Malabsorption syndrome. (3) eSteo Isis - Pefiostisis OA: Arthritis, ON: Neuropathy & lymphadenopathy. ‘CamSeanner se tesajuvenile rheumatoid anther: -d " juvenile chronic arthritis (JCA ), juvent hi : 4 previously term Seriteria must be met to diagnose JIA : i s. > Persistent arthritis of more than 3 month: £16. > Onset : under the age o scans © Exclusion of other diseases that may cause arthritis e.g. septic Classification : The 3 major types of JIA are : 1- Oligoarticular JIA 2- Polyarticular SIA | 3- Systemic onset (Still's disease) © Usually involves the large joi © There are 2 types - exam every 3 months is Tecommended, 5) Late childhood onset : Usually older bo QE. 30% of 14 © Affects 5 or Mote joints, ° Symmetrical pol joi ) Tge joints, ° aa type of J14 is more common jn Sitls than in boys, © Children with Polyatticular JIA are also at tig YS with Sactoiliitis & HLA-B27 ‘arthritis Usually of Small joints s lay Weitis and Should alsg be monit K for developing chronic red by an ophth sé ‘ ‘CamScanners Wee tgaIn Capsule Series Rheumatology 3. ( ease ) o A disease of under-fives but adult cases may occur. o Fever (high with spikes up to 40°C daily), skin rash, Splenomegaly. o RF: usually -ve. o Late arthropathy. So, misdiagnosed as hematological disorder. fi Oligoarticular Polyarticular Systemic (Still disease) 1 Frequency (7%) 60 30 10 Number of joints 4or less 5 or more Variable Female : Male ratio 5:1 3:1 1:1 | Extra-articular Rare Moderate Prominent Uveitis (% ) 20 £ 5 Rare ‘Rheumatoid factor — Rare 15% Rare. “ANA 85% 40% 10% {Prognosis Good Moderate Moderate. 4 ote fuinht hut World War lV ‘camseannerIn Capsule Series __ ery aT tT TAR ot) ul cacao Definition: ‘a-articular & artic, It's a systemic connective tissue disorder characterized by extr: cul manifestations, most probably autoimmune. ——_ eon Etiology; Is still questionable , most probably autoimmune : defect in T suppressor lymphocyte sloxes which ea ib i ¢ complexes which can involve tissue anywhere in the > 1 autoantibody > immune co plexe: — body > systemic manifestations.. Fiure

Vasccustis edema aS dilanevab- Sqmimetriced its sGeRa Ve PhesPheutic Y Jotpeass Renal |: (Lupus nephritis ) Details: see nephroloet heh Neer Aimiene ComPlbKON CBMs v FL¥ lation nephritic enal disease is common. £ inp, Sus = N 7 ; Febues aaa " ve ST um) itefarean voninet? i! ee ote that drug induced lupus tarely atfects the kidneys. uve abep hn Patients with lupus nephritis generally have abnormalities on urinalysis: Proteinuria, either asymptomatic up to nephrotic range. Hematuria and casts may be seen. -Red Ces Casts oedema ATW end stJe Renad Fils@L 6 % of pins Acute or chronic renal failure may develop. Needless to say that patients may suffer from other symptoms of lupus unrelated to kidney function e.g. arthralgia, fever, fatigue, psychosis, i i i i i has clinical or Renal biopsy should be considered in any Patient with SLE who has c laboratory evidence of active nephritis. ss CCamSeanner se tapsi HRITIS * In Capsule Series RON OF Lupus ee | ee | mpiomatic proteinuria or hematuria: affection o This form typically responds completly i Glass II: Mesangial . treatment with corticosteroids. GN Ye Offer responds to treatment with high dogs [ Glass Ii: Focal proliferative Class Il: Focal prolifer i ween glumerili ae apFected. corticoster @ This form is mainly treated With + Diffuse proliferative GN fe err are affected corticosteroids & — immunosuppressive drugs. Class V : Membranous GN o Characterized by extreme edema ang protein loss ( nephrotic syndrome ) Class VI: Sclerosing GN co Significant renal insufficiency or end stage! renal disease in most cases. © Unlikely to respond to medical therapy, draltsis—ortyay i = sa % Pericarditis, ™CO Most imPs Vhanf- & Myocarditis > HR. Satks : oe oe ; NeYeal Ondo j ibman endocarditis (non-infective endocarditis) > ety 5 oe i Systemic Hypertension, oo od © Pleurisy & Pleural effusion é Interstitial Pulmonary fibrog; i 6 Pulmonary infarctio ; 0 (Shrink; 6 Pulmonary hypette mnkin 6 1g lun, Nsion, : Syndrome pin (ios Preun CCamSeanner se tees!Sensory a 7 cufonan Headache. © Depression 6 eee eead Vs thy 2 Stead Calebst'éy | duets steroids 4 Cerebral vasculitis (lupus cerebritis) > stroke. vascullt!S Im PYeFrontal Gfee smut Pe infarction dementio - Nervous manifestations may be due to diffuse cerbritis, vasculopathy, thrombosis, infection or drug effects. Be] & Conjunctivitis. © Scleritis. © Anterior uveitis © Retinal vasculitis. ie err & Nausea & vomiting. S Painless oral ulcer. ¢ Esophagitis. S Gastritis & peptic ulcer. 4 Vasculitis : Abdominal Pain. me SentVic blsvs. é i Pancreatitis. J Gutoamuing 6 HSM. Viii- Blood:| 4 RBCs: Anemia of chronic disease, hemolytic anemia. & 4 Platelets & WBCs : may be due to the disease or a side effect of pharmacological treatment. elt Antiphospholipid antibody syndrome <> thrombo-embolism, CCamSeanner se taps!: 3. 3D In- Articular manifestations: i- Distribution : 1- Polyarthropathy. ee 2- Symmes 3- Affects main! ras 4- Affects Lat Cancer Et bloog, bra, st ), onl a ‘camSeannern Capsule Series Rheumatology (1) Blood, picture: 4 Anemia : anemia of chronic disease, hemolytic anemia. am Satanic Ciscase, hemolytic anemia. A Leukopenia (<4000/mm* ) = 4 Thrombocytopenia (<100000/mm? ) 2 Lymphopenia. Tavestigat 6 ESR: TT Scheme + & CRP: normal but f only with infection. ¢ Lymphopenia U&1C3 & Cy: Consumed. 2G e ci 4 (2) Serological tests : > Non specific Ab: ° LE cells: +ve in 80% of cases. co Rheumatoid factor (RF): +ve ia 1 25 % © Anti-nuclear Ab (ANA): +ve in 95-100% ( most sensitive ). Positive ANA not ee a diagnostic, but negative test argues strongly against SLE. Causes of +ve ANA: (+ve in 5% of normal population) EO o SLE (95%) © Rh. Arthritis (30%). 0 Scleroderma (90%). — gee ee ee © Sjogren's syndrome (60%). © Iry biliary cirrhosis. ‘Sjogren's syndrome (60%) -l oa © Infective endocarditis. © Old age. Semone > Specific antibodies : - Anti double stranded DNA (dsDNA ): in high titre is very specific to SLE. - Anti smith (Anti-SM ): indicates poor prognosis. an s/poor prognosis. = Anti Ro (SS-A), Anti La (SS-B) : found in ANA -ve lupus. > ay = DNS ve ups - Anti histone : specific to » drug | induced lupus. Anticardiolipin Ab : Antiphospholipid antibody syndrome. 8 @)-X ray : Osteoporosis. NB: -PYeP hun with sve anti SS UL get ababy cath Congent al conduct Heart diseese and Pacing in carly DER CPeae maker) (5)- Investigations for lupus nephritis : urine analysis & renal function tests. Chest X-ray: pleural effusion. (4)- Aspiration of synovial fluid : Ab CCamSeanner se taps!h. 1- Malar or butterfly ras) 2- Discoid rash. >i 3- Photosensitivity- |. Painless oral ulcer. / . ae i “ims ants: 2or more peripheral jOin's 5- Peripheral joints arthnitS ° “°° ————_ 6- Pleurisy or pericarditis 7- Proteinuria, 8 Psychosis or seizures. 9% Pancytopenia. 10- +ve ANA antibody, L1- #ye anti ds DNA ab or anism ab, the diagnosis of SLE is strongly suggested, when a person has 4 Or more | these criteria, 2012 SLICC crite ia for SI o SLICC; C: Systemic. Lupus International Collaboratin Cli 8 Clinics ° Classify a patent as having Sup if: Both oR © Patient sat ISfies f i I titers, « “munologic eri Metia Meludin, ce ~™"8 at least one clinical and a CCamSeanner se esl2- Chronic cutaneous lupus . tongue or X Oral ulcers absence of other causes, such as Behget. FE Nonscarring alopecia ‘ag ov hai fragility with visible broken absence of other causes stich as alopecia tron deficiency and androgenic alopecia Involving 6¥0 oF more joint ethan | day or pleural 6- Serositis, Pleurisy form effusions or pleural rub. Pericarditis, pericardial effusion or pericardial rub in the absence of other uch as infection, uremia. 7Renal 8 Neurologic - es, psychosis, mononeut | multiplex, myelitis, peripheral or cranial | | neuropathy. 9- Hemolytic anemia 10- Leukopenia (<4000/mm’) OR Lymphopenia (<1000/mm’) 11- Thrombocytopenia (<100,000/mm') At Icast once in the absence of other known causes such as Felty's, drugs .. \CamScanner seo tees[2 Anti-dsDNA 3. Anti- SM 4 Antiphospholipid antibody = Lupe 4 $F Low complement: Low C3, low C ie absence of hemo! lytic anemia 6. Direct Coombs test : In th yt [Treatment of SLE: 7 © There is no cure for SLE.( SLE is considered incurable, but highly treatal © Goal of treatment is to relieve symptoms by decreasing inflammation, ————— eS autoimmune activity. poeta * Depends on severity and organ involvement. 1 General prophylactic measures: a. Rest, . Sensitivity to light is treated by > protective clothing, sunglas sunscreens. : 4 s . Avoid drug inducing ‘SLE. a |. Psychological treatment, — a - PYodesteron PILIS omy : Severe Cages es Pulse dose: o “ate dose: 1000me/¢ ty sf Slomg the disease dis days, fo i appears ( {——’ “Mowed by Gomg/d till acti . Then lovey mae Symptoms @ ee a 01 ngoes ao Rteuumatology | A -_Hydroxychlorquine ‘Is sometimes used with cortisone (low dose) for skin manifestations & arthritis resistant to NSAIDs. §- Immunosuppressive drugs : for severe cases resistant to cortisone therapy Can use withi aPering of Colt(Ssone- (MAC): 0 Methotrexate. 0 Azathioprine. ‘ ed bY © Cyclophosphamide. S £.- cy sic hemeltegice eee Concer urinary bl qvi9 6- Plasmaphersis : in SLE patients with serious brain or kidney manifestations. 7 Treatment of complications: o Removal of spleen is sometimes done for thrombocytopenia. semoval of spleen. Carombocy opens o End stage kidney damage : dialysis or kidney transplant. = Pathe ensi's of Ant Phosthe Lifid SyndYome — Anti PhosPheLiPid anti bedl@s otteok PhasPholiPids of RBCs that leleare sub sctences which inhibit Protien C]s5 Chateral antics aJulant) wide SPYead Tlmbosis [2 Lupus anticesgulont (wiend term) awtibd} rich ectivere ‘Women tend to love men in their presence, while men tend to love women in their absence Prethramben that activate ¢1 brin-»wide sPread bhrombesis fal Decrease Pletlets bY ConSumPtyn and amipretiots antibody: CCamSeanner so tessa!() DRUG INDUCED LUPUS (DIL) - More common in 3) / a iiistory of drug intake : Hydralazine INH, Phenytion, Penicillami - History NH, Procainamide, « methyl dopa. hesent sux (© There is NO contraindication to using these drugs in idiopathic SLE. - Renal, CNS, skin features of SLE are rare in DIL. ~ Pathogenesis : Unknown, Anti-histone antibody, ANA are +ve, but Anti-DNA Abs are cae eee Investigations: absent. - Treatment : withdrawal of the drug (2) ANTIPHOSPHOLIPID SYNDROME ERHOSPHOLIPID SYNDROME Definition : Consumed nist LC awribeas a0! Recurrent attacks of vé teri rombosi cytop. in urrent attacks enous or arterial thrombosis’ and thrombo enia i association with laboratory finding of antibodies against phospholipids, Classification ; “Yecarrent abortisn, - 6 CcWWSton of wieYing _ se steroid are all that we need, & short course stero: * Secondary : occurs in association with autoimmune disease e.g. lupus 50 ‘lo cP: ~The Connective M5500 dscider ay “J- mall neney tinfech -tnfecha 4) Vascular thrombosis : AS Moke_tap muti "fa reruandemintio..chalen. " Cerebral infarction, less common, Coronary, retinal & visceral man endocarditis, -MI Limb ischemio * Migraine, F s © syndrome or dementia, > chorea, Seizures, Guill ic HVE bucataPanin 10. tas CCamSeanner se teesn apsule Series b Rheumatology Mechanisms of thrombosis in antiphospholipid syndrome : 1 Antiphosphoiid antibodies interfere with the function of pa -bindi Plonged dweto yPletets Coa uation Foatoys coagulation Profile: Si breeding tine H coagutation time Pyolonged cen dwme «) CBC: akon romboryopein, 1 1” Poteet: b) Serologic tests : © Anticcardiolipin antibodies (1gG, IgM) -LuPus 2nl ceaguontontals + Anti-B, glycoprotein I antibodies (IgG, 1gM ) Investigations : |, Laboratory : : for confirming a thrombotic event Il. Imaging studi © CT, MRI of the brain (cerebral stroke), chest (pulmonary embolism) abdomen ( Budd-Chiari syndrome ). a © Doppler ultrasound : for possible detection of DVT. « Two dimensional echocardiography : for possiple detection of endocarditis, valvular insufficiency. Treatment : 2» Treatment of thrombosis : ¢ TVorSC heparin followed by warfarin therapy. © During pregnancy : patients with a history of thrombosis receive therapeutic doses heparin during pregnancy, long term anticoagulation is then continued postpartum = 3 IV steroids. 3 Cyclophosphamide. v Plasmaphresis. treatment oF severe SLE |catastrephic APs hefavine stored Reccurrent aba(tion:. ASTIN,ed by tissue deposition Definition) Gout is a disease hyperurioemi mn of monosodium Ute eee which is characterizs Is due to hat results In = crystals du ° Gouty arthritis. © Tophi. © Gouty Nephropathy. - f inflammatory monoarthritis. x0 xO > xanthine — uric acid - Gout is the most common type 0 TGR - . Purines > IMP(inosine monophosphate] —> hypoxanthine > Production of uric acid (Metabolic gout). > 4 Excretion of uric acid (Renel Gout) (A): ? Production: © Iry : Enzyme defect :}4 HGPRT (hypoxanthine —Guanine— phosphoribosyl — Transeferase) © 2ry : leukemi: i ry emia, lymphoma, sarcoidosis, severe Psoriasis. (B): Ld Exeretion: © Iny: Idiopathic (Isolated t tubular defect o2ry: ee - Renal Failure, Dehydrat Syston >t Ren Drugs: ¢ EE 8. Diuret ties, Pyro SAS Pytazinamy ide,not irritant to joint & urate crystals can be found in asymptomatic vie acid in saints. but when urate crystals phagocytosed by macrophage > release of lysosomal — EE” release OF lysosOmEY mes which produce inflammatory reaction > acute gouty arthrit ee Teacuon) g enty" pronic gout : Deposition of uric acid in: pr Joint: chronic erosive arthropathy et ee Kidney: gouty nephropathy Tissue: Tophi Clinical picture: Asymptomatic hyperurecemia + o Just elevated levels of uric acid, Ji De peal 0 90% of hyperuricemie patient Symptomatic: 4- Acute gouty arthritis: 3D Precipitating factors © Excess protein, Alcohol o Drug: hiazide, lasi o Surgery, Trauma. © Infection. | © Dehydration. Distribution : © Monoarthritis. © Usually in the first metatarsophalangeal joint (of a $< the big toe). © Other sites : the ankle, the dorsum of the foot, the CCamSeanner se tessa!In Capsule Series — a of movement. Description : <<, Swollen & limitation 4 Tenderness, SWOTER og overnight andre. © Hotness. Redness. °° = ien develops overnight and reaches . inful, iO ick is severely pal nthe early morning ) oO Acute atta aii within hours (waking the paver’ © ——— Dramatic relief of pain with colchicl Dram i it. 6. Slight fever & chills may be presen Deformity : No. It should be differentiated from other ca DD of acute gouty arthrit e.g, septic arthritis see later his 2- Chronic gout : i- Arthritis : © Polyarthritis. o Asymmetrical, o Erosive. © Remission & exacerbation, ——_* eenation. 0 Deformity ii- Gouty nephropathy; © precipitation of uric acid into renal tubules > ARF R © Precipitation of uric acid inp ee nal interstiti i iti 5 om stitium > chronic Interstitial nephi © Precipitation of uric acid ji D uri ili. Tophiz Stat > urate Stoi Hematuria, infecti nes : —- a a © Occurs with Vere hyperuri emia, S With sey h © Tophi deposi S Occur ~ 0: CUr Ug Ul ally jj Surface of 4 MY in the ski a: Sue NBCIS & oay lobule, St wound the joints, erate & dicen, -40- "8e chal CCamSeanner se tesaOp Reuatology 4 wvestif ations:| fe Uric acid (N=2.5-7me%) : > 7 mg% in S > >6mg% in 9 —=_—_— | [ 1p It is a biochemical hallmark of gout, but not b y itself diagnostic for gout. a Minority of patients showing acute gout with normal serum uric acid, so — — —. | serum uric ; said does not exclude the di | jis Xray: o Soft tissue swelling around the affected joints. ———— —_—_—_— ° punched out lesion ¢ out lesion & deformity i in chronic gout. Lai Aspiration of synovial fluid ( Arthrocentesis ) : © Urate crystals by Polarized microscopy. L® It is the single most useful diagnostic study in initial evaluation of monoarthritis. iv- Biopsy from tophi. y- Blood picture : leucocytosis, TESR, t CRP. vi- UiS kidney. T reatment; i Asymptomatic hyperuricemia: No need for treatment except with : > +ve family history. > Uric acid > 11mg% eae > 1 excretion of uric acid in urine. {i Acute gouty arthritis: & * NSAIDs: © Indomethacin : : 75mg & then 50mg/6h | for 2 or 3 days. © Diclofenac: 75 ise & then, 50mg/6h orn on use aspirin n because i itcan alter uric pci level. CCamSeanner ss tees!lowed by, 0.6 mg one hour later, e fo —_— aa < cated in renal failure, at the first indicated in renal failure, a vomiting. Contrain - S/E: diarthea, «Short term cortisone: icemic drugs) f III- Chronic gout : (Iypouricemi i Iil- Chronic gout; 1- Diet : oultry intake. © Avoid excessive meat, fish & © Avoid alcohol . Le Excessive fluid intake (2-3 L daily ) 6 Try other good protein such as eggs. 2- Medical treatment : ¢ Allopurinol (zyloric) : © Dose: 100-300 mg/d © Mech, : xanthine oxidase inhibitor. © SIE: fever, diarthea, Allergy , BM depression. © Allopurinol is NOT used during the acute gouty arthritis as it my aggravate the attack. MCQ diarrhea, ash, arthralgia, headache, increased hi © Uricosuric drugs (probencia) 8 ~ Dose: 0.5-len/12h, tH ¢ Febuxostat : is a new xanthine oxidase inhibitor. Dose: 40 me)/d. S/E : nau lepatic serum enzyme. U 5 oe ised with alkalinization ofurine, * Pegloticase : Itisanew drug for the treatm 1) . lent of sever, é fi Pegloticase is recombinant nye eatment-refractory, chron ————— Porcine. —_ a a ain li i allantoin, This reduces the wish ike Uricase, ]t metabolises — ——s of precip; 7 mo! . iit . 7 P *e soluble than uri acid, Pilates, since allantoin is five to loticase ig «5 ECCS given as any ravenous j n & intraartion, Be Infusion e : ter injecto, Very two week: =f corti y SO} None. CCamSeanner se tealjne alkalinization : by the use i ur di Urine alkalinization : by of sodium or potassium citrate or acetazolamide, 500 mg at bedtime. ae A o Excessive fluid intake (2 ~3 L daily ) (chondrocalcinosis) Definition: o Deposition of Ca pyrophosphate ( CPP ) crystals in cartilage. ° Can cause monoarthritis clinically indistinguishable ‘from gout — Pseudogout Etiology: o Idiopathic. o It can occur with certain metabolic diseases: hypercalcemia, DM, CRF, hemochromatosis. Clinical picture: 2 = 9 0 Mono arthritis especially in the knee. o May be asymptomatic (deposition of CPP in cartilage). Investigations: © X ray: cartilage calcification. © Ca-pyrophosphate crystals in synovial fluid. | o ESR Tt _ ‘Treatment: NSAID —_- Intra-articular cortisone aa Ue ee [Pseudo-Pseudo Gout "> Hydroxy appatite arthropathy. ae >d shoulder joint is the commonest site. CCamSeanner soe tesaVasculitis is a group of clinical s od vessel predominately involves the blo. Etiology ¢ As scheme. lassification + © Small vessels : ? Henoch-Schink ein purpura . 2 Wegener's granulomatosis . | — ce, id de; Pest Vvirug —re 2Stt'6 FVasculitis of RA, SLE. | Prete Eh Snreas go en eae ea eae N. © Medium sized vessels : # Polyarteritis no + Kawasaki's disease . ? Churg-Strauss syndrome . © Large vessels : PE 7-5 Sential Choo Yoh uci Chralgia 4 ay y tong | Usual syndromes characterized by inflammation that Js of unknown etiology. pain eb (according to the size of ‘vessel involved ) VASCuUEIS + Ao, -With hePatd ; 5 Ae ©CClussign t sma, ve \dosa . “Anorexia & loss of weight 7 Beneral sch, Aso cay, TCU fog y ome Recife 2 &%.-Yenad inssuPfe aBiecteg, anifestations depending The cg el tata) uy, Same ag my) a CCamSeanner ss tessatxclude other conditions that simulate vasculitis: 1) | + Vascular spasm : e.g. iatrogenic ( ergots, cocaine ) —_——_ —_— er therosclerosis. « Embolic conditions : Infective nfective endocarditis, myxoma, . Conditions with increased thrombotic tendeney : DIC, Tr, antiphospholipid syndrome. 2) Exclude conditions which can cau cause 2° vasculitis : e Connective tissue diseases e.g, RA, SLE. e Malignancy : lymphoma, leukemia, —————— e Drugs : NSAIDs, antibiotics. ¢ Infections : rheumatic fever, infective endocarditis, Lyme's disease. © Cryoglobulinemia. 3) Take notice of the patient's age & gender. 4) Decide the extent of organ involvement and try to fit the finding into a specific vasculitic syndrome. ee eee Investigations! : 1) Laboratory : neva | o CBC, ESR, CRP, urinalysis, renal function tests, hepatic enzymes, -MS Enzymes o Rheumatoid factor, ANA, Antiphospholipid antibodies. a eutrophil cytoplasmic antibodies (ANCA ) :+ve in WG ,-ve in PAN 2) Vascular imaging : Duplex, Angiography. — 3) Other imaging studies : © X-ay and CT scan for chest. © Echocardiography. 9° Abdominal US or CT. 4) Biopsies : May be needed for confirmation or exclusion e.g. skin, muscle, CCamSeanner si tealAllergic purpura) i purpura (Anaphylactoid Dt ’ 's mainly in children, i 1 vasculitis which occurs len, — It is a type of small vesse: ytopenic purpura in children, the most common cause of non thrombocyt le — Itis the mos De SS = Age peak : 2-8 years. i = 1008 1) oe : — ash especially over buttocks & extensor surface of legs, 2) Joints : 75% © Migratory polyarthritis, ©. Particularly knees and ankles. 3) Abdominal pain & GIT bleeding due to mesenteric vasculitis, ? 4) Renal involvement : (the worst, ‘prognosis ) © Focal glomerulonephritis, raed al © Asymptomatic proteinuria & hematuria. ———— na estigations ; 1) Allinvectt..< 7 ) All Investigations of | bleeding tendency are normal exce 2 pt Hiss test maybe ) Platelet count and fiction are normal, 3) Elevated Serum IgA level is suppose 4) is8estive, Skin biopsy TTA deposits, Freatanene Treatment hy Progn Osis : ~ Spontaneous Fesolution wi i - Chronic renal tue CCamSeaner se tesa!| Se nudemions (2 ippes SPOT tract : Sinusitis , epistaxis, . saddle shaPed nose “ yung ¢ : Pulmonary, nodule. Glomerulonephritis. 7 (/a Kidney + Glomerulonephritis, | GIT involvement is uncommon 2 Bye: scleritis. ieee iin 9 Ear: otitis media. 5 ANCA : +ve in 90% of cases . 5 Treatment is with high-dose Steroids, methotrexate, and cyclophosphamide. trauss syndrome! YVlad .-Ypiits -BA - S¥stenvc vasculitis hur 2 Bronchial asthma. 9 Esinophilia. Kawasaki disease} - Usually in children. -Redeye - Red lips & mouth - Red hands & feet - Red skin (rash) - Cervical lymphadenopathy - Vasculitis of coronary arteries : coronary aneurysm myocardial infarction may occur. _ 449) Treatment : > Aspirin & immunoglobulin are used. c® Cortisone is contraindicated (} coronary aneurysms). disease |( Pulseless disease) ‘akayasi “Affects the aorta & its main branches e.g, subclavian artery produeing : © Arm claudication . © Absent pulse .In Capsule Series Gian arter' ; Il (remy od) ult mee Comma; it cel 6 vase aa 1 Sranuwlomtou. nial vessels especially ranches of cting the cr ffe = Vasculitis of large vessels a temporal & ophthalmic arteries. , le to male ratio 4 Hl Oy) with femal lly affects the elderly (rare <50y) ical pit 2 Tbusual Clinical picture : fal anorexia, fever, night sweats, wei tt loss, depressi ae xia, fever, night sweats, welg! > Ge : ; : malaise, hi Pee tenderness (on brushing j, ir ilateral throbbing headache, facial pain, scalp LS Unilateral thr en ion, = > Visual disturbance up to sudden loss of visi i ters. > Jaw claudication : due to ischemia of the masse lymyalgi ica : in 50% of cases > Polymyalgia rheumatica : in Syndrome of Proximal symmetrical muscle pain and stiffness wors " : 2 a ears of treatment F freatment (50% if stopped after 2 ” Relapse ig common after stopp ears), : ‘camSeanner +pefinition ¢ a systemic necrotizing vasculitis with aneurysms formation, affecting medium sized arteries. — tfonly small vessels are affected, itis called microscopic polvangiitis ° microscopic . ogy? Awoimmune, Hepatitis B play a bg role (HBsAg +ve n30% of cass) Giinical pictures 6:9=2:1 @ o Renal (60%): Not Imag ComPlO¥ tAduete Anuteism. > Infarction rather than glomerulonephritis . ifarction ee > Hypertension & renal failure . o Neurological : > Stroke - > Mononeuropathy , mononeuritis multiplex . © No pulmonary involvement in classic PAN . o Cardiac : coronary heart disease. 9 Git mesentric occlusion + acute abdomen.-9it bleeding Ischemic bowed © Genitourinary : Orchitis . “Wansiminase edevatien © Musculoskeletal : Arthralgia, myalgia. — ——_ o Hepatitis B is associated in 30% of cases. Notice that it is Polyarteritis NOdosa : So, thereare2NO: © Investigations : + No pulmonary manifestations. % NocANCA. © Biopsy . © Angiography : shows multiple aneurysms . 9 TESR, mild esinophilia . 2 HBsAg is ve in 30% of cases . © cANCA is -ve (+ve only in < 10% of cases ) . Bevel ane oo Tr P . *atment : Cortisone & immunosuppressive . _ CCamSeanner se tesa!0 te y thickening & fibros disorder characterized b; ' tive tissue It’s a connect vA “Handsbri, witl | organs. nt of blood vessels & internal ‘ith involvemet a Tm st PE Fibers papal rd skin. bo saa ti t literally means hard ski © Skin thtemg © Infact scleroderma (saan the body. . . ay : deposition all over - Fede... Mas Fagg, ty “atholeay: a aint ors patth bearnase, y, a “ Peri or, i linical picture: Q:d=3:1 © ‘UL Ful Yous Clinical : , ~~ others. _ey, the in: “NLADRI,OR3,0R5 etc I5 lena anon: ass. with enuiemental -® Yuritte i ° Early: edema. Patter s © Later : Induration (hardening) of the skin with loss of its Clastigy L~® Skin hyper/hypo-pigmentation , ulceration & Calcification . > init in 70% , all pa scveyedeym® v-@ Raynaud's phenomenon : initial complaint in 70% , al Patients =< Phenomenon \Synest Commen eventually develop it during the course of their disease. Couge. e GIT: © Esophagus : Dysphagia sTeflux. (80% of Cases ) © Small intestine ; Malabsorption syndrome | © Large intestine - Constipation, ° Renal : © Vasculitis of renal bloog Vessels : hypertension & CRF Scleroderma Tenal tits a life-threatening condition menfested by sudden ong of malignant hypertension & ARF: * Cardiac: 9 Cardiomyopathy, oP i . aati & Peticardial effusion, © Ischemic heat diseaggs ee © Heart block . 50 = fibr, SiS of the Con Ucting Pathways, ‘CamScanner sd psane Rheumatology . Chest o Interstitial pulmonary fibrosisu. Coyn, mon o Pulmonary hypertension . vaScutttls - FtbYoSI'S o Aspiration pneumonia : due to esophageal reflux . + CNS : Headache and stroke during hypertensive renal crisis 0 ei rena ISIS + Musculoskeletal : o Arthralgia & rarely arthritis . (Poly- Smatland large Seints J o Myopathy._ myalgia of myoSitt s variants (types) of scleroderma : 8 1-Diffuse scleroderma : - Diffuse skin hardening involving trunk & proximal limbs as well as face & “distal limbs . 7 = —_ - Severe internal organs damage and is generally more life threatening. 1-Limited scleroderma :_ (CREST syndrome ) - Skin hardening is limited to the fingers , face ,feet . BF - Internal organ involvement is not sever, much better prognosis. - CREST (Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia) 3-Localized scleroderma : (Scleroderma without internal organ involvement) ~Morphoea : local patches of scleroderma . 4 Systemic sclerosis sine (without) scleroderma : rare ~ Internal organ involvement without skin manifestations . 5 . ., Overlap syndrome : Scleroderma associated with other autoimmune disease. CCamSeanner se tesa!Jnvestigations : o TESR & CRP. : © ANA: +ve in 90% of patients. © RF: +ve in 25 % of patients. © Anti Set-70 ; 30% of diffuse scleroderma . "Co Anti-centromere: 70% of limited scler © Biopsy. pyym SKIN Anti , Fat @ tePotsmevasel» sPeric put Net Sense pal Because not all people with scleroderma have these antibodies and because not all peo, ple the antibodies have scleroderma, lab test results alone cannot confirm the diagnosis, “relieving symptoms and limiting damage.” © Penicillamine - slow aoa ~ Arthritis : NSAIDs, ~ Raynaud's - S: Ca ch 'annel blocker Vax dl ato } 25a ch a ENN Flyer | Renal si : ACE inhibi 7 rt Cis drug of Choice "jj PGE (Mj Miner BY TF Pound Bs ma, [1 sctere Seley, . duima: | 8 Bi base 2 0b p tthing 4 "IS Fibra ses Or asf Fe Smile ANd the ih d F law. CCamSeanners > esl~ tsa degenerative arthritis characterized by breakdown of the cartilage with osteophytes formations, results from wear & tear on the joints, _ OAs the most common joint disease. McQ _ OAunlike RA, is not an inflammatory disease, 4-Primary : unknown etiology , genetic factors play a role, ws ually DIP (nodal i of non nedat.) o Congenital : hip dislocation. o Mechanical : occupational, sports injuries. 6 Endocrinal : Acromegaly, myxedema, hyperparathyroidism, DM o Metabolic : hemochromatosis. o Others : end of any inflammatory or infectious disease. Risk factors |: © Aging (>45 y) : over time.as a joint moves, cartiage deteriorates &smooth surface becomes rough © People with family history of osteoarthritis . Sex : more common in Q . © Obesity ; loading stress on weight bearing joints e.g. knee . © Overuse of joints. Sommon joints involved are : McQ © Knees : the most common. © Thumb base (1* carpometacarpal joint ) : the 2 most common © Hips. © Spine ( cervical , lumbar ) © Distal interphalangeal joints (DIP) : | Heberden’s nodes. \CamScanner se tpsapsule Series : ‘d's nodes, In Cay Se = caphatangeat fin (PIP) : Bouchar 8. © Proximal inte © 1* metatarsophalangeal joint. ticular Or monoarticular pa ir metrical oligoal > These joints are affected in asym’ nano iter in pri 2 Metacarpophalangeal joints are uninvolve most mon joint disease , Clinical picture] is the most com! (linical picture]: again, OA is occur. mecan'Ch 1. Pain (+ by exercise & | by rest ), stiffness may ak er 2- Crepitus on movement . / 3- Minimal deformity & wasting of muscles around the joint . 4- No systemic manifestations. Moming stiffness is not usually a prominent feature of OA, & when ‘more than a quarter of an hour. Investigations] : Diagnosis is made on clinical & X-ray finding. © Normal laboratory finding. (RF, ANA are -ve, ESR, CRP are Normal) © X-ray : (70% of 70 years old ) Present lasting » Joint space narrowing . > Osteophytes . > Subchondrial Sclerosis, -54- Nd Yorin SULP ate ita Pee CCamSeanner se tpsimmune inf i ositis is an autoimmune inflammation of skeletal muscle. When associated with ae lesions itis called dermatomyositis. PY Dmy 3 ia picture: (MOHEM ) COB Cemmediated 3 coy cp ¢ (M) Myositis Adut-04 ua, — chuaven-adubt — Mainly proximal muscles, symmetrical. Fam oo a - Involvement of esophagus : dysphagia. - Involvement of respiratory muscle : respiratory failure, o (0) Oedema. . [6 (H) Heliotrope discoloration of the eyelids» Puy PIE Yash over eyelid Vf (E) ythema L~ Gottron’s sign : symmetric erythema over the extensor surfaces of metacarpophalangeal joints, elbows and knees.Dt agnostic, oF OMY (Rathopenit) A Diffuse flat erythema over the upper trunk and shoulder, the forehead ( shaw S19n) o (M) Malignancy : this occurs particularly in males with age above 50 years, “C™CiFrcat ‘gations . “7 ShUst 519 = Orythmreus Yosh at WoPat hic Investigations : GuBY® ROCK WAPPEF ehest-sheuey. GSPrimary Wo |- Elevated muscle enzymes : CK , SGOT , LDH Pie malignant Synd —_ 3) ct disease »-slé Ab Abnormal EMG . - Scleroderma 3. Muscle biopsy. Magu ein gourrg 4 Myositis specific antibody : Anti-Jo-1 (30% ) (Sl myoPathic. severe Onk ML-2. Iter: esttustion of yns itis ® Prednisone : mg/kg/d then kept at 10-15 mg/d. laine ae a mre pod 8S -T lymPhé o ~ Glpnarmat Pindue to deqentel © Methotrexate 5-20mg/week ue: x Immunosuppressive : © Azathioprine 50-150 mg/d nd Cyclophosphamide, aN "i 2 pens fe _ *Veanma globulins and anti Rituximab : in severe cases. ‘camseannerSe In Capsule Series a ~ Infont HAnFluenzg ; -ch idern SPC ena Classification : : wl “Seung adust = M- Sonar [fp Bacterial septic arthritis : ~All aes. S-oufeug ; a) Non gonococcal : -Debilu ting Pin Glin yg | ~ Staph aureus. m(P-Pkn with scale cewanenja® - Streptococci . Salmonetla,. ~* = Gram-ve bacilli. b) Gonococcal : caused by Neisseria gonorrhea. [Zo Lyme disease. “ime disease [Bo Mycobacterial (immunocompromised) _e CamUnOCoMmpromused) [Jo Fungal : (sporotrichosis most common) © Viral arthritis, a Risk factors : © Advanced age . —_ et © Drug abuse, © DM & suppressed immune system , — © Rheumatoid arthritis . tienen © Artificial joints recent o1 mn Joint surgery or j icular injecti 'Sery or intra. ular injection of cortisone. M: Usually sing): ie gle joi : ag cotaaetitis) tl specialty the knee. (80% of casesis © Signs Finfammation = 4 of movement “Aotnes: eee S , red, ——’ Sdnes, ——y tendemess 38 CCamSeanner se ealBlood: Leukocytosis , t ESR , blood culture, ood oe ee 4) Xu articular erosions may occur, ¢ Synovial uid examination: +e culture, + neutrophil The synovial fluid should be examined for crystals, because gout & pseudogout can resemble septic arthritis sie, treatment : & o Antibiotic (TV) : according culture & sensitivity test.e.g. : Ox, Clox, Diclox- acillin or vancomycin for staph. For about 4 weeks, o Analgesics. eee co Drainage of the infected joint daily. 4) Gonococcal arthritis & Most common in young healthy adults ( females > males ) © Caused by N. gonorrhea. OO o Clinical features: |. Migratory polyarthritis. 2. Tenosynovitis. 3.Skin lesions, usually as a small macule or papule on the distal extremities. > MES : © Lyme disease is caused by spirochaete, Borrelia burgdorferi, which is transmitted by ticks. © Diagnosis: Lyme disease is diagnosed clinically ~ The earliest manifestation of Lyme disease is reddish rash ( erythema he earliest manifestation ¢ is reddish rash (erythema migrans ) and flu-like symptoms. Then, In subsequent weeks : ~ Neurologic manifestations : Bell's palsy, meningitis. ——<— «sr > Cardiac Cardiac manifestations : AV block, Myopericarditis. 3 > Arthritis can develop with knees being the most commonly affected. antibodies to Borrelia. — —_ ve Camscanero Sopa(or amoxicillin if <8 yrs or pregnan in, manifestations: askin . a osed Lyme is treated with intravenous ceftriaxone, 2» Late diagn i 3- (es Back pain, kyphosis, cord compressigy _ n © Spine involvement (Pott's disease ) : 6 Peripheral joints : monoarthritis typically involves hip, knee or ankle Ee Involves a ee co Common viruses associated with arthropathy include parvovirus. . Tubell HIV & hepatitis B and C. Ce 3: ae Eom : Flu-like symptoms , skin rash & acute polyarthr j © Viral arthritis is usually self limiting. — —_—eeeting. ‘camseanner sihe most common metabolic bone disease, characterized by reduced by ed bone _ itis! ty and increased risk of fracture. ‘the World Health Organization (WHO) defines osteoporosis as a bone mineral density (BMD ) that is 2.5 standard deviations or more below the young adult snean value. Normal bone | T-s. T-score between re greater than and -2.5 } T-score less than -2.5 | 4-Primary osteoporosis : is subdivided into type 1 and type 2. | a) Type | ( postmenopausal osteoporosis ) : Most common in women after menopause due to estrogen deficiency. There is high incidence of vertebral b) Type 2 ( senile osteoporosis ) : occurs in old age and is seen in both females and males at a ratio of 2:1. There is high incidence of hip fracture. 2 Secondary or type 3 osteoporosis : occurs at any age & affects men and women equally due to many causes. pe Causes / Risk factors | : © Genetic factor : the single most significant factor. © Endocrine : Y hormones : Estrogen deficiency in females, hypogonadism in males. ism. Y T hormones : Cushing's syndrome, thyrotoxicosis, hyperparathyroidism. © Drugs : cortisone, heparin, anticonvulsant, chemotherapeutics. —S—e—e— wre eE—E_—e———eooo ° Malignancy : multiple myeloma, leukemia. . © Inflammatory : rheumatoid arthritis, ulcerative colitis. GI: malabsorption, primary biliary cirthosis. : Prolonged immobilization, smoking & alcohol intake, \CamSeanner + tpsc p ie _ngical osteopenia. Tl gain, osteo eros en “oti it jicture] + SF a, | vo tomate —_ or neck pain- (Colles fracture), sping 1. Asymp! only low back pain ihe forest ( wal 2, Pain: particular ns ymmon site ©) — — a = low crushed vertebrae. (vertebral fracture) ee a bdominal 4, Kyphosis & al {vestigations el ally normal i er Ikaline phosphatase : are usually in © Calcium, phosphate & al primary osteoporosis. i yroid function of the cause : e.g. cortisol level, thyroi tests, o Investigations TL. Imaging : o X-ray: may show fractures & osteopenia. Bone mineral density (BMD ) : is measured by DXA (Dual-emission ° X-ray absorptiometry , previously DEXA). [Ereatmeni 1. Prevention : © Diet : adequate dietary calcium & vitamin D intake. © Weight bearing exercise, © Avoid smoking & alcohol, ~~ © Coneet any secondary cause. Il. Medical treatment ; Coes © Calcium & vitamin D supplements ° Bisphosphonates ©.g. aley © Estrogen replaceme (Raloxifene ) a metri © Recombinant Human p ‘rial o breast cang CCamSeanner soe tesa!osteomalacia is the softening of the bones due to de: fective bone mineralization gevondary t0 inadequate amounts of Phosphorus and calcium. The most common cause is vitamin D deficiency, osteomalacia is often restricted to the adult form of the disease adult form of the disease, steomalacia is not the same as osteoporosis. Osteomalacia results from a defect inthe bone-building process, while osteoporosis develops due to a weakening of Causes ]: ‘The most common cause of the disease is a deficiency in vitamin D, which is normally obtained from the diet and/or sunlight exposure. | __ osteomalacia in children is known as rickets, and because of ths, use of the term | | | | | | . Dietary deficiency or inadequate sunlight exposure. Malabsorption syndrome. Malnutrition during pregnancy. Chronic metabolic acidosis : Chronic renal failure. Renal tubular acidosis. ween Abnormal vitamin D metabolism : © Lack of hydroxylation by kidney due to renal disease e.g. CRF. © Long term anticonvulsants e.g. phenytoin : may cause hepatic enzyme induction and increase vitamin D metabolism © Vitamin D dependent rickets type 1 ( VDDR type | ) : an inherited defect in hydroxylase enzyme. 6. Bg tia iene to — D : Vitamin D dependent rickets type 2 (VDDR ‘YPe2), an autosomal recessive disorder of vitamin D receptors. we Npophosphatemia : with normal vitamin D © Familial hypophosphatemic rickets. Renal Fanconi syndrome : phosphaturia, aminoaciduria, & elyeIn Capsule Series ; : muscle p?! 1. Skeletal discomfort : bone oe es 2, Proximal myopathy with wa lop 3, Pathologic fractures may devel a of hypocalcemia : ¢-8- 4, Manifestation: utraphy where the latter shows hyperDhosnhg il osteodyst ducers fom renal axteodstrophy, wher’ 116 SSE NPE phate I. Laboratory : (Biochemical findings ) ion in blood serum, 1. The major factor is low vitamin D concentration in bI 2. The serum calcium is low ( or low normal due to 2°” hyperparathyroidn . The 3. Serum phosphate is low except in cases of renal osteodystrophy, 4. Serum alkaline phosphatase is high, Tl. Imaging : (Radiological findings ) Defective mineralization with Bseudofractures ( Looser's zones ) : A translucent band of bone material of decreased density may form alongside the Surface ofthe bone, This gives the appearance ofa false fracture, Typical sites of Pubic ami, proximal ends of the femur and ulna, involvement are scapula, ribs, © Diet adequate dietary calcium & Vitamin D intak €. © Adequate sun ©xposure ( ultravioles jj ht ) 2 Calcium & Yamin D sei, ebblements, ° Treatment OF the cause, CCamSeanner se tealSSS) FAVS NN = ns pM isa intermittent febrile disorder with inflammatory serositis, arthritis, and rash, gy | pMFis@ hereditary inflammatory disorder. It is an autoinflammatory disease sqused by mutations in the gene MEFV, localized in chromosome 16, - Faster Mediterranean persons ( especially Arabs, Turks, Greeks, Armenians, and Sephardic Jews ) are most frequently affected. ~ More than 80% of all patients have their first attacks before they are 20 years old. Initial attack is very rare after age 40. = Most attacks involve fever. ~ Theattacks last from 6 hours to 3 days, arthritis may last for several weeks. Disease free intervals may last days or months. ~ There are 7 types of attacks : . Abdominal attacks affect the whole abdomen with signs of peritonitis, and acute abdominal pain like appendicitis. They occur in 95% of all patients and may lead to unnecessary laparotomy. 2. Joint attacks mainly occur in large joints, especially in the legs. Usually, only one joint is affected. (75% ) 3. Chest attacks include pleurisy (40 % ) and pericarditis ( rare ). . Scrotal attacks due to inflammation of the tunica vaginalis occurs in up to 5% and may be mistaken for acute scrotum (i.e. testicular torsion). . Myalgia . Erysipelas like rash. - Fever without any of the other symptoms listed above (25%). ‘camseannerid protein is produced in very | iz between them, and accumula : ‘Amyloidosis with renal quantities during attacks, in the kidney, as well as the © The diagnosis is clinically made on the basis of the history of typical a specific test is available to detect familial ions in the MEFY gene. failure ¢ amylo' and at a low rate ° heart & gastrointestinal tract. Mediterranean fever. © A genetic test : to detect mutati © Markers of inflammation : ~ Leukocytosis, elevated ESR & C-reactive protein levels. = Sterile exudative peritoneal or pleural fluid. © Monitoring the renal function is important especially in patients with a lo history of attacks. Differential Diagnosis] : © Acute appendicitis, rheumatic fever, septic arthritis, systemic juvenile arthrit > ‘enile arthrit * Pyrexia of unknown origin (PUO ). : © Symptomatic relief. is the goal of ther Colchicine as prophylaxis Tapy e.g. NSAIDs ° CCamSeanner se tealom oss is 8 multisystem granulomatous disorder of unknoy eto ogy, i most ting young adults. Granul ve nly affecting Y‘ ‘omas most often appear in the lungs or the a nodes, but any organ can be affected. ye the characteristic granuloma composed of macrophages, lymphocytes with iprace_ltular inclusion bodies. _ The exact cause of sarcoidosis is not known. — Most probably due to alteration in immune response after exposure to antigenic stimulus (an environmental, occupational, or infectious agent ). 1. Itmay be asymptomatic. 2, Pulmonary : dyspnea, cough, wheezing , may be hemoptysis. 3, Upper airway : nasal obstruction, hoarseness of voice. 4, Lymphadenopathy : painless, rubbery LN enlargement. The cervical & scalene lymph nodes are most frequently affected. 5. Skin : SARcoidosis Osa2xO© 58: Chest, Skin, Cardiac, CNS, Ca. 2A: Anterior uveitis, Arthritis. Lymphadenopsthy © Erythema nodosum ( red painful bumps on the anterior aspect of the leg) © Lupus pernio : violaceous lesions on the nose & cheeks, 6. Bye: Anterior uveitis, 1. Joints : Polyarthritis. 8. Cardi i Cardiac : Arrhythmia, congestive heart failure. i oe ' CNS : cranial neuropathy (facial nerve most often affected ), diabetes insipidus 65> CCamSeanner se tealIn Capsule Series to 10. Renal : ARF can be due el 11, Hypercalcemia : due to exe —_@ Chest X-ray: “ral ilar Iymphadenopathy 4 [Stage 1 { Bilateral moi infiltray Bilateria Iymphedenopathy and pulmonary infigag Stage IT | Bilat [ Stage IIT | Pulmonary infiltration only > 3 Stage IV [ Fibrosis. ia & granulomatous interstitigne Icem! ercall oe hyp: ction of vitamin D. thema i © The combination of biateral hilar ymphadenopathy erythema nodosum in ia is faren syndrome. association with fever & arthralagia is known as Lo} © The main differential diagnosis are TB & lymphoma, and every effort Should be ‘ ade to confirm the diagnosis of sarcoidosis histologically ( Lung ,LN or skin vl 1. Cortisone is the main therapy, 2. Others: Methotrexate, chloroquine, 3. Lung transplantation in advanced cases, Og G00d prognosis Lofgren's syndrome P Oorer prognosis with chro, chronic uveitis has complete Tesolution in 80% of peop nic diseas, Lupus Pernio, nasal Mucosa involvemé » Chronic hypercalcag, mia, Nephrocalcinosis, Neural involve 388 Breater than 40 ang Black race, © Prognosis according to chest Xray: v Stage || ; 50% Cases "ecover \CamSeanners se tgain is a common musculoskeletal disorder. Nearl took a ly everyone has low back ime. sot ya |, Mechanical «5 Lumber dise prolapse. o Lumbar spondylosis. o Lumbar spinal stenosis : is a medical condition in which the spinal canal narrows and compresses the spinal cord and nerves at the level of the lumbar vertebra. This is usually due to spinal degeneration that occurs with aging. Osteoarthritis. ° Fractures. ° ° Bad posture ( sitting, standing ). ° Leg length difference. ° Congenital : Scoliosis. 2. Inflammatory : © Seronegative spondylarthritides (e.g. ankylosing spondylitis). o Rheumatoid arthritis. 3. Infection : © Osteomyelitis. © Pott’s disease (TB ) 4. Neoplastic : © Bone tumors (primary or metastatic). ‘ © Intradural spinal tumors. Metabolic ; i © Osteoporosis. 9 Osteomalacia, CCamScanners Use tsao Peptic ulcer. o. Pancreatitis. © Pyelonephritis. © Prostate cancer. 7. Psychogenic low back pain. rer a cel = The intervertebral disc is formed of ; i pulposus ", surrounded by a fibrous tissue ring i ' ilagi te. covered from above & below by a cartilaginous pla ntral gelatinous part " Tuc} annulus fibrosys » Acute disc prolapse : sudden rupture of the annulus fibrosis followeq ty Cu ; bulging ( herniation ) of the nucleus pulposus. This compresses the spiny roots. It may occur at any age due to trauma e.g. lifting heavy objects o7 Jumping to the floor from a height, Spondylosis : It is a gradual, progressive degeneration of the intervertebral discs, specially freely mobile discs e.g. cervical & lumbar regions, leading hemiation of the nucleus pulposus, Dingnostc appreac nerve rot compress Ing to buttocks or thighs, Only true sciatica fi Tessin radiates below th i e kn © Ahistory of low back pain With 'ee to the foot, ic Morning st . . S ec oF ankylosing Spondylitis SSifness in a younger patient rai © Spinal stenosis . ” Pein Pain that worsens with Standing and relieved wit CCamSeanner ssoack signs : tenderness, deformity 0 * e root compression : Se : sjgsof nerve root compression 886 neurology, and you may understand @ Disc herniation (e.g. Lumbar disc prolapse ) occurs at the LA-LS level or L5-S1 level in 95% of all disc herniation : 1. Motor manifestations : Weakness of dorsiflexion occurs in LS root compression, and weakness of plantar flexion occurs in $1 root compression. 2. Sensory examination in these patients may reveal saddle anesthesia (decreased sensation over buttocks, perineum and posterior thighs ). 3. Bladder dysfunction. 4, Straight leg raising test : positive sign indicates sciatica. | | ee 3] Mechanical Inflammatory) | oe ample Disc prolapse Ankylosing spondylitis Acute Gradual 9) fee | ba : Any age Usually <35 Eiyonelnee nn Better. oy HH eg bladder CCamSeanner ss teesIn Capsule Series 10. (XSi : . . X. CT, MRI & Myelography : 17 the cases where "red flags" are res © Xray, CT, Recent significant trauma. Unexplained weight loss or to fever. ye 0 Previous or current cancer. i icosteroid use. Osteoporosis or chronic corticosteroid Age greater than 70 years. Focal neurological deficit. ye Xe xe ye Duration greater than 6 weeks. Treatment of mechanical LBP} 1. (ERR; ( patients education ) 1. Rest. 2. Position : © Avoid prolonged standing. © Sleeping on medium firm mattresses. 3. Avoid lifting heavy object while unsupported back especially in dise prolape 4. Weight reduction. 1, SEI = NSAIDs during acute attacks, 2 Muscle relaxants. 1. BTS 1. Physical agents : heat, ice, massage, ultrasound, and electrical stimulation. © Local anti-inflammatory effect, © Decrease pain & muscle spasm, © Decrease fibrosis & adhesion. 2. Exercise pr : . Program : e.g. flexion €xercise ( abdominal exercise ). WV, SORT on reg, i / g. microdiscectomy, laminectomy or spinal fusion. - Indications ; CCamSeanner se tesa!og eS ——— COLLECTIONS OF RHEUMar : a | 7 Mechanic: I- Mechanical | |, trauma. - Po MYaidia |© trauma. + Cervical spondylosis. Y™¥™SC4) 4 T umbar spondylosis, | |, piseprolapse. ~F'OFMYIEA! 5 Diss prolapse, fuscle spasm. © Muscle spasm, Inflammatory: Il- Inflammatory: ca « Sero-ve arthropathy © sero —ve arthropathy. Rheumatoid arthritis. © Brucellosi: Mca Iil- Metabolic: ILI- Metab © Osteoporosis. © Osteoporosis. * Osteomalacia, © Osteomalacia. IV- Neoplasm: IV- Neoplasm: © Metastasis e Metastasis * M. Myeloma © M. Myeloma « Lymphoma Lymphoma | [WeReferred Pain: V- Referred Pain: ~ Angina - Pancreatitis, Peptic ulcer | -Pancost tumor - Cancer pancreas | jane aneurysm, - MI— Renal colic | _ _| = Prostate or cervix disease, * Trauma, ’ Osteoarthritis, *moartherosis (hemophilia). harcot Joint. DoF i omy apthriticil : reeltflanmato arthritis} 3 “umatoid arthritis, ' an athritis, CCamSeanner se tees> Pain ( by cx > Moming stiffness. > ESR, CRP: tt > Extra-articular manifestations. Non-inflammatory : Pain ({ by exercise & | by rest ) > Normal laboratory finding. > No systemic manifestations. 1D of polyarthropathy _; 1. Causes of chronic polyarthritis: (> 6 weeks) © Rheumatoid arthritis, o SLE. © Systemic sclerosis, © Sero-ve arthropathy &g Reiter's syndrome, Psoriatic arthropathy. © Sarcoidosis, © Chronic gout, ° Osteoarthritis, © Vasculitis, 2. Causes of acute Polyarthritis E © Acute theumatic fever, © Bacterial endocarditis, © Rheumatoiq arthritis, (10 % OF cases ) © Reiter's Syndrome, Psoriatic rth; _ . arthriti © Viral infection : Tubella, hepatig, = ‘SB, Hiv Epstein-Barr. \CamSeanner se tps= DD of Polyarthropathy ' helpful i i e differential di, i iseg, ght May have the similar distribution of joint involvement but Tarely erosive, ; clinical manifestations & serological finding : . oli pro spondylopathy : re asymmetrical joint involvement. Central joints > peripheral joints. | oRF:-ve | qsteoarthritis : Absence of systemic inflammatory manifestations o Onset in later life. | Pain: T by exercise, ! by rest. © Common joints involved are : Knee, thumb base, Hip: | | o Normal lab finding & normal ESR | quroniegout : © Clinical manifestations : ....... o Investigations : | | Viralinfections : | | | ° May result in a polyarthritis clinically mimic RA. ° Skin rash, self limited course. © Itis important to exclude chronic hepatitis & HIV. b Gserg Ge. ° Rheumatic fever, _chYonle nen influmateyy oA - Auescslat” au? Acute an Neco sis Preudogout, Septic arthritis, Traumatic hemoarthrosis ical rheumatoid arthritis, | ShYonic -inpyematory ag TS i ca is CCamSeanner se tesaIn Capsule Series eee otred joint, high fave; trem! hils. Culture =~ Suggested bY opin: 1 HeUFOP oe ; Joint yseudomonas or go, Confirmed bY or streptococci OF P Bon oc 5 eg. siaphylocoeet or TB, et seately inflamed joint (osually small ean e Suggested by" 0m ther joints in hands, arms, legs, and feg, but ot ita time, a one 4, Tophi on ears and tendon sheaths. am pot serum urate (not always). Urate crystals Confirmed by: ively bireftingent in plane-polarised light) present on (negative joint aspiration. Pseudogout (Capyrophosphate arthropathy, chondrocalcinosis) Tuggested yr one painful joint (usually knee) especially in ut 7 elderly or history of hyperparathyroidism or myxedema or osteoarthritis or hemochromatosis or acromegaly. 'Ca-pyrophosphate crysialsin | Confirmed by: joint aspiration. synovial fluid ‘Rheumatic fever In fact, rheumatic fever is flitting polyarthritis. Diagnostic criteria... Traumatic hemarthrosis Suggested by: acutely inflamed joint after trauma. Confirmed by: joint aspiration: aspiration of blood from joint Rheumatoid arthritis tacs—————_| Leukemic joint deposits [Reactive artieiis a ; ing stiffness AS iuggested by: morning stiffness , Articular manifestations : ( arythema , amyloidosis Kray, atoid factor, Anti COP, charactoil Si ueeested by acutely inflamed joint Confirmed by. i by. leukemic Picture on perj = Peripheral film and bone Surgested by: asymmetri Sb00 Week ane Ot * infectig, ° oligoarthritis developing n elsewhere, CCamSeanner ss teal1 ntraarticular : : o Inflammatory : RA, sero-ve arthropathy, pseudogout o Septic arthritis. ° Osteoarthritis I. Extraarticular : Fractures, tumors, trauma, tendonitis. Referred pain : Hip, lumbosacral spine. of recurrent arthritis: o Rheumatic fever © Gout o Pseudogout © Rheumatoid arthritis, o Sero-ve arthropathy. Dof Symmetric arthritis; o Rheumatoid arthritis, oSLE. Hepatitis B What are 3 principal crystals associated with joint inflammation ? © Urate : Gout. °Calcium pyrophosphate : Pseudogout. ° Hydroxyapatite : Pseudo-pseudogout. fem ba induced rheumatic diseases : > Art ralagia: qunidine, quinolones, cimitidine, interferon, amphotericine B yal: - agia/Myopathy — ; glucocorticoids, Penicillamine, chloroquine, statins, quinolones, interferon, alcohol. + diuretics, aspirin, alcohol. : hydralazine, INH, Penicillamine, phenytoin. carbidopa , tryptophan. allopurinol , amphetamine , CCamSeanner se esl: Psoriatic , Reiter's , fie ee Psoriatic, SLE, Ra. ei Hs + RA, Pseudogout em ocho "4st CMC : OA 4 4- face RA,Pseudogout 9ONoLo¢cq) ad owe Carpal tunnel syndrome It Juvenile arthritis. DIP : distal interphalangeal PIP : proximal interphalangeal MCP : metacarpophalangeal CMC : carpometacarpal ‘uses of purpura in a patient with connective tissue disease 1- Vasculitis 2- Cortisone therapy, 3- Thrombocytopenia due to NSAIDs, 4- Amyloidosis, 5- Factor VIII antibodies ( SLE, RA), hat are the physical finding in carpal tunnel syndrome: 1- Wasting of the thenar eminence, 2 Weakness of the thumb. 3- Sensory loss of the first 3 4-Numbness & pain reprog pene an N Of the wrist for one minute. SA Actomegaly, Myxedema, (p, Ahmed Mowafy®) | Risk factors of amyloidosis | © Age: > 59 © Chronic infection Or i © Family history,eumatic fever. hy heumatoid arthritis. Ye 5. Gout. psiills disease. fect I- Infection: Bacterial 0 N. gonorrhoeae oTB o Staph aureus © Syphilis 0 Streptococci 0 Gram-ve bacilli : Brucella >Viral: © Rubella © Hepatitis B,C © Chronic septic arthritis . 1I- Immune: © Alll collagen diseases . TII- Metabolic: © Chronic gouty arthritis, © Pseudogout. © Amyloidosis © Haemochromatosis °Pseudogout || IV- Degenerative: V- Endocrinal: © Acromegaly. © Myxedema, T - Miscellaneous:| CCamSeanner ss teal—pveate jatfefever Pose streptococcal pharyngitis : SE Wee yyy | SB weeks oe a fis ‘mall joints. articular manifestations Big joints Erosive | Not erosive : | | Extra-articular t manifestations Investigations (Treatment ~~ Penicillin for pharyngitis | v= Aspirin for Arthritis. 2 Cortisone for Carditis. Differentiate between Reiter's & Behcet's syndrome ; | | Reiter's syndrome ee Behcet's syndrome [ Male : Female ratio 10-1 ? es a re —_ [fe Conjunctivitis -—— Uveitis Spondylitis & Frequent cx ———— en [827,85 ————_ BS ied aaa — Pere | @ || Treatment [Raps ——_[ Norole a [Immunosappressive CCamSeanner se tees