Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

2020

st
1 Edition
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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

CHILLER’s Q-45 (QAMC-BWP) presents:

“An Easy approach to Excel your Exams”

2020
CHILLER’s
Medicine Notes
FIRST EDITION
Edited by
Sami Ur Rehman, Haroon Ahmad Abdal, M. Hamza Hassan, M. Sohaib, M. Naveed, Zain Ghaffar, Hussnan Mehboob
Quaid-e-Azam Medical College, Bahawalpur
(2014-2019)

“RHEUMATOLOGY TOPIC”
14. Pseudo Gout 16
Contents (Davidson)
15. Vasculitis Syndromes 16
* Basic Concepts of Rheumatology 03
(Kaplan & Davidson)
(Davidson & Kaplan)
15(A). Wegner’s Granulomatosis 16
1. Rheumatoid Arthritis 04
(Kaplan & Davidson)
(Davidson)
15(B). Henoch Shonlein Purpura 16
2. Osteoarthritis 06
(Kaplan & Davidson)
(Davidson)
15(C). Behcet Disease 16
3. Ankylosing Spondylitis 08
(Kaplan & Davidson)
(Davidson)
15(D). Polyarteritis Nodosa 17
4. Systemic Sclerosis 09
(Kaplan & Davidson)
(Davidson & Kumar&Clark )
15(E). Churg Strauss Disease 17
5. Systemic Lupus Erythematosus 10
(Kaplan & Davidson)
(Davidson)
15(F). Temporal Arteritis 17
6. Septic Arthritis 11
(Kaplan & Davidson)
(Davidson)
16. Inflammatory Myositis 17
7. Gout 12
(Davidson)
(Davidson)
16(A). Dermatomyositis 18
8. Reactive Arthritis 13
(Kaplan & Davidson)
(Davidson)
17. Disease Modifying Antirheumatic drugs 18
9. Psoriatic Arthritis 14
(Kaplan & Davidson)
(Davidson)
18. Fibromyalgia 18
10. Antiphospholipid Antibody Syndrome 15
(Davidson)
(Kaplan & Davidson)
19. Raynaud’s phenomenon 18
11. Drug Induced Lupus 15
(Davidson)
(Kaplan & Data from Slide-Share)
20. Osteoporosis 19
12. Sjogren Syndrome 15
(Davidson)
(Davidson & Kaplan)
21. Paget’s disease 19
13. Enteropathic Arthritis 16
(Davidson)
(Davidson)

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

• Some Basic Concepts of Rheumatology:-

 Distribution of joint involvement:-
1. Polyarticular: When >5 joints are involved. e.g. see table, all have symmetric involvement except psori-
atic & reactive arthritis.
2. Monoarticular: When only 1 joint involved. e.g. see table.
3. Oligoarticular: When ≤5 joints ae involved. e.g. Spondyloarthropathies (AS) & OA involving small joints
of upper extremities.
Diseases presenting as an inflammatory Diseases presenting as an inflammatory
“monoarthritis” “polyarthritis”
• Crystal arthritis, e.g. gout, pseudogout. • Rheumatoid arthritis,
• Septic arthritis, • Reactive arthritis,
• Palindromic rheumatism, • Seronegative arthritis associated with psoriasis or ankylos-
• Traumatic ± hemarthrosis, ing spondyloarthropathy,
• Arthritis due to juxta-articular bone tumor, • Post viral arthritis,
• Occasionally, psoriatic, reactive, rheumatoid may present • Lyme arthritis,
as asymmetric monoarthritis. All other have symmetric • Entero-pathic arthritis,
involvement. • Arthritis associated with erythema nodosum , SLE

 Findings on joint Aspiration:-

1. The basic tests to run on synovial fluid are 3C’s(Cell count, crystals & cultures) and the Gram stain.
Disease WBC’s Crystals/Polarization
3
OA & Traumatic arthritis (DJD’s) 200-2000 cells/mm Negative traumatic
(<50% neutrophils)
Inflammatory disease (Crystal induced Ar- 5-5000 cells/mm3 Gout: needle-shape, -ve birefringent
thritis) (80% neutrophils) Pseudogout: rhomboid shape, +ve birefringent
Septic Arthritis >50,000 cells/mm3 -ve ( Gram stain & culture, usually –ve for GC but
(>90% neutrophils) +ve in Staph, strept, & gram –ve)

 Anti-Nuclear Antibodies (ANA):- present in different patterns depending on the staining of cell nu-
cleus: Specific ANA’s Diseases
1. Peripheral (Rim) pattern: may be seen with SLE.
2. Nucleolar pattern: is commonly seen with limited cutaneous systemic
1. Anti (naïve SLE only (60%)
sclerosis (lcSSc). DNA)
3. Speckled pattern: is more commonly seen in healthy people.
4. Diffuse pattern: non-specific. 2. Anti-Sm SLE only (25%)
with renal disease
 Antineutrophil cytoplasmic antibodies (ANCA):- are
antibodies directed against certain protein in cytoplasm of neutro- 3. Anti-histone Drug-induced lupus
phils. (95%)
1. Cytoplasmic (c) ANCA: diffuse staining pattern; seen in >90% of 4.Anti-Ro(SSA) Neonatal lupus,
pts. with Wegner granulomatosis. Sjogren & in 3%
2. Perinuclear (p) ANCA: localized staining pattern; found in PAN ANA –ve lupus
(polyarteritis nodosa) & Churg Straus disease. 5.Anti-La (SSB) Sjogren
6. Anti- lcSScl
 Conditions with +ve RA factor:- centromere (CREST syndrome)
1. Rheumatoid Arthritis → 70-100 %
2. Sjogren syndrome → 90% 7. Anti-RNP 100% mixed connec-
tive tissue disorder
3. Mixed essential cryoglobulinemia → 90%
4. Primary biliary cirrhosis → 50% 8. Anti-Jo1 anti- Polymyositis,
5. Infective endocarditis body dermatomyositis
6. SLE 9. Anti-Scl-70 & Diffuse cutaneous
7. T.B., 8. Age >65 years Anti-RNA poly- systemic sclerosis
merase (dcSScl)

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

1. Rheumatoid Arthritis:-
 Extra-articular manifestations and complications of RA:-  CRITERIA FOR
DIAGNOSIS OF
1. Systemic:- fever, wt. loss, fatigue, susceptibility to infection RHEUMATOID ARTHRITIS
2. Musculoskeletal:- muscle wasting, bursitis, swan-neck deformity, but-
ton-hole deformity, cock-up deformity, flat foot, ulnar deviation of fingers, 1.Joints affected:- score
*1 large joint 0
Popliteal (baker’s) cyst, Atlanto-axial subluxation is very common. *2-10 large joints 1
3. Hematological:- Iron-deficiency anemia, eosinophilia, thrombocytope- *1-3 small joints 2
nia *4-10 small joints 3
*>10 small joints 5
4. Lymphatic:- felty’s syndrome (RA+ splenomegaly+ neutropenia+
thrombocytopenia + lymphadenopathy) , lymphoma 2. Serology:-
5. Nodules:- sinuses, fistula, baker’s cyst *negative RF and ACPA 0
*low +ve RF or ACPA 2
6. Vasculitis:- Digital arteritis, Ulcer, Pyoderma gangrenosum, mono- *high +ve RF or ACPA 3
neuritis multiplex, visceral arteritis
7. Cardiac:- pericarditis, myocarditis, endocarditis, conduction defects, 3.Duration of symptoms:-
coronary vasculitis, granulomatous aortitis • <6 wks 0
• >6 wks. 1
8. Pulmonary (S-2013):-Nodules, pleural effusion, Fibrosing alveolitis,
RA + Pneumoconiosis (Caplan syndrome) 4.Acute phase reactants:-
9. Ocular:- Episcleritis, Scleromalacia, keratoconjuctiva sicca, scleritis *normal CPR and ESR 0
*Abnormal CPR and ESR 1
10. Neurological:- Cervical cord compression, compression neuropathies
(carpal tunnel& tarsal tunnel), peripheral neuropathy, mono-neuritis multi- **Patient with score ≥6 are
plex considered to have definite
11. Amyloidosis:- Nephrotic syndrome RA. ( Mcq Annual-2019)

 INVESTIGATIONS: (S-2012,S-2013)
1.To establish
diagnosis:-
1. Clinical criteria
2. Acute phase reactants
(raised ESR&CRP)
3. Serological test (A-2014)
(RF&anti-CCP +ve), high-
ly specific: anti-CCP
4. Radiology:-(A-2014)
X-ray, USG, MRI
(osteopenia, narrowing of
space, erosion)
5. CBC:- anemia, thrombo-
cytopenia, eosinophilia
6. joint aspiration:- in-
crease turbidity, proteins,
polymorphs and decrease
viscosity, C3
2.To monitor disease 3. To monitor disease 4. To monitor drug
activity and drug effi- damage:- safety:-
cacy:-
1. pain:- visual ana- * X-ray * Urinalysis
logue scale
* Functional Assess- * Biochemistry (urea&
2. Early morning stiff- ment creatinine, LFT’s)
ness(>1 hr.)
* Hematology (CBC)
3. Joint tenderness
* Chest X-ray (CXR)
4. Joint swelling
5. DAS28 score( no. of
swollen and tender
joints) DAS-28 score
6. APR
7. Doppler usg and >5.1 = High activity
arthrogram for baker’s 2.6-5.1 = Moderate activity
cyst <2.6 = Remission
8. ESR and CRP

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

 D/D:- (A-2010,S-2012) UHS Q’s

1. Rheumatic fever
2. Osteoarthritis
3. Gouty Arthritis
4. SLE
5. Septic arthritis
1) A 42 yrs old house wife complaints of low grade fever & pain in proxi-
mal IPJs of both hands over the past 6 months. She had taken many pain
killers & antipyretics with some temporary relief.
A. 3 most likely diagnosis, B. 3 most imp investigations for this case,
C. 3 appropriate treatment steps (S-2012)
2) A 60 yrs old man with RA has Hb of 8g/dl. What are the possible causes
of this anemia? (S-2010) (Ans: Anemia of chronic disease, NSAIDS in-
duced GI blood loss, Hemolysis, Hypersplenism)

 Management:-(S-2012: Write three appropriate treatment steps)

*Pharmacological treatment:- *Non- *Surgical Treatment

(S-2013: Enlist drugs used in it) Pharmacological:-
1.On first diagnosis:- Physical and occu- 1.Synovectomy of wrist or finger
*prednisolone(30 mg, reducing pational therapy tendon sheath for pain relief
5mg every 2 wk. for 12 wk.)
*Methotrexate(15 mg weekly)
*Folic acid (5 mg weekly)
2. Patient fails to respond:- Self help and coping 2.Osteotomy: neurosurgery in pt.'s
Triple therapy strategies with Atlanto-axial joints involve-
(Methotrexate, sulfasalazine and ment
hydroxychloroquine)
3.If disease activity remains high 3.Arthrodesis:- excision of meta-
despite above treatment:- tarsal head in pt.'s with involve-
1st line:- TNF-inhibitors ment of MTP
(infliximab, adalimumab, etaner-
cept)
2nd line:- JAK-inhibitors 4.Arthroplasty:-
(Tofacitinib, boracitinib)

4.Transient flares:- Intra-articular 5. Joint replacement surgery.

glucocorticoids

Button-hole deformity
(Boutonniere)

Swan-neck deformity

ULNAR DEVIATION
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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

2. OSTEOARTHRITIS:-
• most common form of arthritis.

 Risk factors for Osteo-arthritis:-

Genetic Repetitive load- Developmental Adverse Obesity Trauma Hormonal Defi-

ing Abnormalities biomechanical ciency
*Skeletal dyspla- *Farmers *Dysplasia of hip *Ligament rup- *Estrogen defi-
sia *Miners *Slipped femoral ture ciency
*Polygenic in- *Elite athletes epiphysis *Paget’s disease *Aromatase in-
heritance hibitors
 Causes of early onset O.A. :-
• Mono-articular:- Previous trauma, localized instability
• Poly-articular:- 1.Juvenile idiopathic arthritis, 2. spondylo-epiphysial dysplasia, 3.Metabolic/endocrine
diseases: hemochromatosis, amyloidosis, onchronosis, 4.late avascular UHS Q’s:
necrosis, 5.Neuropathic joint, 6.Kashin-Beck disease 1) A woman in her mid 30’s started
having stiffness of both hands on
 Clinical Features:- waking up in the morning, followed
by swelling of small joints of hands.
• Most common joint to be affected is knee joint; 2nd is base of thumb A. Possible causes, B. How will u
• Oligo-articular asymmetric or mono-articular pattern investigate, C. Enlist drugs used in
• Pain and functional restriction are the main presenting symptom. this condition. (A-2013)
• Joint pain increased by exercise and relieved by rest. 2) A 42 yrs old female presented
• Morning stiffness is less than 15 min and brief getting after rest(<5 min) with c/o pain with swelling og small
joints of hands& feet for 3 months.
• Crepitation may be noted with movement of joint. Her lab shows: CBC: Hb 10g/dl,
• Bony swelling around joint margins. TLC 6200/cmm, platelets 165000/
• There are no systemic manifestation. cmm &ESR 75mm in 1st hr & CRP
• Defining features of O.A:- 1. degeneration of articular cartilage 96.
2. osteophyte formation A. Provisional diagnosis?, What
serological investigations u will ad-
3.subchondral osteosclerosis vise?, C. What radiological findings
4.remodeling of joint contours expected on X-ray? (A-2014)

Generalized osteoarthri- Knee O.A Hip O.A. Spine O.A.

tis
• polyarticular finger IP •
joint OA.
• Heberden ± Bouchard
nodes
• Peak onset: middle • Pain localized to anteri-
age
• First CMC joint; most
commonly involved. •
• Female preponder-
ance
• Involve other joints
like knee
O.A. commonly targets • targets superior as- • cervical + lum-
patella-femoral & me- pect of joint bar spine most
dial tibio-femoral com- • Hip pain usually commonly in-
partment maximal deep in an- volved
terior groin • Femoral stretch
or and medial aspect of • ON examination; test is +ve
knee. same as knee • Neurologic signs
On examination: antal- • Pain + restriction of are seen
gic gait, jerky, Varus or internal rotation with
valgum, quadriceps hip flexed.
weakness

• X-rays views for different types of osteoarthritis:-

Hip O.A. Knee O.A. Patello-femoral involvement Spine O.A.

Non-weight bearing PA of Standing AP X-ray Flexed skyline view Plain X-ray
pelvis

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

 Investigations:-
1. Diagnosis is clinical
2. ESR & CRP: normal
3. X-ray:- Osteophyte formations, unequal joint spaces
4. MRI
5. Routine biochemistry
6. CBC
7. Autoantibody tests are normal
8. Synovial fluid aspiration: viscous with low cell count

 Management:-

1. Education and Life style:-

• Full explanation of the condition
• Adverse mechanical factors should be addressed (e.g. Shock-absorbing foot wears, walking aids, weight
loss)
• Local strengthening exercises should be combined with aerobic exercises to maximize the benefit.
• Tuition in coping strategies like yoga, relaxation, maladaptive pain behavior, etc.
2. Pharmacological Treatment:-
A. Paracetamol and Topical NSAIDS and then capsaicin for knee and hand O.A.
B. Oral NSAIDS for patients who remain asymptomatic
C. Opiates may be required occasionally
D. Intra-articular injection of corticosteroids in knee & first CMC joint OA.
E. Intra-articular injection of hyaluronic acid
F. Nutraceuticals: Chondroitin sulfate and Glucosamine sulfate for knee O.A.
G. DMARD’s are not indicated nut reduce the progression and improve the symptoms.
3.Non-Pharmacological therapies:-
A. Acupuncture, B. Transcutaneous Nerve stimulation (TENS), C. Local physical therapies (Heat or cold)
4.Surgical Treatment:-
A. Osteotomy
B. Total joint replacement surgery is most common for
patients with O.A.
C. Cartilage repair

• Characteristic Distribution:-
Hips, Knee joint, PIP’s and DIP’s of hand, neck and lumbar spine.

 Sero-negative arthropathies:-
1. Ankylosing spondylitis
2. Reactive arthritis
3. Psoriatic arthritis
4. Entero-pathic arthropathy
UHS Q’s
1. A 32 yrs. old female complaints of early morning stiffness of small joints of hands &
feet for last 6 months. There are no urinary complaints but she has small nodules near
both elbows.
A. Probable diagnosis?, B. 4 specific investigations, C. 4 systemic complications of the
disease. (A-2007)
2. A 20 yrs. old lady presented with polyarthralgia & fever for 3 months duration. For
last 1 week, she is complaining of edema feet & shortness of breath. Examination reveals
B.P. 140/100 mmHg, pedal edema, raised JVP, heart examination normal, bilaterally
reduced breath sounds at the bases of lung, there is 2 finger breadth enlarged liver& asci-
tes present in abdomen.
A. What is D/D?, (Ans: RA with pericardial effusion, Rheumatic fever, SLE, RHF), Joints involvement in Osteoarthritis
B. Enumerate investigations fort this case. (A-2010)

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

3.Ankylosing spondylitis:- (male: female = 3:1)

 Clinical features  Extra-articular features(A-2017)

1. Insidious onset 1. Anterior uveitis & conjunctivitis

2. Recurrent episodes of low back pain, radiates 2. Cardiovascular disease:-
to buttock and posterior thigh and most *Aortic & mitral incompetence
marked early morning *Cardiac conduction defect
*Pericarditis
3. Marked stiffness of sacro-iliac joint 3. Prostatitis 80%
4. Spinal fracture in late stages of the disease 4. Amyloidosis

5. Restriction of movements 5. Upper lobe pulmonary fibrosis

6. Kyphosis 6. IBD
7. Pleuritic chest pain 7. Fatigue and anemia
8. Plantar fasciitis, Achilles tendonitis, En- 8. Osteoporosis
thesitis
 Investigations:- (A-2017)
1. Schober’s test
2. X-ray Sacroiliac joint shows:-
• irregularity, loss of cortical margins, widening of joint space, sclerosis, joint space narrowing and fusion,
• later; anterior squaring of vertebrae, Bridging syndesmophytes,
• In advanced disease: ossification of anterior longitudinal ligament and facet joint fusion seen (typical bam-
boo spine)
• Atlanto-axial dislocation in late stages
3. MRI: more sensitive for detection of early sacroiliitis
4. ESR and CRP: raised
5. RF and anti-CCP negative, ACP and ANA –ve
6. HLA-B27(Annual-2017); for pauci-articular JIA ( this HLA-B27 is responsible for transmission from parents
to children)
7. DXA scanning; for fragility fracture assessment
8. To assess disease activity:- BASDAI score, BASFI score, ADAS-CRP score, ASAS-H score
UHS Q’s
 Management:- 1) A 35 yrs old complaints of pain & stiffness
1. General Management:- of both hands especially in the morning.
Blood tests show Hb 12g/dl & ESR 65 mm in
* Education and appropriate physical activity 1st hr. Rheumatoid factor & ANA tests –ve.
* Back extension exercise daily A. Most likely diagnosis?, B. What treatment
* Morning warm-up routine may be indicated to relieve her symptoms?, C.
* Swimming is ideal exercise What treatment may be indicated to stop fur-
2. Pharmacological Treatment:- ther progression of the diseases? (S-2008)
* NSAIDS & analgesics 2) An 18 yrs. old boy presented with low
* Peripheral arthritis treated by methotrexate or sulfasalazine backache that radiates to buttocks & thighs &
* Anti-TNF therapy associated stiffness that persists for more than
* Local corticosteroid injection: for plantar fasciitis, enthesopa- 1/2 hr. Pain is worse at night & after inactivi-
thies, & peripheral arthritis ty, and relieved by activity.
A. Diagnosis?, B. 3 investigations?, C. 3 ex-
* Oral corticosteroids: for acute uveitis traarticular manifestations?, D. What is the
3. Surgical Treatment:- risk of transmission from parents to children?
* Total hip arthroplasty (A-2017)
* Spinal osteotomy

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

4. Systemic Sclerosis:- (SScl.)

• Autoimmune disorder of connective tissue resulting in fibrosis; affects skin, internal organs & vasculature.
• Peak age of onset:- 4th & 5th decade
• Female: male (4:1)
• Two types of SScl. :- 1. Diffuse cutaneous SScl (dcSScl) 30% , 2. Limited Cutaneous SScl (lcSScl) 70%
 Clinical findings/ Complications:- (S-2011,S-2016)
Skin • Non-pitting edema of fingers& flexor tendon sheaths; skin becomes shiny and taut
• Skin involvement restricted to site distal to elbow or knee classified as limited or
CREST syndrome
• Involvement proximal to knee and elbow and trunk classified as diffused.
Raynaud syndrome &
digital ulcer
Musculo-skeletal fea- Arthralgia, Morning stiffness, Flexor tenosynovitis, muscle weakening and wasting due to
tures myositis.
(Restricted hand function is due to the skin)
GIT Esophageal reflux, Erosive esophagitis, Dysphagia& Odynophagia, Water-melon stomach
(antral vascular ectasia), malabsorption, Pseudo-obstruction of large intestine
Pulmonary involve- Pulmonary Hypotension, Fibrosing Alveolitis
ment
Renal Complications Hypertensive renal crisis (one of the main cause of death), Malignant HTN, Renal failure

 Investigations:-
1. Serological tests:(S-2011)
• ANA positive, ESR raised, Raised IgG
• Anti– topoisomerase I antibodies, Anti-Scl 70 antibody, Anti-RNA polymerase antibody (dc-SScl)
• Anti-centromere antibodies (lc-SScl)
2. Other tests: RFT’s, LFT’s, urinalysis, CBC, Bone function tests, CXR, Transthoracic echo, lung function
tests, High resolution lung CT-scan, Barium swallow, Hydrogen breath test, Right heart catheter measurement
* Poor prognostic factors:- older age, proteinuria, high ESR, pulmonary HTN, diffuse skin disease, low factor TLCO

 Management:- Focus is to ameliorate the effects of disease on target organs; because no treatment is
available to reverse the fibrotic changes of the disease.
Complication Pharmacological treatment Life style modification
Raynaud phenomenon, calcino- *Ca-channel blockers *Avoid exposure to cold
sis& sclerodactyly *Intermittent infusion of prostacyclin *Wear heated mittens (gloves)
*Endothelin I antagonist (Bosentan) *stop smoking
*Antibiotics *Avoid sympathomimetic drugs
Esophageal reflux *Proton-pump inhibitors *Avoid foods that cause gas
*Anti-reflux agents *Avoid late night meals
*Antibiotics *Elevate the head-side of your bed
*Metoclopramide/domperidone
Hypertension/Renal Complica- *ACE-inhibitors *Stop smoking & Low fat diet
tion (S-2016) *ARBs *Reduce alcohol & salt intake.
*Restrict protein diet.
*Avoid nephrotoxic drugs
Joint involvement *Analgesics *Exercise
*NSAIDS *Reduce weight, if over-weight
*Methotrexate (in-case of synovitis)
Pulmonary Hypertension *Bosentan *Surgery:
*Corticosteroids & Cytotoxic drugs Heart-Lung Transplantation
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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

5.Systemic Lupus Erythematosus(SLE):-(S-2007, A-2018, S-2018)

• 90% patients are females
• Peak age of onset: 20-30 years
• Renal disease and cutaneous manifestations are more frequent in juvenile onset SLE.
• Non-scaring alopecia occurs with active disease.
 Revised American Rheumatism Association criteria:- (A-2018, S-2018)(4 out of 11 features at-least serial-
ly or simultaneously) *mnemonic: SOAP BRAIN MD
Serositis Pleuritis or pericarditis (most common cardiac manifestation) , myocar-
ditis and LSE also occurs.
Oral ulcers Oral or nasopharyngeal ulceration, which may be painless
Arthritis (90% of patients) Non-erosive, involving two or more peripheral joints (Jaccoud’s ar-
thropathy)
Photosensitivity Rash due to unusual reaction to sunlight
Blood (hematological disorder) Hemolytic anemia or leucopenia, lymphopenia, thrombocytopenia
Renal Disorder Persistent proteinuria >0.5 g/day or cellular casts
Anti-nuclear antibody Abnormal titer of ANA by immunofluorescence
Immunological disorder Anti-DNA antibodies in abnormal titer or presence of antibody to Sm
antigen or +ve Anti-phospholipid antibodies
Neurological disorder Seizures or psychosis, in the absence of provoking drugs or metabolic
derangement
Malar rash Butterfly shaped, fixed erythema, flat or raised, sparing the naso-labial
fold
Discoid rash Erythematous raised patches with adherent keratotic sparing and follic-
ular plugging

 Investigations:- (S-2007, A-2018, S-2018)

A. Blood: normochromic normocytic anemia, leucopenia, lymphocytopenia, lymphopenia
B. ESR is raised but CRP is normal except in serositis
C. Immunological findings:- ANA(95-100%), Anti-dsDNA(60%), Anti-sm antibodies (30%), Antibodies to
ENA and complement, Anti-dRNP (30%), Anti-phospholipid antibodies, RA factor(20%), LE cells(70%)
D. Serum Complement:- low in active disease
E. Urinalysis: proteinuria, hematuria & red cell casts or granular casts.
F. Serum creatinine to assess the renal status
G. Histology: deposition of complement

 Management:-
A. General management:-
* Educate the patient about the nature of disease and control symptoms and damage.
* Avoid sun and UV light exposure and employ sun blocks (photosensitivity)
* Avoid smoking
* Control hypertension and hyperlipidemia
B. Specific Treatment:-
• Mild disease:- Analgesics/NSAIDS, Increased dose of glucocorticoids for flares, Hydroxychloroquine,
Monoclonal antibodies like belimumab, short course of oral steroids with immunosuppressants
• Severe & Life threatening:- (for renal, CNS and cardiac involvement) - (A-2018)
1. High dose of methotrexate or MMF + Steroid (pulsed methylprednisolone 500 mg– 1g i/v) +
immunosuppressive (cyclophosphamide 15 mg/kg i/v) : repeated at 2-3 week interval on 6-8 occasions.
2. Maintenance therapy (following control of acute episode):
*Oral prednisolone,

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

*Azathioprine, methotrexate or MMF

*CVS risk factors should be controlled & should stop smoking
• APS– life long warfarin
• For renal disease:- MMF (mycophenolate mofetil) +high dose steroids, pharesis
• For pneumocystis pneumonia:- Co-trimoxazole
• Hemorrhagic cystitis:- mesna is given with bolus cyclophosphamide, plus good hydration
• Rash:- Hydroxychloroquine, sun-screen, topical steroid retinoids.

6.Septic Arthritis:-  Risk factors & etiology:-

(most rapid & destructive joint disease) • Increasing age
• Pre-existing joint disease
• Mode of spread:- (especially RA)= skin is portal of
1. hematogenous spread from either skin or upper respiratory tract entry– (mcq uhs)
2. Infections from direct puncture wounds or secondary to joint
aspirations. • Diabetes melitus & RA (Staph.
Aureus most likely)= in adults
• Clinical features:- • Immunosuppression by drugs an
disease
1. Acute & subacute mono-arthritis & fever.
2. Joint is swollen, hot & red; is held in loose pack position. • I/V drug abuse (gram –ve bacilli
3. Effusion, rest pain & stress pain on movement. or group B,C & G streptococcus)
4. Knee & hip joints are commonly targeted. = elderly
• Young sexually active adults
• Investigations:-(A-2011) (disseminated gonococcal infec-
tion)
1. Aspiration of joint: Synovial fluid turbid & blood stained. • s/s: migratory arthralgia, low
Gram staining =ve 50%, Culture of synovial fluid 90% +ve. Use grade fever, tenosynovitis & pap-
imaging, if needed (hip). ular skin rash.
2. CBC: Leukocytosis
3. CRP & ESR: Raised • Differential Diagnosis:-
4. LFT’s & RFT’s
5. Genital tract swab: culture (+ve in 70-90% cases) 1. Lyme disease (conformed by bor-
6. Plain X-ray of joint relia serology): monoarthritis +
7. Serum uric acid level to rule-out gout. headache
2. Gout
• Management:- 3. Pseudo-gout
4. Reactive arthritis
(A-2011)
1. Admit patient to hospital & perform urgent investigations.
2. Relieve pain: UHS Q’s
a. oral or i/v analgesics, b. consider local ice packs 1. A 50 yrs. Old lady on 2nd post-operative
3. Antibiotics: day following hysterectomy developed acute
a. 1st choice: Flucloxacillin (2g i/v 6 hrly) for 2-3 wks. followed pain in left knee joint. On examination, left
by oral treatment for 6 wks. in total. knee was hot, swollen & tender.
b. If penicillin allergic: Clindamycin(450-600 mg) 6 hrly or if A. 3 D/D’s?,
B. 3 imp. Investigations?
methicillin resistant then: Vancomycin i/v BD(OD if age >65)
c. If high risk of gram –ve sepsis: i/v gentamicin (5mg/kg OD) ,
i/v vancomycin (50-100mg/kg OD)
4. Adequate drainage/Joint decompression:
a. Joint aspiration, b. Lavage or Arthroscopy, c. Surgical drainage
5. Physiotherapy/Early active rehabilitation:
a. Early regular passive movements, b. Active movements, once pain controlled & no effusion.

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

7. Gout:-
 Factors that predispose to Chronic hyperuricemia and Gout:-
Diminished Renal excretion Increased production of uric acid Increased in-
(common) take
• Inherited isolated renal tubular • Increase purine turn-over: Chronic myeloprolifera- • Game
defect (under-excretors) tive, e.g. Polycythemia & CLL. • Sea food
• Renal failure • Increased de-novo synthesis (over producer) specific • Offal
• Chronic drug therapy: Thiazide & enzyme defect: • Red meat
Loop diuretics (furosemide), Low 1. Hypoxanthine guanine phosphoribosyl transferase
dose aspirin, Ciclosporin, Pyra- deficiency
zinamide (Model paper & Supp- 2. Phosphoribosyl pyrophosphate synthetase deficiency
2014 seq) 3. Glucose 6-Phosphate deficiency
• Lead toxicity (e.g. In moonshine 4. Psoriasis
drinkers) 5. High fructose intake
• Lactic acidosis (alcohol) 6. Glycogen storage disease

 Clinical features:-
 Acute gout:
A. 1st MTP joint is effected in 50% cases– podagra (seizing the foot)
B. In decreasing frequency: Ankle→midfoot→knee→Hand→wrist→elbow
C. Extremely rapid onset: maximum severity in 2-6 hrs., often early morning.
D. Severe pain; worst ever pain Differential Diagnosis
E. Extreme tenderness: unable to wear socks or to let bedding rest on joint.
F. Marked swelling with overlying red, shiny skin 1. Septic arthritis
G. Self-limiting: over 5-14 days 2. Pseudogout
 Recurrent & Chronic Gout: 3. Infective cellulitis
A. 2nd attack occurs within 1 year (Supp-2014 seq)
B. Continued MSU deposition causes joint damage and chronic pain.
C. Main determinant is SUA; higher it is, the earlier and most extensive damage will be.
 Chronic tophaceous Gout:
A. Large MSU crystals deposit produce irregular firm nodules (tophi) around extensor surfaces of fin-
gers, hands, forearm and elbow.
B. White color of MSU crystals may be evident.
C. Large nodules may ulcerate, discharging white gritty material.
D. Tophus with white MSU crystals visible beneath the skin: diuretics induced gout in pts. with pre-
existing nodal OA.
 Renal and urinary tract manifestations:-
A. Uric acid stones (* not MSU) cause renal colic
B. Progressive renal disease: important complications of untreated severe tophaceous gout. This results
from MSU crystals deposition in the interstitium of medulla and pyramids with consequent chronic inflamma-
tion, giant cell reaction, fibrosis, glomerulosclerosis and secondary pyelonephritis.

 Investigations:- (A-2012: Enumerate 4 most imp. Investigation)

 Joint Aspiration: definitive diagnosis by identification of MSU crystals. In Acute gout: Synovial fluid
shows increased turbidity due to greatly elevated cell count. (90% neutrophils); whereas in Chronic gout:
fluid is more variable but occasionally white due to high crystal load.
 Serum uric acid levels
3. Elevated CRP and neutrophils: usual in acute phase
4. 24 hrs urinary uric acid excretion on low purine diet; identify over production
5. RFT’s , serum creatinine, & urine analysis.
6. Hypertension, Blood glucose & serum lipid profile.
7. CBC & ESR detect chronic myeloproliferative disorders.
8. ESR, often modestly raised in tophaceous gout, but chronic elevation.
9. X-rays: Punched-out defects affecting 1st MTP joint in chronic gout. Normal in acute gout.

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

10. Ultrasound: Detects subclinical micro-tophi and MSU deposits.

 Management:-
 Acute Attack:- (A-2012 , Model paper)
) Oral NSAIDS (plus PPI) is standard treatment with local ice packs.
) Oral Colchicine (1st choice) is effective; often cause vomiting & severe diarrhea, so low dose (0.5 mg 6-12
hrly)
) Joint aspiration; gives instant relief.
) Intra-articular steroid injection prevents reaccumulation.
) Bed rest.
) I/M or oral corticosteroids used in elderly patients. (oral prednisolone or I/M dexamethasone)
 Pts. who have had a single attack of
 Long-Term Treatment:- gout, don’t need urate lowering thera-
A. General Treatment: py; but individuals with >1 acute at-
i. Weight loss tack within 12 months & those with
ii. Predisposing factors should be corrected. complications should be offered.
iii. Reduction of excessive alcohol.
iv. Purine diet (sea food, red meat, offal) should be tempered.
v. Diuretics should be stopped & losartan should be substituted for HTN.
B. Specific Treatment:
I) Urate Lowering Therapy (ULT): Allopurinol 100mg/day is drug of 1st choice.
• Indications of ULT :-
 Recurrent attacks of acute gout. • Aim of ULT is to bring the SUA be-
 Tophi low the therapeutic target of 360
 Evidence of bone or joint damage µmol/l .
 Gout with greatly elevated serum uric acid. • Acute flares follow ULT, so NSAIDS
II) Febuxostat (xanthine oxidase inhibitor): when allopurinol is & Colchicine for first few months re
C/I. ( No dose adjustment required for renal disease). Starting given for it.
dose 80mg/daily + Colchicine or NSAIDS for initial 6 months.
III) Uricosuric drugs: A. Probenecid or Sulfinpyrazone: requires several doses. ( C/I in renal impairment,
over-producers & urolithiasis), B. Benzbromarone:(50-200mg daily) effective and safe in mild to moderate
renal impairment.
IV) Asymptomatic Hyperuricemia: not usually treated unless plasma levels are very high or in pts. with can-
cer to prevent tumor lysis syndrome.
V) Pegloticase is biologic treatment, if standard therapy fails.

 Counselling:-
) Initiation of ULT can partially dissolve MSU crystals and trigger attacks, so pts. should be warned of this
and told to continue ULT.
) Very high purine diet should be tempered like sea food, red meat, offal.
 Follow-up:-
a) Serum uric acid should be measured every 3-4 wks. And the dose of allopurinol is increased in 100mg in-
crements until this reaches 900mg/day.
b) Annual monitoring is advised subsequently to treatment effectively.

8. Reactive Arthritis:-
 Reactive arthritis is a reaction to a number of bacterial triggers with C/F in keeping with all SpA condi-
tions.
 The arthritis in RF is also an example of it, not associated with HLA-B27. Conjunctivitis
 Reiter’s disease: (Classical triad) :-
 16-35 years of age, mostly involved.
Reactive
 Onset is acute or subacute with inflammatory oligoarthritis. Urethritis Arthritis
 Asymmetrical & targets lower limb joints: ankle, MTJ, knee joint .
 Fever, wt. loss, lower back pain& stiffness, Achilles insertional Enthesitis & plantar fasciitis.

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

• Precipitating factors:-
SpA related triggers Non-SpA related ReA (reaction is myoarthralgias)

• Bacterial dysentery, by Salmonella, Shigella, Campylo- • Viruses

bacter or yersinia. • Mycoplasma
• Sexually acquired infections with Chlamydia. • Borrelia
• Streptococci
• M. leprae (Hansen disease)

• Complications/Extra-articular features:-
1. Circinate Balanitis(20-50%)→vesicles on coronal margin of prepuce& glans penis, which later rupture.
2. Keratoderma blennorrhgica(15%)→waxy yellow-brown vesico-papules with desquamating margins on
palms and soles of feet.
3. Nail dystrophy & Pustular psoriasis.
4. Buccal erosions(10%) & Mouth ulcers.
5. Conjunctivitis & uveitis.
6. Aortic incompetence, Conduction defects & pleuro-pericarditis.
7. Peripheral neuropathy, seizures & meningoencephalitis.

• Investigations:-
1. Joint Aspiration: to exclude crystal arthritis and infection. Synovial fluid is inflammatory & contains giant
macrophages (Reiter’s cells)
2. X-ray: a. Proliferative erosions at enthesis
b. Periostitis of metatarsal, phalanges & pelvis.
c. Coarse, asymmetrical & non-marginal syndesmophytes
d. Fluffy calcaneal spurs.
3. ESR & CRP: may be raised.
4. Urethritis: conformed by two glass test (mucoid threads in 1st void specimen & clear in 2nd.
5. High vaginal swab: may reveal Chlamydia o culture.
6. Serum agglutinin: Conform previous dysentery.
7. Stool Culture: only +ve in post salmonella arthritis.
8. RF, CCP & ANA -ve

• Management:-
1. Acute attack: a) Bed rest, b) oral NSAIDS, c) Analgesics & d) Intra-articular injection of corticosteroids
for mono-articular disease and systemic corticosteroids for poly-articular disease.
2. Chlamydial Urethritis: Short course of doxycycline or single dose of azithromycin.
3. DMARD’s : for persistent symptoms, recurrent arthritis & keratoderma.
4. Topical or subconjunctival corticosteroids: anterior uveitis (emergency).
5. Anti-TNF therapy: for DMARD’s recalcitrant cases.

9.Psoriatic Arthritis:-
• Clinical features:-
 Pain with stiffness, joints not swollen. Different disease patterns are as follows:-
1. Asymmetrical inflammatory Oligoarthritis (40%): involves hand & feet.
(Tenosynovitis, Enthesitis, sausage digit or dactylitis.)
2. Symmetrical polyarthritis (25%): Resembling RA; in women, nodular & extraarticular features of RA ab-
sent.
3. Distal IPJ arthritis: quite common pattern associated with psoriatic nail disease.
4. Psoriatic spondylitis: Resembling to AS.
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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

5. Arthritis Mutilans: Bone destruction (Targets fingers and toes) ; the en- CASPAR criteria for
casing skin appears invaginated & telescoped (main en lorgnette), & fingers psoriatic arthritis
can be pulled back to its original length.
6. Nail dystrophy: Pitting, oncholysis, sub-ungal hyperkeratosis & ridges.  Inflammatory articular dis-
7. Psoriatic rash: On scalp, natal cleft & umbilicus. ease (joint, spine or enthesis)
with ≥3 points form the fol-
lowing:-
• Investigations:- 1. Current Psoriasis
1. X-ray: shows “pencil in cup” deformity in IPJ, other changes are same 2. Hx of psoriasis in 1st OR 2nd
as RA, ivory phalanx. X-ray of axial skeleton is same as in Reiter’s ar- degree relatives.
thritis. 3. Psoriatic nail dystrophy
2. ESR & CRP: may be raised, but ESR may be normal. 4. -ve IgM rheumatoid factor.
3. RF, CCP & ANA _ve. 5. Current dactylitis.
4. MRI & USG with power doppler: to detect synovial inflammation & in- 6. Hx of dactylitis.
flammation of enthesis. 7. Juxtra-articular new bone
disease.
• Manangement:-
1. Exercise
2. NSAIDS & analgesics.
3. Intra-articular injection of corticosteroids (trimcilone)→for isolated synovitis.
4. DMARD’s (methotrexate is drug of choice); hydroxychloroquine is not given as it causes exfoliated skin
reactions→For persistent pts. , not responsive to above treatment.
5. Anti-TNF therapy→for active synovitis & inadequate response to DMARD’s.
6. Retinoid acitretin: effective for skin lesion.
7. Photo-chemotherapy with methoxy-psoralen & lomg wave UV light (psoralen+ UVA=PUVA)→for skin
disease
8. Ustekinumab & secukinumab can also be used.

10. Antiphospholipid Antibody Syndrome:-

• A hypercoagulable state associated with a group of antibodies that are directed against phospholipids or
cardiolipins.
• Anti-phospholipid antibodies cause: Coagulation defect, Livedo reticularis, Thrombocytopenia, Syphilis
tests (RPR or VDRL) false +ve, elevated PTT.
• Clinically, it presents with spontaneous abortion in otherwise healthy women or thromboembolism in oth-
er pts. Two 1st trimester abortions suggests antiphospholipid antibodies.
• Investigations: Mixing study and RVVT
• Treatment: Low dose aspirin or warfarin, if recurrent thrombosis.

11. Drug-induced Lupus: a side-effect of certain medicines.

• Most common drugs causing it are remembered by a mnemonic: *Drug-induced Lupus:
(High Quality Vice Chancellor GLIMPSE). 1. Hydralazine
• Symptoms typically include arthralgia, fatigue, fever & pleurisy (rare). 2. Quinidine
• Acute onset SLE is usually not confused with Drug-induced lupus , due to the 3. Valproate
lack of skin disease, kidney disease & milder symptoms seen in latter. Also, pho- 4. Griseofulvin
5. Lovastatin
tosensitivity, hair loss &CNS changes not seen in drug-induced lupus. 6. Isoniazid
• Investigations: Anti-histone antibody 95% ( Cases due to hydralazine have on- 7. Methyl DOPA
ly 35% +ve anti-histone antibodies) 8. Phenytoin
• Treatment: Suspected medication is stopped s/s resolve in 1-2 wks. 9. Sulfasalazine
10.Ethosuximide
12. Sjogren Syndrome:-
• Chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, resulting in
xerostomia & dry eyes.
• May be seen alone (primary) or with other autoimmune diseases (secondary) like RA, PBS or SE.
• Clinical features: 1) Itchy eyes (sandy feeling under the eyes due to reduced lacrimal production &
destruction of corneal epithelium– keratoconjunctivitis sicca, 2) Difficulty swallowing food,

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

3) Possible increase in dental carries, 4) possible parotid enlargement.

• Investigations:-
1. Shirmer’s test
2. Anti-Ro (SSA) & Anti-La (SSB) will be positive.
3. Biopsy of salivary glands will show lymphocytic infiltration.
• Treatment:- is symptomatic only.
1. Use artificial tears, Sugar-free gum, Water the mouth.
2. Pilocarpine.
3. Cevimiline.

13. Enteropathic arthritis/arthropathy: (Ulcerative colitis & Crohn’s disease)

• Pts. May develop characteristic skin lesions, pyoderma gangreosum & erythema nodusum.

14. Pseudo-Gout:-
• CPPD crystal deposition is more common with in elderly and with pre-existing joint disease.
• Remember 4H’s: Hyperparathyroidism, Hemochromatosis, Hypophosphatemia, Hypomagnesemia.
• Clinical features: 1) possible acute presentation like gout or possible asymptomatic & chronic form,
2)Knee is the most affected joint; other joint involved are wrist, shoulder & ankle.
• Investigations:1)Joint aspiration: typical rectangular,rhomboid, +ve birefringent crystals on synovial flu-
id evaluation,2)X-ray: Linear radio-dense deposits in joint menisci or articular cartilage(chondrocalcinosis)
• Treatment: Same treatment as of gout. Low dose of colchicine may be considered to prevent recurrences.

15. VASCULITIS SYNDROMES:-

• Vasculitis is an inflammatory process involving the blood vessels, resulting in a decrease of the
lumen diameter and eventual ischemia of the tissues supplied.
• Different types are as follows:-

15(A). Wegener Granulomatosis:-

• small vessel vasculitis.
• Clinical features: Typically affects the respiratory tract (sinuses, nose, trachea, and lungs) and kidneys,
but can involve any organ system. The most common sign of Wegener granulomatosis is involvement of
the upper respiratory tract. Symptoms include rhinitis, sinusitis, and, rarely, nasal ulcers.
1. A common sign of the disease is chronic rhinitis that does not respond to usual treatment and that be-
comes increasingly worse.
2. Despite lack of symptoms, lungs are affected in most people; they include cough, hemoptysis, and dyspnea.
3. Kidney involvement (>80% of patients) (major cause of morbidity and mortality)
4. Arthritis (60% of patients)
• Investigations: 1) Presence of antineutrophil cytoplasmic antibodies (C-ANCA)-not a diagnostic test.
2)Diagnostic test: Biopsy of an involved organ demonstrating the presence of vasculitis and granulomas.
• Treatment: Glucocorticoid plus an immunosuppressive agent (cyclophosphamide).

15(B). Henoch-Shonlein Purpura:-

• small vessel vasculitis caused by immune complex deposition.
• Clinical features: Purpura over legs& buttocks, abdominal pain, GI bleed, arthralgias & nephritis.
• Investigations: Biopsy shows IgA deposits in vessel wall.
• Treatment: Glucocorticoids & immunosuppressive drugs.

15(C). Behcet’s disease:-

• Target small arteries & venules. (HLA-B51 association)
• Clinical features: oral ulcers, erythema nodusum, acneiform lesions, ocular (anterior& posterior uveitis,
retinal vasculitis) & neurologic involvement.
• Investigations: 1) Pathergy test, 2) On clinical ground.
• Treatment: Glucocorticoids, colchicine, thalidomide.

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

15(D). Polyarteritis Nodosa:-(multisystem disease)

• Clinical features:
A. Non-specific Complaints:- fever, malaise, weight loss, anorexia, and abdominal pain.
B. Specific Complaints: The disease can affect nearly any site in the body, except the lungs. It has a predispo-
sition for organs such as the skin (palpable purpura, most common), kidney, nerves, and GI tract.
1. Peripheral neuropathies are very common: tingling, numbness, and/or pain in the hands, arms, feet, and
legs, and mononeuritis (e.g., foot drop).
2. GI manifestations are common: abdominal pain and GI bleed (occasionally mistaken for IBD).
3. Active hepatitis B infection is seen in a minority of patients.
• Investigations: 1)Biopsy of involved organs will show pathologic changes in medium-size arteries.
2) Angiogram of the abdominal vessels may also be helpful for diagnosing PAN, since aneurysms
affecting the arteries of the kidneys and/or GI tract are found.
• Treatment: high doses of corticosteroids and immunosuppressive drugs (cyclophosphamide).

15(E). Churg-Strauss Syndrome:-

• involves the small- and medium-sized arteries. Any organ can be involved. To remember Churg-Strauss
• Clinical features: syndrome, think of it as
1) The cardinal manifestations are asthma, eosinophilia, and lung involvement. PAN in asthmatic patient.
2) The typical patient is middle-aged, with new-onset asthma. Asthma symptoms
may begin long
before the onset of vasculitis.
3) Other symptoms include mononeuropathy, transient pulmonary infiltrates on chest x-ray, paranasal sinus
abnormalities, nasal polyps, and allergic rhinitis.
• Investigation: Biopsy.
• Treatment: combination of prednisone and cytotoxic agent.

15(F). Temporal Arteritis:-(also known as giant cell arteritis)

• Affects the large arteries that supply head, eyes, and optic nerves. New-onset headache in any patient age
>50 prompts consideration of this diagnosis, which if left untreated may result in permanent vision loss.
• Symptoms include: * Commonly associated with
• Constitutional symptoms like fever, wt. loss, night sweats are common. Polymyalgia rheumatica, which
• Headache and pain in one or both temples (most common symptoms). presents with:
• Scalp tenderness (pain when combing hair). 1.Symmetrical immobility, asso-
• Jaw claudication (jaw pain when chewing). ciated neck &shoulder girdle
• Decreased vision or blurry vision (amaurosis). pain.
• Tongue numbness. 2.Stiffness.
• Sudden loss of vision (rare). 3.Age of onset: 70 years
• Investigations: 4.male:female = 3:1
1) Erythrocyte sedimentation test (ESR) (100% sensitive). Therefore, the first test to do when TA is suspected
is ESR.
2) Diagnostic test: Biopsy of the temporal arteries, which will demonstrate the characteristic giant cells.
3) USG of temporal arteries (halo sign)
4) 19FDG PET-scan
• Treatment: 1)When TA is suspected and ESR is elevated, start corticosteroids immediately, before the
temporal artery biopsy is performed. Do not withhold treatment waiting for the biopsy to be done. Use
higher dose in TA than PMR.

16. INFLAMMATORY MYOPATHIES

• Inflammatory muscle diseases that present with progressive muscle weakness.
• Include polymyositis, dermatomyositis, and inclusion body myositis.
• Patients have difficulty with tasks involving the proximal muscles: lifting objects, combing hair, getting up
from chair.
• Fine-motor tasks involving the distal muscles, e.g., writing, are affected only late in the disease.
• Ocular muscles are never involved (this feature differentiates the inflammatory myopathies from myas-
thenia gravis and Eaton-Lambert syndrome).
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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

16(A). Dermatomyositis:- (Associated with cancer in 25% cases {mcq-uhs})

• Clinical features:
1. Skin involvement; the heliotrope rash is a purple-lilac discoloration of the face, eyelids, and sun-exposed
areas of the body.
2. Gottron’s papules are the scaly lesions seen sometimes over the knuckles.
• Laboratory Findings: 1) Elevated muscle enzymes (creatine phosphokinase (CPK), and aldolase),
(sometimes up to 50-fold).These are most sensitive tests to perform in pts. suspected of an inflammatory
myopathy. 2) Autoantibodies (anti-Jo-1) occur in patients with inflammatory myopathies, supporting a
possible autoimmune origin.
• Diagnosis: 1) Electromyography (EMG) shows evidence of myopathic potentials characterized by
short-duration, low-amplitude units. 2) Diagnosis is confirmed by muscle biopsy.
• Treatment: 1) For polymyositis and dermatomyositis, steroids are useful.
2) Inclusion body myositis is resistant to immunosuppressive therapy.
3) Hydroxychloroquine or tacrolimus for skin disease.

17. Disease Modifying Anti-Rheumatic drugs (DMARDs):-

• Best initial drug is methotrexate (MTX), If MTX doesn’t control disease, anti-TNF * Methotrexate is a
medication is added to treatment. drug of choice in:
• Hydroxychloroquine & sulfasalazine are used in early, mild disease. (mnemonic: CANCER)
• Steroids are used briefly to control disease while waiting for methotrexate to work.
• Biologic agents: includes TNF inhibitors: Infliximab, Adalimumab & Etanercept. 1. Choriocarcinoma
• MTX: is an anti-metabolite, stops cell in S-phase & causes folic acid deficiency. 2. Autoimmune disease
3. Non-hodgkins lym-
• MTX toxicity:- (mnemonic: METHO): Mouth ulcers, End of WBC’s (leucopenia), phoma
Tiredness (fatigue), Hepatotoxicity, fibrOsis of liver, Flare the rheumatoid nodules; 4. Crohn’s disease
so CBC & LFT’s are performed every 4-8 wks. 5. Ectopic pregnancy
• Rx of MTX toxicity: Folinic acid tablets (leucovorin, citrovorin) 6. Rheumatoid arthritis
• Hydroxychloroquine causes retinopathy, so regular eye examination is required.
• HydroxyChloroquine is a safer drug in pregnancy. (mcq-uhs)

18. Fibromyalgia:- ( in young woman)

• Chronic musculoskeletal pain & tenderness with trigger points of focal tenderness at:
a) trapezius, b) medial fat pad of knee & c) lateral epicondyle.
• Pain occurs at many sites (neck, shoulder, back & hips) & is associated UHS Q’s
with: 1. A 30 yrs. Old female present-
1) Stiffness, numbness & fatigue, 2) Headache, 3) Sleep disorder ed with symmetric pallor & cya-
• Investigations:- nosis of hand figers ppted by
1. No tests are there to conform it. cold & improved by warmth.
(S-2017-18)
2. Diagnosis is based on complex S/S with trigger points at predictable points. A. What is this condition?, B.
3. All lab tests, like ESR, CRP, RA, CPK, are normal. treatment?, C. What is the prog-
• Treatment:- nosis? (Ans: Good)
1. Amitriptyline (best initial treatment)
2. Milnacipran
3. Pregabalin Primary Raynaud phenome-
4. Trigger point injections with local anesthetics. non
• Up to 5% of general popu-
19. Raynaud Phenomenon:- lation
• Diagnosis: Secondary Raynaud Phenom-
1.Examination of capillary nail fold using ophthalmoscope (and oil placed on enon
nail) • Age at onset of over 25 yrs.
2.Capillary fall out, 3. Dilatation & blanching of loop • Absence of family hx.
• Treatment:- ( see page 07 ) + Intermittent infusion of epoprostenol in digital • males
ischemia, high dose antibiotics.

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Medicine Notes by Chillers (Q45, QAMC-Bahawalpur) Rheumatology

• Additional Topics:-
1. Osteoporosis:-
• Causes:
1. Post-menopausal osteoporosis
2. Endocrine diseases: hypogonadism, hyperthyroidism, Cushing's disease, hyperparathyroidism.
3. Inflammatory disease: IBD, AS, RA
4. Drugs: Corticosteroids, GnRH agonists, Aromatase inhibitors, Heparin, etc.
5. GIT diseases: Malabsorption, CLD
6. Miscellaneous: Myeloma, Gaucher’s disease, Homocystinuria, Highly trained athletes, HIV infections, etc.
• Investigations:
1. DEXA (Dual energy X-ray absorptiometry) scan- (mcq-uhs)
2. Bone mineral density (BMD): a) Osteoporosis: T-score ≤ -2.5, b) Osteopenia: T-score b/w –1 to –2.5
3. Investigations of cause: RFTs, TFTs, LFTs, Immunoglobulins & ESR, TTG antibodies, Serum 25(OH)D,
PTH level, Hypercalcemia, sex hormones & gonadotrophins level.
4. Trans-iliac bone biopsy
• Treatment:-
1. Life style advice & falls
2. Drugs Treatment:
i) Bisphosphonates
ii) Denosumab
iii) Strontium ranelate
iv) HRT: Raloxifene or Tibolone
v) Calcium/Vit. D
vi) Parathyroid hormone
3. Suregry.

2. Paget’s Disease:-
• Clinical features: bone pain & increase warmth over the affected area.
• Investigations: 1) Isolated ALP↑, 2) X-ray: bone expansion with alternating radio-lucency &osteosclerosis.
• Treatment: 1) Bisphosphonates, 2) Inhibitors of bone resorption (Etidronate), 3) Calcitonin.

Diagnosis Calcium Phosphate PTH ALP Ca+2

1 Hyperparathyroidism ↑ or N ↓ or N ↑ or N N or ↑ ↑ ( or N)

2 Hyperparathyroidism ↓ or N ↑ or N ↑ ↑ or N N

3 Hyperparathyroidism ↑ or N ↑ or N ↑ ↑ or N ↑

4. Rickets& Osteomalacia ↓ or N ↓ or N ↑ (or N) ↑ N ( or ↓)

(Deficient intake)
5. Renal Failure ↓ or N ↓ or N ↑ ↑ N

6. Fanconi’s syndrome ↓ or N ↓ or N N ↑ N

7. Osteoporosis N N N N N

8. Paget’s disease N (or ↑) N N ↑ N

19