RHE General

History  Basic RHI hx

1. Characterize:
a) Site (mono/oligo/poly, symmetrical), onset (max intensity, after trauma/wound), duration,
distribution, progression
b) Inflammatory vs mechanical (3): morning stiffness, gets better with activity, constitutional sx
(LOW, LOA, fever, fatigue)
2. Extra-articular manifestations: skin (ulcers, rash, alopecia), eye (pain, redness,
photosensitivity), respi, renal CNS
3. Function: bADL, occupation, hand dominance
4. Meds and compliance to meds (pred, NSAIDs)
5. PMHx: mental disorder, DM, PUD, pTB
6. Social hx

 Ank spond/LBP hx
1. Characterize back pain
a) Site, onset, duration, any preceding trauma
b) Inflammatory: morning stiffness, worse in morning and improves w exercise, constitutional
sx
c) Progressive limitation of motion?
2. Extra-articular complications :
a) Eyes: anterior uveitis/irits
b) Heart: AR< AV block
c) Lung: apical fibrosis
d) Renal (frothy urine): amyloidosis
e) Foot: achilles tendonitis, plantar fasciits
3. Rule out other seronegative arthropathies:
a) Reactive arthritis/reiters: urethral discharge, urinary sx, sexual hx
b) Enteropathic (IBD): bowel sx esp bloody diarrhoea, pyoderma gangrenosum
c) Psoriatic arthritis: nail changes, salmon pink plaques
d) JIA: onset before 16 years old
O/E  Look:
1. Face: scleroderma, Cushings, thyroid eye signs
2. Hands (expose/remove brace):
a) Back: tophi or heberdens nodes, Boutonniere deformity of PIPJ/swan neck deformity of DIPJ,
ulnar deviation and/or volar subluxation of MCPJ, dorsal guttering indicating wasting of intrinsic
muscle, any joint swelling/deformity
b) Nails: clubbing, onycholysis, pitting
c) Palm (erythema), thenar/hypothenar eminence
d) Elbow: rheumatoid nodule, tophi or psoriasis rash

 Feel: synovitis (loss of joint space) over DIPJ and PIPJ + MCPJ, squeeze wrist, bogginess +/-
tinels

 Move (4): grip, pincer, coarse (turn doorknob), fine (cap pen, transfer coins)

 Offer:
1. Extra-articular: lungs, PHTN, proximal myopathy +/- skin, eye, CVS, abdo
2. BP, urine dipstick
RA
O/E  Summary: note presence of symmetrical deforming polyarthropathy or synovitis of the wrist
and MCPJ (more common in OCSE now)!
 Look:
1. Fingers:
a) Boutonniere deformity of PIPJ, swan neck deformity of DIPJ
b) Dropped fingers from tendon rupture (ddx PIN lesion of radial nerve)
c) Intrinsic muscle wasting
d) MCPJ: ulnar deviation, volar subluxation, synovitis
e) Thumb: swelling, subluxation, Z deformity
2. Nails: vasculitic lesions, nail fold infarcts but no nail changes or skin lesions of psoriasis
3. Palm: palmar erythema, thenar wasting from CTS
4. Elbow: rheumatoid nodules 25%, swelling, deformity
 Feel: Synovitis of MCPJ and wrist
 Move: grip, pincer, coarse, fine
Diagnosis  Diagnostic criteria (Eular criteria): 6 or more points = RA, duration 6 weeks or more!
1. Joint distribution: smaller joints, more joints involved
2. Serology: RP and ACPA positive
3. Duration: 6 weeks or more
4. Acute phase reactants: ESR OR CRP raised!

 Ddx: psoriatic arthritis RA type, SLE, Jaccoud's arthropathy

Invx (4) RA is a clinical diagnosis but presence of RF and anti-CCP increase likelihood
1. Autoimmune panel for diagnosis: RF (more joints and more extra-articular manifestations),
anti-CCP (prognostic marker for faster rate progression and erosive disease), ANA +/- synovial
fluid
2. Inflamm markers for disease activity: both DAS28-ESR and CRP (compared to ESR only in SLE),
anemia + leukopenia but increased platelets
3. Before start meds: FBC, RP, LFT, Hep B/C, T-spot, HIV
4. XR:
a) Early: soft tissue swelling -> widening of joint spaces due to effusions -> marginal erosions
(compared to juxta-articular in gout) -> juxta-articular osteopenia
b) Late: articular destruction and uniform loss of joint space (compared to compartmental loss
in OA), dislocation
c) C spine: atlanto-axial subluxation
Extra-art  Causes of anemia
manifest 1. Megaloblastic (3): MTX/azathioprine due to folate deficiency, pernicious anemia, liver
cirrhosis
2. Pancytopenia: aplastic anemia or marrow suppression from penicillamine/gold,
hypersplenism in felty's syndrome
3. NCNC: anemia of chronic disease, CKD
4. Microcytic: Fe def from NSAID/nutritional
5. AIHA

 Causes of eye involvement:

1. Keratoconjunctivitis sicca (most common), episcleritis, scleritis, scleromalacia perforans
(painful red eye)
2. Drugs: cataracts from steroids, retinopathy from HCQ
3. Sjogrens
4. Extraocular muscle involvement: from mononeuritis multiplex, penicillamine-induced MG,
tendon synovitis
  Causes of renal disease:
1. Heavy proteinuria: penicillamine/gold induced membranous nephropathy, renal amyloidosis,
minimal change from NSAIDs
2. Blood in urine: analgesic nephropathy / papillary necrosis, FSGS, rheumatoid vasculitis
3. RA is associated with mesangioproliferative GN

 Causes of respiratory disease:

1. Cricoarytenitis (flow volume loop)
2. Pulmonary fibrosis, pneumonitis, PHTN, bronchiolitis obliterans
3. Pleural effusion
4. Pulmonary nodules, Caplans syndrome
Mgt  Pharmaco
1. Acute: steroids and NSAIDs
2. DMARDs:
a) First line MTX with folic acid - monitor FBC LFT and CXR. Start at 10mg/wk then escalate 5mg
every 2 weeks. Usually started with steroid as takes 1-3 weeks to work
b) Leflunomide, sulphasalazine, HCQ for mild/seroneg disease
c) Safer in pregnancy: HCQ, sulphaslazine. MTX and NSAID absolutely CI!
3. TNF inhibitors: if inadequate responde to at least 2 DMARDs including MTX
a) SC etanercept: can cause demyelination
b) IV infliximab: can cause reactivation of TB, need to stop at least 2-4 week before major
surgery
c) Adalimumab
d) Non TNF = Infusions 2 weeks apart: ritux (anti CD20 mAb), abatacept, tocilizumab
4. CI to biologics (4): recent malignancy, ongoing infection, NYHA 3-4 HF or poor EF (for anti TNF
alpha), RA overlap with lupus

 Non pharmaco:
1. Patient education, PTOT for functional aid/rehab +/- surgery for CTS
2. Malignancy, osteoporosis screen
3. Manage CV RF and smoking cessation as RA increases CVS risk
SLE
O/E  Look:
1. Face/mouth:
a) This young lady has a butterfly rash on her face sparing the nasolabial folds
b) There is scarring alopecia, mouth ulcers, and telangiectasia
2. Hands:
a) Symmetrical deforming (reversible) arthropathy of the fingers - a/w swan neck, Boutonniere,
Z deformity, ulnar deviation, swelling, subluxation
b) There is a vasculitic rash and nail fold infarcts
c) Palmar erythema
d) Raynauds phenomenon
e) I did not note any other rash, pallor, bruising or peripheral edema
 Feel: synovitis
 Move: grip, pincer, coarse and fine function

 Extra-articular:
1. Auscultate heart and lungs for pericardial rub, pleural rub or effusion
2. Neuro: proximal myopathy, pronator drift, cerebellar, peripheral neuropathy
3. Neck: LN
 4. Abdo: scars suggestive of peritoneal dialysis/renal transplant, hepatosplenomeg, shifting
dullness
5. Eyes: episcleritis, cataracts, DM/HTN changes

 Offer:
1. BP for HTN from renal disease or steroids
2. Temperature
3. Urinalysis for blood and protein from renal disease, glucose due to steroids/DM
Diagnosis  SLICC criteria: 4 out of 11
1. 4 skin: malar rash, discoid rash, oral ulcer, photosensitivity +/- alopecia
2. 4 organ: renal (proteinuria >0.5g/day or >3+ on dipstick, cellular cast), serositis (pleuritis,
pericarditis), CNS incl seizures/psychosis, Jaccouds arthropathy (symmetrical non-erosive
involving 2 or more joints, deforming but reversible)
3. 3 blood:
a) ANA +ve in 95%!
b) Haem: hemolytic anemia, leukopenia, thrombocytopenia, leukopenia, lymphopenia
c) Immune: anti-dsDNA, anti-Sm, anti-Rho, anti-RNP, antiphospholipid, low C3 and C4

 Pathophys: type 3 hypersensitivity reaction with circulating immune complex, antibodies

against dsDNA in 60-70%, female adolescent, HLA DR2 and DR3
Invx 1. Diagnostic: 3 blood in SLICC
2. Supportive: FBC RP LFT looking for cytopenia, normal CRP but raised ESR
3. Complications:
a) Renal: RP, UFEME, 24hr UTP showing protein >/=0.5g/day, cellular cast
b) Baseline eye screen prior to HCQ use
c) CVS: CXR, TTE, cardiac cath for coronary artery abnormalities
d) CNS: CT/MRI brain, cerebral bloodflow studies, LP showing pleocytosis/elevated protein/low
glucose
e) XR spine
Mgt  Mgt:
1. Steroids for ALL!
a) if organ-threatening disease, pulsed IV methylprednisolone and cyclophosphamide, followed
by maintenance steroids + MMF/Azathioprine
b) Organ threatening disease (5): severe lupus nephritis, CNS lupus, AIHA, autoimmune
thrombocytopenia, acute pneumonitis
2. Add HCQ for all mild disease (skin)
3. Steroid-sparing agents: cyclophosphamide, MMF, azathioprine
4. Last line if HCQ/steroids fail: ritux, CNI, IVIG, plasmapharesis
Systemic sclerosis
O/E  History:
1. Sx:
a) Hands: Raynauds (1st!!), skin tightness/puffy fingers, SC nodules from calcinosis,
telangiectasia, vitiligo
b) GI: dysphagia/indigestion, diarrhoea
c) Lung: cough from IPF
d) Hx of CCF, renal crisis
2. Treatment received

 Look:
1. Face:
a) The skin over the fingers and face is smooth, shiny and tight
b) The nose has a pinched appearance
c) There is telangiectasia with hypopigmentation and vitiligo
d) There is microstomia, with perioral tethering and pseudoraghades
2. Hands:
a) Swollen hands and fingers (early)
b) Flexion deformities of the fingers
c) Sclerodactyly with atrophy of the soft tissues at the ends of the fingers
d) The nails are beaked and atrophic +/- pitting, with evidence of vasculitic lesions and
Raynauds phenomenon
e) CTS scar
 Feel:
1. Taut skin with double pinch test
2. Nodules of calcinosis palpable in some fingers
3. Synovitis
4. Tinels
 Move: grip, pincer, hand function
 Extra-articular:
1. Auscultation of chest:
a) Fine end insp creps suggestive of pulmonary fibrosis
b) Loud pulmonary component of 2nd heart sound suggestive of PHTN
2. Proximal myopathy
Request
1. Measure BP
2. Further hx about dysphagia, dry eyes and mouth, Raynauds
Types  Types/variants: ANA +ve!!
1. Limited cutaneous: face and extremities, often w CREST, a/w anticentromere
2. Diffuse cutaneous: face, trunk, greater risk of cutaneous disease, aw SCl-70
3. Localized scleroderma: linear, morphea
4. Mixed connective tissue disease (MCTD), involving features of SLE, SSc, polymyositis and RA.
A/w antibody to nuclear ribonucleoprotein (RNP) antigen.
5. Scleroderma-like syndromes E.g. eosinophilic fasciitis
Ix  Diagnostic
1. ANA +ve in 90%, RF +ve in 30%
2. Anti SCL-70 +ve in diffuse, anti-centromere +ve in limited
3. ANA -ve in localized morphea

 Ix for complications: HRCT, RP, UFEME/UPCR, TTE

1. Lungs: IPF, PHTN
 2. Renal crisis: GN, malignant HTN, MAHA
3. CVS: spasm of cornary vessels, myocardial fibrosis, pericarditis
Mgt 1. Education and counselling E.g. avoid cold for Raynauds
2. Symptomatic E.g. PPI for esophagitis
3. ACEi for renal crisis
4. Antifibrotic/immunosuppressive
5. Short courses of low dose pred E.g. 10mg/day may decrease edema during early skin
involvement, but does not prevent skin induration when SSc progresses
Psoriasis
O/E  Look/feel:
1. Bilat deforming polyarthropathy, tender
2. Sausage-shapeed fingers, tenosynovitis
3. Dorsal guttering and wasting of the thenar and hypothenar eminence
4. Nails: pitting, onycholysis, subungual hyperkeratosis, discoloration of nails (80% involvement
if polyarthropathy)
5. Skin: well circumscribed plaques on the extensor surfaces of the elbows and scalp, with
salmon pink hue and silvery scales
6. Surgical scars
7. Mention no evidence of gout, as a/w psoriasis!!
 Function: impaired/preserved, grip, pincer, coarse (doorknob), fine (transfer coins, cap pen),
abduct and internally rotate shoulder --> Mention treatment complications like steroids!

 Complete examination:
1. Examining for other joint involvement
2. Skin: esp scalp, kness, natal clef, intragluteal folds, submammary folds, Koebners
phenomenon
3. Enquire on aggravating factors
Types  Types of skin lesions:
1. Plaque
2. Guttate: numerous small papular, streptococcal
3. Pustular: can be localized or generalized
4. Erythrodermic: generalized erythema and scaling, can be life threatening
5. Inverse: plaques involving intertriginous area without typical silvery scales
6. Type of joint involvement (5): OA, RA, AS, oligo/mono, arthritis mutilans
Common  Radiological features:
qns 1. Periostitis i.e. "fluffy"
2. Destruction of small joints
3. "Pencil in cup appearance"
4. Non marginal syndesmophytes in AS type
5. Prognosis: 40% deforming and erosis, 10% disabled

 Assessment of severity = Psoriasis Area and Severity Index

1. Based on area, thickness, redness and scaling
2. <10 mild, 10-50 mod, >50 severe
 What is Koebner's phenomenon: new skin lesions at site of cutaneous trauma. Occurs in 30%
psoriasis 10-20 days post trauma. Also occurs in eczema, lichen planus, vitiligo, lichen sclerosus
et atrophicus

 Ddx for onycholysis (3): fungal infection, thyrotoxicosis (Plummer's nails), lichen planus

 Aggravating factors:
 1. Emotional stress
2. Alcohol
3. Strep infection, classically a/w guttate
4. Drugs: BB, ACEi, indocid, lithium, antimalarial
5. Injury to skin inclulding mechanical, sunburn
Mgt 1. Non pharm: education and counselling, avoidance of aggravating factors
2. Topicals: topical steroids, coal tar, calcipotriol (vit D3), dithranol, retinoids
3. Systemic: UVB, MTX, retinoids, systemic steroids, cyclo, tacrolimus/MMF
4. Novel: Infliximab, etanacept
OA hands
O/E /  Look at hands:
present 1. Presence of Heberden's nodes which are bony swelling affecting the DIPJ, Bouchard's nodes
affecting DIPK
2. Squaring of both hands as a result of subluxation of the first MC
3. No muscle wasting

 Function is preserved with full ROM and grip strength, pt was able to perform coarse
function like turning a door knob and fine motor like transferring coins +/- Tinel's sign

 Establish cause:
1. Primary
2. Secondary: there are no features of acromegaly or hemochromatosis

 Request to examine other joints

1. Knee: varus/valfus deformity, crepitus, wasting of the quadriceps
2. Hips
3. Cervical and lumbar spondylosis
4. Gait: trendenlenburg's sign with downward tilting of the pelvis on the affected side

 Summarize: I would like to offer the diagnosis of primary generalized OA or nodal OA with
OA of the hands in this middle aged lady
Types 1. Primary generalized OA/nodal OA
a) AD, middle aged woman
b) OA of DIPJ/Heberdens with marked deformity but preservation of function. Also affects
CMCJ of thumb, knees and hips
2. Secondary
a) Trauma
b) Inflamm arthropathies: RA, gout, septic arthritis
c) Endocrine: acromeg, hyperparathyroidism
d) Metabolic: chondrocalcinosis, hemochromatosis
e) Neuropathic joints: DM, tabes, syringomyelia
Ix 1. Radiographical: subchondral bone cysts and sclerosis, osteophytes narrowed joint space,
varus deformity
2. Joint tap: <100 WBC/ml
Mgt 1. Education: appropriate footwear, weight mgt
2. PTOT
3. Pharmaco: analgesia (NSAID), tetracycline, diacerin (anti IL-1), HCQ
4. Intra-articular steroids, knee and hip replacement
Gouty hands
O/E  Look:
1. On examination of the hands, there is asymmetrical swelling affecting the small joints of the
hands, with tophi formation, some of which are exuding chalky material.
2. There is wasting of the intrinsic muscles
3. Rest of body: look for tophi on the extensor aspects of the forearms, olecranon, bursa, pinna
of the ear, small joints of the feet, achilles tendon
 Feel: tenderness and warmth of hand joints suggestive of active arthritis
 Move: grip, pincer, coarse and fine movt
 Etiology:
1. Not obese looking, no DM dermopathy or xanthelasma which are a/w gout
2. There is no evidence of chronic ethanol ingestion such as palmar erythema, dupuytren's
contracture and parotidomegaly
3. There is no sallow appearance suggestive of chronic renal failure
4. I did not detect any conjunctival pallor, hypertrophic or bleeding gums and pt is not plethoric
which may suggest presence of lymphoproliferative disease or polycythemia
5. There are no psoriatic skin lesions
 Complete exam:
1. Test gait esp if feet affected
2. Take BP and urinalysis for glycosuria, as well as proteinuria suggestive of uric acid
nephropathy
3. Take a detailed drug history, dietary history and history of alcohol consumption
Ankylosing spondylitis
O/E  Steps: "Question Mark posture!"
1. Ask if there is pain!
2. Stand the pt, then ask pt to walk, turn around and return to original position
3. Spine movts: touch toes with fingers, lateral flexion, rotation
4. Neck: Look left and right, touch chest with chin
5. Offer systemic exam for extra-articular manifestations
a) Lungs: reduced chest expansion, fibrosis
b) Heart: early diastolic murmur (AR), pacemakers
c) Eyes: anterior uveitis/iritis
d) Feet: achilles tendonitis and plantar fasciitis
6. Offer:
a) Heels-hip-occiput test, measure of occiput to wall distance, modified schoeber's test and
chest expansion
b) I would like to rule out other ddx by examining skin for psoriatic arthritis, abdomen for IBD
(enteropathic arthritis), and for Reiter's syndrome (triad of urethritis, arthritis and
conjunctivitis)

 Present:
1. This pt has ankylosing spondylitis as evidenced by a stooped, question-mark posture with loss
of lumbar lordosis and a fixed kyphosis with extension of the cervical spine in an attempt to
maintain a horizontal visual gaze. There is also a protuberant abdomen.
2. Spinal movts are restricted as evidenced by the finger to toe test, with limited flexion and
lateral movts of the cervical spine
3. Mention extra-articular manifestations if any
Diagnosis  Rome/New York criteria for diagnosis of AS:
1. Radiological features of sacroilitis
2. Symptoms of back pain (lumbar spine)
3. Physical signs of limited spinal mobility in all 3 planes and chest expansion <2.5cm
  Extra-articular manifestations:
1. Anterior uveitis, iritis
2. Atlanto-axial subluxation
3. Apical fibrosis
4. Aortic regurg, AV block, arrhythmias
5. Amyloidosis
6. Achilles tendonitis, plantar fasciitis
Invx 1. Imaging with AP view of SI joints and AP/Lat of spine
a) Early: erosion and sclerosis of SIJ
b) Later: syndesmophytes in margins of lumbosacral vertebrae
c) Advanced: bamboo spine
2. Blood tests (not so impt): elevated ESR, HLA B27 (95% in AS)
3. Look for complications:
a) CXR: fibrotic changes
b) ECG for conduction blocks
Mgt 1. Education and counselling
2. Non-pharm: PTOT, regular exercise lifelong
3. Pharm:
a) NSAIDs first line, 2nd line anti-TNF like eternecept/infliximab
b) MTX/sulphasalazine for peripheral arthritis, not useful for spine
c) Pred for uveitis
4. Surgery
Dermatomyositis
O/E  Look, hands:
1. Gottron papules (raised violaceous scaly eruption) around knuckles, sparing the phalanges
2. Nail fold telangiectasia, Raynauds
3. Look for arthritis or joint deformity suggestive of concomitant RA, SLE

 Look, face/neck:
1. Heliotrope rash (purplish-blue) around eyes, back of hands, extensor surfaces of
elbows/knees, trunk
2. Erythematous rash on neck and upper chest in V shape
3. Subcutaneous edema mainly around the eyes (due to transient increase in capillary
permeability)
4. Rounded facies and abdo striae suggestive of cushings syndrome from chronic steroid use

 Feel/move:
a) Proximal muscles weakness and tenderness i.e. polymyositis

 Offer:
1. Examine for dysphagia as a/w scleroderma
2. Examine breast, lungs and abdomen for signs of occult malignancy
Diagnosis  Diagnosis: Bohan and Peter criteria
1. Symmetric proximal muscle weakness
2. Typical dermatomyositis rash, which is only distinguishing feature from polymyositis!
3. Elevated muscle enzymes
4. EMG findings
5. Characteristic muscle biopsy abnormalities with absence of histopathologic signs of other
myopathies
  Associations:
1. Malignancy: GI, lung, breast, ovary
2. CTD: RA, scleroderma, SLE, MCTD

Invx 1. Serum muscle enzymes: CK and aldolase

2. EMG showing myopathic facies with spontaneous fibrillation, polyphasic potentials
3. Muscle biopsy showing necrosis and infiltration of inflammatory cells
4. +ve urine dipstick if pseudohaematuria due to myoglobinuria
Mgt 1. Steroids
2. 2nd line azathioprine, MTX, IVIG
3. Malignancy screen