ICC-2 Rheumatology — Disease Index & Clinical Criteria

Written by Betra Youhanna, a medical student and not a doctor — check every detail against your own teaching.

Disease Index · 40 diseases across 10 categories · built from 13 v4 TSVs (171 cards) with PDF-cross-checked content

Criteria & Scores · 42 scoring systems, classifications, and mnemonics across 5 categories

Treatment Reference · every drug used in rheumatology · mechanism, main indications, main side effects · from ICC-2 [025] LGW

C/P — Clinical Presentation

Inves — Investigations

Mng — Management

Special — Pathognomonic / disease-unique

Inflammatory Arthritides

1 entries

Rheumatoid Arthritis (RA)

C/P

Symmetrical small-joint polyarthritis — MCP/PIP/MTP, wrists, elbows, shoulders, knees, ankles; DIP spared
Effusion + pain + loss of hand function early; deformities later (Boutonnière, swan-neck, Z-thumb, ulnar deviation MCPs, radial deviation wrists)
4 patterns: insidious 70%; abrupt acute polyarthritis 20%; acute monoarthritis; palindromic 10%
Early-morning stiffness, improves with movement; fatigue, weight loss
F:M 3:1, peak onset 30 yr, prevalence 1%, HLA-DR4/DR1
Extra-articular in 75% within 5 yr
Extra-articular — Pulmonary (frequent): pleuritis (chest pain + pleural effusions, common); peripheral pulmonary nodules (1–8 cm, may cavitate + cause effusions); lower-lobe pulmonary fibrosis (mild, non-progressive)
Extra-articular — Cardiac: pericarditis (most common cardiac manifestation); endocarditis; myocardial disease from nodular granulomatous lesions
Extra-articular — Renal: renal amyloid (can cause nephrotic syndrome); glomerulonephritis (drug-related)
Extra-articular — Neurological: entrapment neuropathies (e.g. carpal tunnel); peripheral neuropathy (mild glove-and-stocking sensory loss); mononeuritis multiplex (from vessel inflammation spreading to nerves)
Extra-articular — Vasculitis: nailfold splinter haemorrhages (mild, chronic); small-vessel vasculitis (nodule formation); systemic vasculitis complicating RA is uncommon

Inves

CBC (normochromic-normocytic anaemia, thrombocytosis), ESR/CRP raised, U&E + LFTs (pre-DMARD)
RF — IgM vs IgG Fc, 70–80%, high titre tracks severity / extra-articular / nodules
Anti-CCP — 97% specific, 78% sensitive, marker of erosive disease
X-ray hands: soft tissue swelling → periarticular osteopenia → joint erosions → subluxations
US, MRI for early synovitis
Joint aspirate: clear yellow, variable WCC (lymphocytes), low glucose, high protein, RF +

Mng

MDT (doctor, specialist nurse, physio, OT)
DMARDs = main line; methotrexate drug of choice (folic acid 1 mg/day cover)
Bridging: NSAIDs ± steroids (intra-articular, oral, IV)
Other DMARDs: hydroxychloroquine, sulfasalazine, leflunomide, gold (no longer used), penicillamine
Immunosuppressives: azathioprine, cyclophosphamide, cyclosporin
Biologics: anti-TNF (etanercept SC, infliximab IV + MTX)
Surgery: synovectomy, arthroplasty

Special

Affects only synovial joints
Atlanto-axial subluxation noted (deck cites but bedside test and anaesthetic risk details from textbook)
5 ocular: keratoconjunctivitis sicca, episcleritis, scleritis, scleromalacia, scleromalacia perforans
Caplan's syndrome — pneumoconiosis + multiple peripheral pulmonary nodules (coal miners)
Subcutaneous nodules in RF+ patients at pressure sites (elbows, scalp, fingers)

Connective Tissue Diseases

8 entries

Systemic Lupus Erythematosus (SLE)

C/P

Chronic autoimmune multisystem disease, F:M 9:1, age 15–50, 1:1000, Afrocaribbean/Asian:Caucasian 9:1
Constitutional: fatigue, lethargy, fever, weight loss
Skin: photosensitive malar rash (55–85%), DLE (15–30%), mouth/vaginal/nasal ulcers, alopecia 50%
Joints: non-erosive polyarthritis 90% (hands, wrists, knees); Jaccoud's arthropathy (RA-like but correctable — tendon pathology)
Persistent single-joint pain on steroids → osteonecrosis
Pericarditis (commonest cardiac, responds to NSAIDs); myocarditis; fibrinous endocarditis; MI <35 yr from accelerated atherosclerosis; vasculitis
Renal: 50% white, 75% black; mild GN → rapidly progressive lupus nephritis; renal failure 5%
CNS: epilepsy, hemiparesis, cranial nerve lesions, brainstem/cord lesions, aseptic meningitis, psychoses; PNS neuropathies
Sjögren's overlap 20%

Inves

CBC (cytopenias), ESR high + CRP normal split, LFTs, KFTs, urinalysis + 24-h urine protein
ANA 100% sensitive (must be there); dsDNA 60%; anti-Sm 20% (highly specific); anti-RNP 30%; anti-Ro 30%; anti-La 10%
Complement C3/C4 low in active disease (immune complex deposition); C1q deficiency homozygous link
Kidney biopsy ± skin biopsy; MRI brain for CNS lupus
WHO/ISN class 1–6 (see Criteria tab)

Mng

Patient education (explanation, reassurance), symptomatic, minimise flare + infection, monitor BP + urine
Sun blocks high SPF, NSAIDs, steroids (intra-articular/pulsed/oral/topical)
DMARDs: hydroxychloroquine; immunosuppressives azathioprine + cyclophosphamide per organ
Biologics if refractory
CNS: anticonvulsants + antipsychotics; status epilepticus / coma — pulse methylprednisolone + IV cyclophosphamide ± plasmapheresis
Pregnancy-safe: prednisone, azathioprine, hydroxychloroquine

Special

Top cause of death = infection; then CVS, organ failure (renal), malignancy
Fertility normal but foetal loss with active disease / APS
ESR high + CRP normal classic split (also Sjögren's; rises in tandem suggests drug reaction)
ACR criteria 4/11 (see Criteria tab)
Pre-eclampsia + premature birth risk in pregnancy

physiology · background · low-yield

Mechanism

Genetics: polygenic (≥30 susceptibility genes, no single lupus gene); HLA-DR2/DR3/DR4/DR8 present in 75%; high monozygotic-twin concordance; 5–12% of relatives affected

Other

Myositis with elevated CK + weakness rarely occurs; myositis overlap in ~5% of SLE patients

Antiphospholipid Syndrome (APS, from SLE)

C/P

Venous and arterial thrombosis
Recurrent foetal loss (≥10 wk, premature <34 wk from pre-eclampsia/placental insufficiency, ≥3 abortions <10 wk)
Thrombocytopenia
Livedo reticularis

Inves

Lupus anticoagulant
Anti-cardiolipin IgG/IgM
Anti-β2-glycoprotein IgG/IgM

Mng

Antiplatelet / anticoagulation (deck does not specify regimen)

Special

The vasculitis-mimic that needs testing in any unexplained thrombosis under 50

Sjögren's Syndrome

C/P

Dry eyes + dry mouth (sicca)
Autoimmune inflammation of salivary + lacrimal glands
May overlap with SLE (20%) or other CTDs

Inves

Schirmer's test — filter paper in eye to measure tear wetting
Histology salivary glands
Objectively dry mouth
ANA + anti-Ro + anti-La (most common autoantibody trio)
RF positive but anti-CCP negative → no erosive arthritis
Very high ESR + normal CRP (high IgG)

Mng

Deck does not detail Sjögren-specific therapy

Special

ESR-CRP split (high ESR / normal CRP) due to polyclonal IgG
Distinguishes from RA by positive RF, negative anti-CCP, no erosions

Drug-induced Lupus

C/P

Polyarthritis, rash, pleurisy / pericarditis, pulmonary infiltrates
Mild — renal and psychiatric disease rare
Resolves when offending drug is stopped

Inves

ANA positive (mainly anti-histones + anti-ssDNA)
Anti-histone antibodies
Complement NOT depleted (vs SLE)
ESR + CRP rise together (vs SLE split)

Mng

Stop the drug — antibodies disappear

Special

5 classical inducing drugs: procainamide, hydralazine, isoniazid, anticonvulsants, chlorpromazine
Rare: penicillin, penicillamine, sulphonamides, sulfasalazine, OCP, anti-TNF, minocycline

Chronic Discoid Lupus (DLE)

C/P

Circular, scaly, hyperpigmented skin lesions with erythematous rim + atrophic centre — disfiguring
Photosensitive

Inves

ANA often negative or low — ONLY variant where ANA not required

Mng

Sun protection; topical / intralesional steroids (deck not detailed)

Special

5% progress to systemic SLE

Subacute Cutaneous Lupus (SCLE)

C/P

Photosensitive, non-scarring rash
Mild illness
Low incidence of renal disease

Inves

ANA positive
Anti-Ro antibody (specific marker)

Mng

Sun protection; antimalarials (deck not detailed)

Special

Anti-Ro overlaps with Sjögren's and neonatal lupus

Neonatal Lupus

C/P

Born to mothers with anti-Ro
Transient rash
Complete heart block — 2%

Inves

Maternal anti-Ro
Foetal ECG / echo for heart block

Mng

Pacing for complete heart block (deck not specified)

Special

Incidence 1 in 20 babies of anti-Ro+ mothers
Mortality with heart block 15–25%
Recurrence in subsequent pregnancy 15%

Lupus in the Elderly

C/P

15% of SLE cases begin >60 yr
Insidious onset
Interstitial lung disease common
CNS and renal disease uncommon

Inves

As SLE

Mng

As SLE

Special

Different organ pattern: ILD common, renal/CNS uncommon — opposite of young-onset SLE

Inflammatory Myopathies

2 entries

Polymyositis / Dermatomyositis (PM/DM)

C/P

Weeks-to-months onset
Constitutional: fever, weight loss
Non-erosive inflammatory polyarthritis
Raynaud's 20%
Calcification (especially childhood DM)
Proximal muscle weakness — can't comb hair, reach height, rise from chair, climb stairs; Gower's sign
Pharyngeal weakness → dysphagia + breathing difficulty
DM skin: photosensitive rash, heliotrope (purplish eyelid), V sign, shawl sign, Gottron's papules (MCP/IP), mechanic's hands, dilated nail-bed capillary loops, calcinosis cutis, periungual erythema
PM has NO rash (the key DM-vs-PM split)
PM cardiac: AV conduction defects, tachyarrhythmias, myocarditis
ILD common (PFT + CXR)

Inves

5-pillar Dx: symmetrical proximal muscle weakness + ↑ muscle enzymes (CK, AST, ALT, LDH, aldolase) + abnormal EMG + muscle biopsy + rash (DM-specific)
CK can be NORMAL in active DM + IBM
EMG: fibrillations, complex repetitive discharges, small polyphasic units
ANA up to 80%; anti-Jo-1 (anti-histidyl-tRNA synthetase) — anti-synthetase syndrome
Muscle biopsy if other tests inconclusive
PM = diagnosis of exclusion — rule out drug-induced, endocrine, neurologic, muscular dystrophy

Mng

Prednisone 0.5–1.5 mg/kg/day; muscle-strength improvement LAGS enzyme fall
Taper slowly (5 mg/wk → 20 mg/day; 2.5 mg/2wk → 10 mg/day; 1 mg/month)
Bisphosphonates + PPI prophylaxis
Steroid-sparing: MTX, AZA, cyclosporine, IVIG, MMF, cyclophosphamide
Biologic: rituximab (RTX)

Special

Malignancy 4–42% — DM > PM/IBM
Screen with CT chest/abdomen/pelvis; commonest cancers GI, lung, breast, ovaries, NHL
Anti-synthetase tetrad: ILD + myositis + arthritis + Raynaud's
Gottron's papules pathognomonic for DM
Childhood DM = calcinosis cutis hotspot

physiology · background · low-yield

Epidemiology/Risk

Aetiology/epidemiology: genetically predisposed + infective triggers (CMV, Coxsackie, influenza, parvovirus); UV light a risk factor for DM; incidence ~10/million; F>M; onset 40–50 yr

Other

Muscle-weakness DDx (7 groups): medications (statins, corticosteroids, others), infectious, neurologic, endocrine, genetic (muscular dystrophies), metabolic (hypokalaemia, hypercalcaemia), miscellaneous (amyloidosis, sarcoidosis)

Inclusion Body Myositis (IBM)

C/P

Males > females
Onset >50 yr
Slow + insidious
BOTH proximal AND distal muscle weakness
Bilateral but asymmetric

Inves

CK may be normal even in active disease
Muscle biopsy

Mng

Typically non-responsive to steroids

Special

The PM/DM mimic that does NOT respond to steroids
Demographic inversion: males >50 (opposite PM/DM)
Distal involvement = giveaway vs PM/DM (proximal only)

Vasospasm & Scleroderma

4 entries

Raynaud's Phenomenon

C/P

Exaggerated vasoconstrictor response to cold or stress
3-phase: pallor (digital arterial vasospasm) → cyanosis (deoxygenation of static venous blood) → reactive hyperaemia (return of flow)
~1:10 females; thumbs typically spared in primary
Symmetric (primary) vs trophic complications (autoimmune)

Inves

History + exam
Thermography
Capillaroscopy of nail-fold capillaries
ANA autoimmune profile

Mng

Single short attacks: warmth, heating appliances, smoking cessation, vitamins C+E
Frequent / digital ulcers: CCBs (nifedipine, diltiazem) → ARBs (losartan, irbesartan) → SSRIs (fluoxetine) → GTN patches
Severe: IV iloprost (synthetic prostacyclin), sympathectomy
Gangrene: auto- or surgical amputation; antibiotics + surgery if infected

Special

Good signs (primary): teen / early-20s onset, stable, familial, normal capillaries, ANA−
Bad signs (secondary): male, ≥35 yr onset, increasingly severe, no FH, abnormal capillaries, ANA+/ENA+
10–15% of ANA+/abnormal-capillary Raynaud's progress to defined CTD
Frequency by CTD: SSc 96%, SLE/DM/PM/Sjögren 20%, RA <5%
Drug causes: ergot alkaloids, β-blockers, bromocriptine, chemo
Haem causes: polycythaemia, leukaemia, lymphoma, cryoglobulinaemia, cold-agglutinin disease, myeloma, Waldenström
DDx (mimics): vasculitis, APS, Buerger's, macrovascular (embolic/thrombotic/atherosclerotic)

physiology · background · low-yield

Epidemiology/Risk

Secondary Raynaud's — further causes: endocrine (hypothyroidism, phaeochromocytoma, acromegaly); mechanical/occupational (vibration white finger, thoracic outlet syndrome, carpal tunnel syndrome, reflex sympathetic dystrophy)

Limited Cutaneous Systemic Sclerosis (lcSSc / CREST)

C/P

Skin sclerosis distal to elbows + knees + face/neck
Sclerodactyly, microstomia (stiff mouth), calcinosis cutis
Telangiectasia common (more than diffuse) — red blanching facial spots
Raynaud's 96%
GIT involvement (both subsets): lack of peristalsis, dysmotility, reflux, oesophagitis → Barrett's
Pulmonary hypertension equal in both subsets
Renal/cardiac/pulmonary fibrosis: lcSSc < dcSSc
Pulmonary hypertension / cor pulmonale signs: raised JVP, ankle oedema, parasternal (RV) heave, loud P2, tricuspid regurgitation murmur

Inves

ANA 90% positive (nucleolar or centromere pattern)
Anti-centromere antibody — very specific to limited (80–90% of CREST)
Anti-Scl-70 normally negative

Mng

Raynaud's — see Raynaud entry
PH: diuretics, anticoagulation, O₂, digoxin; PDE-5i (sildenafil, tadalafil), prostanoids (epoprostenol, treprostinil, iloprost), ET-1 antagonists (bosentan, ambrisentan)
GIT: small meals, bed-head raise, PPI, prokinetics (domperidone, metoclopramide), yearly endoscopy for Barrett's, cyclical antibiotics for SIBO, ambulatory TPN

Special

CREST = Calcinosis · Raynaud's · Oesophageal dysfunction · Sclerodactyly · Telangiectasia
Better prognosis than diffuse
GAVE (watermelon stomach) — gastric antral vascular ectasia
"If diarrhoea = small bowel; if constipation = large bowel; alternating = both"

physiology · background · low-yield

Other

GI dysmotility workup: barium swallow, oesophageal scintiscan, hydrogen breath test (bacterial overgrowth); barium follow-through — caution, may cause pseudo-obstruction in bowel hypomotility

Diffuse Cutaneous Systemic Sclerosis (dcSSc)

C/P

Distal AND proximal skin sclerosis including trunk + chest
More widespread cutaneous + visceral involvement than limited
Pulmonary fibrosis (lcSSc < dcSSc) — anti-Scl-70 association
Cardiac: pericarditis (effusion, rarely fenestration), myocarditis → HF, arrhythmias, heart block
Renal crisis (see separate entry)
Interstitial lung disease sign: late inspiratory crackles

Inves

ANA 90% positive (nucleolar pattern)
Anti-Scl-70 (anti-topoisomerase-I) — diffuse-specific, 40% of progressive SSc
Anti-RNA polymerase (60% of renal crisis)
PFTs (FVC, DLCO), HRCT (basal fibrosis), echo, right-heart catheter

Mng

Pulmonary fibrosis: N-acetylcysteine → MMF or AZA → monthly IV cyclophosphamide + oral steroids → rituximab → ± anti-fibrotic
See lcSSc for PH and GIT management
Cardiac: per HF protocols (deck not detailed)
Muscle: proximal myopathy (normal CK) — mild; myositis overlap (anti-PM/Scl) → steroids + MTX

Special

Pulmonary fibrosis worse in diffuse
Tendon friction rub on examination
Other features: impotence, depression
Overlap with RA, SLE, Sjögren's, myositis

physiology · background · low-yield

Epidemiology/Risk

SSc epidemiology + triggers: incidence 3.7–56/million/yr, prevalence 31–399/million, peak onset 30–50 yr, F 75–96%; predisposing environmental factors — epoxy resin, rapeseed oil, silica, bleomycin

Scleroderma Renal Crisis

C/P

~5% of SSc, 80% in dcSSc
New-onset BP >150/85
Acute hypertensive retinopathy
Flash pulmonary oedema
Encephalopathy
Affects dcSSc within first 3 years

Inves

eGFR fall ≥30%, creatinine ↑
Microangiopathic haemolytic anaemia on smear
Proteinuria, haematuria, oliguria/anuria
Anti-RNA polymerase 60%
Renal biopsy after BP control: thrombotic microangiopathy

Mng

Control seizures; stop nephrotoxic drugs; gentle BP control
CVP monitoring
ACE inhibitor — most important treatment
IV iloprost (continuous low dose)
Renal biopsy AFTER BP controlled
Dialysis (haemo initial, peritoneal maintenance); transplant outcomes at least as good as SLE

Special

Avoid high-dose steroids in dcSSc within first 3 years — precipitates renal crisis
Screening dcSSc <3 yr from onset: education + monthly BP + 6-monthly GFR

Spondyloarthritides

4 entries

Axial Spondyloarthritis (axSpA) — including Ankylosing Spondylitis (AS) and nr-axSpA

C/P

Dominant symptom: back pain
M:F 3:1; onset 15–25 yr; average diagnostic delay 8 yr
Constitutional: weight loss (early), fatigue, feverishness, night sweats
Inflammatory back pain criteria — 4/5: age <40 onset, insidious, improves with exercise, no improvement with rest, night pain
Question-mark posture: thoracic kyphosis + loss of cervical lordosis
Reduced lumbar flexion (Modified Schober)
HLA-B27 95% in AS

Inves

Modified Schober's test (15→<20 cm = limited)
Occiput-to-wall / Tragus-to-wall (cm)
FABER (Flexion, Abduction, External Rotation) — SIJ stress
Chest expansion 3–15 cm
X-ray SIJs: sacroiliitis (grades 0–IV, see Criteria tab)
MRI SIJs — gold standard; "shiny corner" sign
HLA-B27 (95%); ESR/CRP

Mng

NSAIDs first line — trial 2 before escalating
Biologics: anti-TNF; ustekinumab (IL-12/23) for PsA/psoriasis; secukinumab (IL-17) for PsA/AS
NO role for steroids or conventional DMARDs in axial disease
Screen for hidden infections (TB, HBV) before biologic initiation

Special

4 A's: Anterior uveitis (40%), Aortic valve/root disease (AR), Apical fibrosis, Amyloidosis
nr-axSpA = clinical + MRI inflammation without X-ray sacroiliitis
Only ~5% of HLA-B27+ develop AS
Strongest genetic association in the SpA family

physiology · background · low-yield

Epidemiology/Risk

SpA-family prevalence: PsA ~1% (commonest), AS ~0.25%, ReA 4–5/100,000, EntA variable (IBD ~0.05% pop, ~10%); HLA-B27 association across spectrum

Psoriatic Arthritis (PsA)

C/P

Up to 1% of population (most common SpA); 5% UK has psoriasis
40% develop erosive deforming arthritis
5 patterns: DIP (55–70%, dactylitis "sausage finger"), asymmetrical oligo (<5 joints, 30–50%), symmetrical poly (RA-like, 15–70%), spondylitic (5–33%), arthritis mutilans (3–5%)
Nail involvement: pitting, onycholysis, oil-drop sign, ridging
Enthesitis (sites of tendon/ligamentous insertion)

Inves

Bloods + serology (RF, anti-CCP, ANA, Ig, anti-TTG)
Stool calprotectin if enteropathic suspicion
X-ray hands/feet/SIJ/spine; US peripheral joints; MRI SIJs with STIR

Mng

DMARDs: MTX, SSZ, leflunomide
NSAIDs, intra-articular or systemic steroids
Biologics if 2 DMARDs fail: anti-TNF (etanercept, infliximab, adalimumab, golimumab), ustekinumab (IL-12/23 for psoriasis + PsA), secukinumab (IL-17 for PsA/AS)

Special

DIP joint involvement = the PsA giveaway (vs RA)
Poor prognosis: strong family-history psoriasis, onset <20 yr, HLA-B27/Cw6/DR4, polyarticular, erosive, extensive skin
Gottron's papules NOT PsA — they're DM

Reactive Arthritis (ReA)

C/P

Onset 1–3 weeks after infection
Most resolve 3–12 months; 15–50% recurrent; 30% chronic
Reiter's triad: arthritis + urethritis + conjunctivitis
50% eye, 20–40% GU, 10% aortic valve / pericarditis
Large lower-limb joints: hind-foot, ankle, knee, wrist

Inves

Exclude septic arthritis FIRST
GUM screen (blood, urine, swabs, PCR)
Stool / serology
Synovial aspirate (microscopy, culture, crystals, PCR)

Mng

NSAIDs first; steroids (intra-articular)
DMARDs (MTX, SSZ) if persistent
Treat underlying infection

Special

Pathognomonic: keratoderma blenorrhagica; pustular rash (gonococcal); tenosynovitis small joints (gonococcal); conjunctivitis
STD triggers: Chlamydia, Gonorrhoea
GI triggers: Campylobacter, Salmonella, Shigella, Yersinia
TB-related: Poncet's (general arthritis), Pott's (vertebrae)

physiology · background · low-yield

Microbiology

Additional triggers: Parvovirus B19, streptococcal, enteroviruses, hepatitis A/B/C/E, EBV, CMV, HIV; GU organisms also include Mycoplasma genitalium + Ureaplasma urealyticum

Enteropathic Arthritis (EntA)

C/P

Associated with IBD (UC, Crohn's), coeliac, Whipple's, jejuno-ileal bypass
Peripheral: feet, ankles, knees
Axial more in Crohn's
6 extra-articular features to scan for: erythema nodosum, pyoderma gangrenosum, oral ulcers, clubbing, uveitis, iritis

Inves

Stool calprotectin; colonoscopy
Vitamin D, BMD (esp. Crohn's)
Standard SpA bloods

Mng

DMARDs: MTX, SSZ
NSAIDs, intra-articular steroids
Biologics: anti-TNF
DO NOT use secukinumab (IL-17) in enteropathic — can worsen IBD

Special

IL-17 contraindication is the EntA-specific exam trap
Low BMD + vitamin D deficiency association (especially Crohn's)

Vasculitides

10 entries

Giant Cell (Temporal) Arteritis (GCA)

C/P

Most common form of systemic vasculitis in adults
Large-vessel + medium/small (esp. superficial temporal artery)
Also ophthalmic, occipital, vertebral, posterior ciliary, proximal vertebral arteries
F:M 3:1
Constitutional + headache + temporal/carotid tenderness + jaw claudication + visual/ocular symptoms

Inves

Inflammatory markers raised
Temporal artery biopsy (still useful up to 2 weeks post-steroids; negative doesn't exclude)
Ultrasound: narrowed lumen, hypoechoic halo, turbulent flow — "halo sign"
ACR–EULAR classification criteria

Mng

Prednisolone 40–80 mg od
40 mg if no visual sx
60–80 mg (1 mg/kg) if visual involvement
Mandatory bone protection (Ca + vit D + bisphosphonate) + GI protection (PPI)
Bisphosphonate mandatory if >3 months steroids
Relapse: increase to previous + minimum effective dose
Steroid-sparing: MTX or tocilizumab

Special

Visual symptoms = ophthalmology referral, do not delay
Halo sign on TAU is the cranial-artery cue

physiology · background · low-yield

Other

When to suspect vasculitis (general): unexplained systemic illness (no sepsis/malignancy/drugs), multi-organ involvement, organ ischaemia; clues — palpable purpura, glomerulonephritis (haematuria), mononeuritis multiplex, lung infiltrates

Takayasu's Arteritis

C/P

Large-vessel vasculitis affecting aorta + subclavian arteries

Inves

Arteriography (deck not detailed)
PET-CT (newer imaging cited)
Arteriography — segmental large-vessel features: corkscrew, narrowing (stenosis), aneurysm, luminal irregularity

Mng

Prednisolone + steroid-sparing agent (e.g., MTX)
CVS risk-factor management
Surgery: reconstructive / angioplasty after remission or as emergency

Special

Young female (lecturer mentions but not detailed)

Polyarteritis Nodosa (PAN)

C/P

Medium-vessel vasculitis
Aneurysm + organ infarction dominant
Middle-aged adults (vs Kawasaki children)

Inves

ACR 1990 criteria (≥3/10 for classification)

Mng

Steroids + immunosuppression (deck not detailed)

Special

"Aneurysm + infarction in middle-aged adult" = think PAN
HBV association (not deck-cited but classical)

Kawasaki Disease

C/P

Mucocutaneous lymph-node syndrome
Infants + children

Inves

Clinical diagnosis (deck not detailed criteria)

Mng

IVIG + aspirin (deck not detailed)

Special

Pediatric medium-vessel vasculitis (lecturer cites name and age group only — coronary details from textbook, verify)
Coronary vasculitis is the major cause of morbidity and mortality (deck-taught, not textbook-only)

Wegener's Granulomatosis (Granulomatosis with Polyangiitis, GPA)

C/P

Triad: upper airway (ENT) + lung + kidney
Pulmonary: nodules, vasculitis, haemorrhage
Renal: variable → RPGN; asymptomatic haematuria

Inves

cANCA (anti-PR3) >95% positive
Renal biopsy; chest imaging

Mng

Steroids + cyclophosphamide induction; AZA/MTX/MMF maintenance; rituximab option
Pre-biologic screen: HBV/HCV + TB

Special

ENT + lung + kidney triad
cANCA/anti-PR3 specificity is the cue
Impaired renal function predicts poor outcome

Churg–Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis, EGPA)

C/P

Lanham 1984 triad: asthma + eosinophilia + systemic vasculitis (>2 extrapulmonary organs)

Inves

pANCA (anti-MPO)

Mng

Steroids; immunosuppression (deck not detailed)

Special

Asthma + eosinophilia = think EGPA
pANCA (vs GPA's cANCA)

Microscopic Polyangiitis (MPA)

C/P

Necrotising vasculitis with FEW immune deposits
Small vessels predominantly (also medium)
Kidney (GN very common) + lung (pulmonary vasculitis common)

Inves

pANCA (anti-MPO) often positive

Mng

As ANCA-associated vasculitis (steroids + cyclophosphamide / rituximab)

Special

Pauci-immune GN clue
pANCA shared with EGPA — distinguish by asthma/eosinophilia (EGPA) vs not (MPA)

Henoch–Schönlein Purpura (HSP)

C/P

Most common vasculitis of childhood
Leucocytoclastic vasculitis of small vessels
Often preceded by URTI
Tetrad: symmetrical palpable purpura + GIT (colic, melaena, haematemesis) + arthritis/arthralgia + renal (haematuria, proteinuria)

Inves

Clinical diagnosis
Biopsy: perivascular IgA deposition

Mng

Usually self-limiting; supportive
Severe: corticosteroids, immunosuppressants, plasma exchange

Special

Pediatric IgA vasculitis
FMF association (HSP can complicate FMF)

Essential Mixed Cryoglobulinaemia

C/P

Skin: palpable purpura (exposed areas, extremities)
Joints: arthritis / arthralgia
Neuro: peripheral neuropathy
Lung: alveolitis
Kidney: GN

Inves

Positive cryoglobulins
Low C4
RF positive

Mng

Treat underlying HCV or haematological malignancy
Immunosuppression
Plasma exchange if organ- or life-threatening

Special

Cryoglobulin = cold-precipitating Ig
HCV is the classic trigger to identify
Triad lab pattern: cryoglobulin+ / low C4 / RF+

Behçet's Disease

C/P

Recurrent oral ulcers (mandatory) + 2 of: genital ulcers, eye (uveitis, retinal vasculitis), skin (erythema nodosum, pseudo-folliculitis, papulopustular, acneiform nodules), pathergy
Venous: thrombophlebitis, thrombosis
Arterial: aneurysm
Neurological involvement
Epididymitis

Inves

Clinical diagnostic criteria
Pathergy test (skin hypersensitivity to needle prick)

Mng

Corticosteroids (local or systemic)
Colchicine — mucocutaneous
Immunosuppressants
Anti-TNF refractory
Antiplatelet / anticoagulation for vasculopathy

Special

Variable-vessel vasculitis (arterial AND venous)
Pathergy test is the named bedside test
Aortic aneurysm on imaging = think Behçet's or Takayasu

Degenerative & Crystal Arthropathies

3 entries

Osteoarthritis (OA)

C/P

Loss of articular cartilage + bony changes at joint ends/margins
Most common arthritis (UK + Egypt); main symptom = pain
Can be radiographic without symptoms
Higher prevalence in African Americans vs white
Primary: idiopathic, age-related; hips, knees, DIPs, CMC of thumb
Secondary: congenital (epiphyseal dysplasia), metabolic (haemochromatosis, acromegaly, CPPD), traumatic, inflammatory (RA); atypical joints (MCPs, wrists, elbows, shoulders, ankles)
Symptoms: pain, stiffness, crepitus, soft/hard swellings
DIP = OA (vs RA spares DIP)

Inves

Largely clinical
X-ray: osteophytes, joint-space narrowing, subchondral sclerosis, cysts
Primary knee = medial/central compartment; secondary = anywhere

Mng

Exercise (local strengthening + general aerobic) first
Paracetamol + topical NSAIDs first-line
Standard NSAIDs or COX-2 + PPI cover
Arthroscopic lavage/debridement ONLY for mechanical locking
Joint replacement: substantial QoL impact + refractory non-surgical Mx (mostly stage 4)

Special

DIP involvement distinguishes OA from RA (RA spares DIP)
"Bony swellings" + crepitus = OA vs warmth + soft swelling = RA

physiology · background · low-yield

Mechanism

Cartilage pathophysiology: articular cartilage = chondrocytes + ECM (collagen II, IX, XI + proteoglycans); early OA — ↑ water content + ↓ type-II collagen/proteoglycan; chondrocytes shift to a catabolic phenotype (↑ proteolytic enzymes)

Gout

C/P

Monosodium urate monohydrate crystal arthritis secondary to hyperuricaemia
8× more common in males; uncommon premenopausal women; rare in children
Onset males 40–50 yr; females >60 yr; prevalence 8.4/1000
Acute: rapid pain + swelling + erythema + exquisite tenderness; skin desquamation; systemic features possible
1st MTP "podagra" in 50–70% first attack
Monoarticular 90%; polyarticular with progression, in females, myelo/lymphoproliferative
Non-articular deposition: olecranon bursa, Achilles tendon
Chronic: tophi (urate + lipid/protein/MPS matrix) → damage bone/cartilage/tendons/soft tissue
Precipitants: acute illness, trauma, surgery, alcohol (beer/wine), starvation, drug initiation (allopurinol)

Inves

Crystal identification — gold standard (needle-shaped, strongly negatively birefringent, yellow parallel to axis)
Serum urate CAN be normal in 25–49% of attacks (acute-phase reactant — sequestered in joints)
WCC/ESR/CRP usually elevated
X-ray: punched-out, well-defined erosions; subarticular cysts; joint-space narrowing; soft-tissue swelling around 1st MTP in acute
Hyperuricaemia defined as serum urate > 6.8 mg/dl (> 360 µmol/l)

Mng

Acute: cold pack + rest; colchicine OR oral steroids OR NSAIDs
DO NOT start/stop ULT during attack without cover
2020 ACR: initiate ULT during flare is now conditionally recommended
ULT indications: tophaceous gout, radiographic erosions, uric-acid nephrolithiasis, urate nephropathy, chemo-induced gout prophylaxis, ≥2 attacks/year
Allopurinol — XO inhibitor (oxypurinol active metabolite, t½ 24h, renal, titrate to CrCl); life-long
Febuxostat — XO inhibitor (hepatic clearance, can use in renal impairment); CVD-death warning (ACR 2020)
Uricosurics (sulfinpyrazone, losartan, fenofibrate) — only with normal renal function (renal calculi risk)
Recombinant uricase — debulks tophi; limited by neutralising antibodies
URAT-1 inhibitor; IL-1 antagonist (acute flare)
Lifestyle: limit alcohol, purine, HFCS; weight loss
Uricosuric prophylaxis drugs also include probenecid and benzbromarone

Special

CAN'T LEAP mnemonic for under-excretion drugs: Cyclosporin, Alcohol, Nicotinic acid, Thiazides, Lasix, Ethambutol, Aspirin (low-dose), Pyrazinamide
Aspirin paradox: low-dose CAUSES gout, high-dose TREATS it
DDI alert: clarithromycin inhibits CYP3A4 → colchicine toxicity (pancytopenia); use azithromycin not erythromycin (rhabdo risk)
Metabolic syndrome cluster — ↓ renal urate excretion + ↑ uric acid production
Polyarticular features in females + myelo/lymphoproliferative

physiology · background · low-yield

Mechanism

Hyperuricaemia mechanism split: under-excretion (90%) — idiopathic/genetic, chronic renal failure, CAN'T LEAP drugs; over-production (10%) — polycythaemia, leukaemia, pernicious/chronic haemolytic anaemia, lymphoproliferative disorders, psoriasis, cytotoxics (tumour lysis)
Biochemistry: purines → hypoxanthine → xanthine → uric acid; humans have inactive uricase so cannot oxidise urate to highly-soluble allantoin

Calcium Pyrophosphate Dihydrate Crystal Deposition Disease (CPPD / Pseudogout)

C/P

Inflammatory arthropathy from CPPD crystal deposition in hyaline + fibrocartilage
Female preponderance; increases with age
Spectrum: pseudogout + pseudo-OA
Pseudogout: acute knee synovitis in elderly female; pain + swelling + erythema + fever
Pseudo-OA: generalised OA background + wrist/shoulder/knees + marked inflammatory response
Precipitants: trauma, illness, surgery, spontaneous

Inves

Synovial fluid: turbid, blood-stained, intracellular CPPD crystals on compensated polarised light microscopy
Crystal: rhomboid, weakly (positively) birefringent
X-ray: linear + spotty calcification of hyaline AND fibrocartilage; well-preserved joint space

Mng

Acute attack: NSAIDs or colchicine — symptomatic only
Colchicine prophylaxis for frequent attacks
NO crystal-dissolving therapy (unlike gout)

Special

Classic deposition sites: knee, wrist, pubic symphysis, shoulders, intervertebral discs
Most common cause of cartilage calcification (chondrocalcinosis)
Crystal contrast vs gout (see Lab Pattern table)

Acute Swollen Joint Causes

4 entries

Septic Arthritis

C/P

Medical emergency; most serious cause of acute monoarthritis
Mortality 10%; long-term sequelae 30%
Painful + swollen + red joint
85% monoarthritis; knee most common
Fever only in 50% — absence does NOT exclude
Sub-acute onset over 1–2 weeks

Inves

Aspirate joint FIRST (before antibiotics) — colour/clarity/frank pus; Gram stain, WCC, C&S, crystals
Blood cultures
Infective markers may be absent (CRP most sensitive)
X-rays limited value acutely
Prosthetic joint → orthopaedics

Mng

Sepsis management
IV antibiotics after aspiration (local guidelines; modify per Gram + cultures)
2 weeks IV then 4 weeks oral typical
Arthroscopic washout OR daily aspiration
Surgical removal of prosthesis if infected
Analgesia

Special

Most common organism: Staphylococcus aureus
Pie chart: S. aureus > Strep > S. epidermidis > MRSA > Gram-negatives
Normal WCC / no fever does NOT exclude — high index of suspicion in any red hot joint
Always differentiate from acute gout (urate crystals vs organisms)

Haemarthrosis (Haemophilias)

C/P

Spontaneous joint bleeding
1 in every 10,000 males
Haemophilia A — factor VIII deficiency
Haemophilia B — factor IX deficiency
Von Willebrand disease — factor V deficiency (as listed in lecture; classically vWF deficiency)

Inves

Coagulation studies (deck not detailed)

Mng

Replacement of deficient clotting factor
Rest in EXTENSION + ice + analgesics + NSAIDs (NSAID use with bleeding-disorder caution implied)
Severe: arthroscopic synovectomy

Special

Acquired bleeding from heparin/warfarin → minor-trauma haemarthrosis
vWD factor-attribution in lecture is non-standard — confirm with tutor

Pigmented Villonodular Synovitis (PVNS)

C/P

Locally invasive synovial growth — knee most commonly affected
Bloody effusion = the alert sign

Inves

X-ray, radionuclide studies, MRI

Mng

Surgical (deck not detailed)

Special

Neoplastic cause of monoarthritis
Bloody effusion in a knee = think PVNS (or trauma)

Palindromic Rheumatism

C/P

Recurring acute arthritis with months-long symptom-free periods

Inves

Clinical pattern recognition

Mng

Deck not detailed (in RA module hydroxychloroquine is mentioned as adjuvant for palindromic)

Special

Some progress to RA — listed as one of 4 RA patterns (10%)
Affects one joint, then "skips" to another

Chronic Pain Syndromes

3 entries

Complex Regional Pain Syndrome (CRPS)

C/P

Continuing regional pain disproportionate to inciting event
Affects distal limbs (upper:lower 2:1)
Spontaneous + evoked pain, autonomic disturbance, motor + trophic changes
Type I — no nerve lesion (90%, formerly RSD); Type II — nerve lesion present (formerly causalgia)
Triggers: fracture 45%, sprain 18%, elective surgery 12%, spontaneous <10%
Age 30–50; F:M 3:1; 75% resolve within 1 yr
Higher incidence in diabetic children
Stage 1 (acute, 1–3 mo): burning/throbbing pain, sensitivity to touch/cold, localised oedema, warm + red, increased sweating
Stage 2 (dystrophic, 3–6 mo): soft-tissue oedema, skin thickening, muscle wasting + osteoporosis; cold + cyanotic + sweaty
Stage 3 (atrophic): contractures, severe osteoporosis, waxy trophic skin, brittle ridged nails
Sensory anomalies: allodynia, hyperalgesia

Inves

Clinical diagnosis — disproportionate pain
NO definitive test; exclude other causes
Causalgia (synonym cited by lecturer); other historical names from textbook reading

Mng

Physiotherapy — single most important; relieve pain + mobility + prevent stiffness/atrophy + promote circulation
TENS (transcutaneous electrical nerve stimulation)
OT + psychology
Drugs: paracetamol/codeine/tramadol/NSAIDs; morphine if severe (slow-release); buprenorphine or fentanyl patches; amitriptyline + duloxetine (low-dose); bisphosphonates; capsaicin cream; lidocaine patches; sympathetic nerve blocks
Steroids — early stages only (swelling, heat, redness)

Special

Stage matters for therapy: steroids early, physio always, contractures-driven Mx late
"Disproportionate pain after trivial injury" = trigger phrase

physiology · background · low-yield

Background

Synonyms (lecturer-listed): Sudek's atrophy, reflex sympathetic dystrophy, shoulder-hand syndrome, causalgia, acute atrophy of bone, transient osteoporosis, algodystrophy

Fibromyalgia

C/P

Common chronic widespread muscular pain
NOT inflammatory or degenerative — no permanent joint damage
Major QoL impact but not life-threatening / progressive
Triggers: viral infection, physical/mental trauma, period of stress/anxiety, depression (bidirectional)
Central sensitisation — brain pain-processing abnormality
Symptoms: chronic fatigue, diffuse pain (joints/muscles/bone), restless leg syndrome, TMJ disorder, headache, IBS; hypothyroidism association
Sleep: loss of deep sleep; vicious cycle (poor sleep → tiredness → more pain)

Inves

Clinical
Tender-point exam (figure)

Mng

Aim: reduce symptoms + improve QoL (not cure)
MDT: GP, rheum, pain physician, psychology, physio
Non-drug: aerobic + aquatic + moderate-to-high resistance training; CBT; relaxation; acupuncture
Drugs: TCAs (amitriptyline, dosulepin) 2–3 h before bed; SSRIs (fluoxetine, paroxetine); SNRI (duloxetine); gabapentinoids (pregabalin, gabapentin) — 6-week trial

Special

Real pain, no peripheral cause
"Start slow, cut back if symptoms worsen, then increase" exercise rule
Distinguishes from PMR/inflammatory disease by normal CRP, no swelling, no objective findings

physiology · background · low-yield

Other

DDx of generalized chronic pain: inflammatory (RA, SpA, vasculitis), degenerative (OA), low back pain/sciatica, post-herpetic neuralgia, peripheral nerve entrapment, IBS, cancer

Benign Joint Hypermobility Syndrome (BJHS)

C/P

Hypermobility = joint movement beyond normal range
Common cause of generalised pain in young people; girls > boys
Decreases with age
Often asymptomatic — no consequences in many
Symptomatic: joint/ligamentous injuries, pain, fatigue
Reduced muscle strength + balance association
Fibromyalgia frequent overlap

Inves

Clinical exam (deck does not specify scoring system)

Mng

Reassurance — most important; benign, lessens with age, can be beneficial (gymnastics, ballet)
Supportive footwear
Re-establish muscle power + reconditioning
Occasional evening dose paracetamol or NSAID

Special

Mechanism: joint instability + microtrauma + central sensitisation
"Most important treatment = reassurance" is the locked teaching point

Auto-inflammatory

1 entries

Familial Mediterranean Fever (FMF)

C/P

"Recurrent polyserositis"
Autosomal recessive auto-inflammatory disorder
Paroxysmal attacks: peritonitis + pleuritis + arthritis + fever
Patients completely normal between attacks
Onset over 2–4 h; attack lasts 12–72 h
Classical onset WITHOUT warning; sometimes premonitory (irritability, nervousness, anxiety, nausea, myalgia)
Fever: 38–40°C, rises rapidly; may be only manifestation in mild paroxysms
Peritoneal: episodic abdominal pain (almost all); diffuse > localised; constipation during, diarrhoea after; misdiagnosed as appendicitis/cholecystitis (unnecessary surgeries)
Pleural: unilateral chest pain, worse with deep inspiration/coughing; mild effusion
Pericarditis: retrosternal + pleuritic
Joints: large lower-limb joints (hip, knee, ankle); monoarticular; normal between attacks; arthritic episodes longer than abdominal
Skin: erysipelas-like lesion in 50%, lower extremities, resolves spontaneously
Vasculitis: HSP association

Inves

Acute-phase reactants ↑ (CRP, ESR, amyloid A, fibrinogen)
WCC ↑ during attack
Urinalysis: proteinuria → suspect amyloidosis; haematuria 5%
Synovial fluid: inflammatory, WCC up to 100,000/μL
MEFV gene mutation — can SUPPORT diagnosis, cannot exclude
FMF is a CLINICAL diagnosis
Imaging non-specific

Mng

Colchicine — treatment of choice
Aims: prevent attacks + normalise interval inflammation + prevent amyloidosis
Also halts amyloidosis progression
Side effects: diarrhoea + vomiting
Safe in pregnancy + breastfeeding
Resistance suspected if max-tolerated dose (3 mg/day adult) ineffective despite adherence, or APRs stay high between attacks
Biologic options: IL-1 receptor antagonists (anakinra), TNF inhibitors (etanercept), interferon-α

Special

MEFV gene mutation → uncontrolled IL-1 → serositis
Systemic amyloidosis (AA) → nephrotic syndrome → ESRD — the devastating complication
Renal vein thrombosis (loin pain), hypertension (35% late)
Adhesions rare even with recurrent peritonitis (vs surgical peritonitis)
35% develop HTN late in disease

physiology · background · low-yield

Other

Differential diagnosis: fever attacks — other autoimmune disease, rheumatic fever; polyserositis — SLE; acute abdomen — appendicitis, cholecystitis, renal colic; episodic arthritis — gout/pseudogout

Diagnostic Criteria

17 entries

ACR 1987 Rheumatoid Arthritis Classification (4/7)

Criterion	Asterisk
Morning stiffness ≥1 hr	*
Swelling in ≥3 joints	*
Swelling in hand joints	*
Symmetric joint swelling	*
Erosions or decalcification on hand X-ray
Rheumatoid nodules
Abnormal serum RF

Asterisked items must be present ≥6 weeks.

ACR/EULAR 2010 RA Criteria — 4 scored domains

Joint involvement
Serology
Acute-phase reactants
Symptom duration

Mandatory entry: at least one joint with unexplained synovitis.

ACR SLE Classification (4/11)

Criterion	Notes
Malar (butterfly) rash	Sparing nasolabial folds
Discoid rash
Photosensitivity
Oral / nasopharyngeal ulceration
Non-erosive arthritis
Serositis
Renal disorder
Haematological disorder	Anaemia + leukopenia + thrombocytopenia
Neurological disorder
Immunological	Anti-dsDNA, anti-Sm, or +ve APS
ANA positive

Newer sets: SLICC, ACR-EULAR.

Bohan & Peter — PM/DM 5-Pillar Diagnosis

1. Symmetrical proximal muscle weakness

2. Elevated muscle enzymes (CK, AST, ALT, LDH, aldolase)

3. Abnormal EMG

4. Muscle biopsy

5. Rash (Gottron's papules, heliotrope) — DM-specific

Inflammatory Back Pain Criteria (4 of 5, with back pain >3 months)

Age at onset <40 years
Insidious onset
Improvement with exercise
No improvement with rest
Pain at night (improvement on getting up)

Wegener's / GPA Triad

Upper airway (ENT)
Lung
Kidney
cANCA (anti-PR3) >95%

Churg–Strauss / EGPA Triad (Lanham 1984)

Asthma
Eosinophilia
Systemic vasculitis involving >2 extrapulmonary organs
pANCA (anti-MPO)

Reiter's Triad (Reactive Arthritis)

Arthritis
Urethritis
Conjunctivitis

HSP Tetrad

Symmetrical palpable purpura
GIT: colic, melaena, haematemesis
Joints: arthritis / arthralgia
Renal: haematuria + proteinuria
Biopsy: perivascular IgA deposition

A10

Behçet Diagnostic Criteria

Recurrent oral ulcers (mandatory) + 2 of:
Recurrent genital ulcers
Eye: uveitis, retinal vasculitis
Skin: erythema nodosum, pseudo-folliculitis, papulopustular lesions, acneiform nodules
Pathergy test (positive)

A11

Scleroderma Renal Crisis Diagnosis

New-onset BP >150/85
eGFR fall ≥30%
↑ creatinine
Microangiopathic haemolytic anaemia on smear
Retinopathy of acute hypertensive crisis
Proteinuria / haematuria
Oliguria or anuria
Flash pulmonary oedema
Renal biopsy: thrombotic microangiopathy
End-organ effect — especially encephalopathy

A12

CREST Acronym (limited SSc)

Calcinosis
Raynaud's
(O)Esophageal dysfunction
Sclerodactyly
Telangiectasia

A13

4 A's of Ankylosing Spondylitis (extra-articular)

Anterior uveitis (40%)
Aortic valve / root disease (AR)
Apical fibrosis
Amyloidosis (renal impairment, proteinuria, ↑ CRP/ESR)

A14

ACR–EULAR Vasculitis Criteria

Used for GCA and other vasculitides — clinical + investigations + imaging combined (specific scoring details not deck-detailed)

A15

ACR 1990 PAN — ≥3/10 criteria for classification

A16

FMF — Clinical Diagnosis

MEFV testing supports but cannot exclude — diagnosis is clinical

A17

ACR 2020 Gout — Initiation of ULT

Conditionally recommended to initiate hypouricaemic drugs DURING flare (with NSAID/colchicine/steroid cover)
ULT continued indefinitely (conditionally recommended)

---

## B. Severity / Activity Tools

Severity / Activity Tools

7 entries

Modified Schober's Test (AS)

Lecturer cites the test by name; threshold <20 cm = limited lumbar flexion
Full technique not detailed on slide — verify from textbook

Cervical mobility (AS)

Occiput-to-wall
Tragus-to-wall (cm)

SIJ Stress Test (AS)

FABER = Flexion + Abduction + External Rotation

Chest expansion (AS)

Normal: 3–15 cm

Glucocorticoid Dose Definitions (Prednisone equivalent/day)

Dose	mg/day
Low	≤7.5
Medium	>7.5 to ≤30
High	>30 to ≤100
Very high	>100
Pulse therapy	≥250 for 1 day or a few days

CRPS Clinical Stages

Stage	Duration	Hallmark
1 — Acute	1–3 months	Burning pain, localised oedema, warm + red, ↑ sweating
2 — Dystrophic	3–6 months	Soft-tissue oedema, skin thickening, muscle wasting + osteoporosis, cold + cyanotic
3 — Atrophic	Late	Contractures, severe osteoporosis, waxy trophic skin, brittle ridged nails

Sensitivity vs Specificity (test selection)

High sensitivity → negative test RULES OUT (screening)
High specificity → positive test RULES IN (confirmation)

---

## C. Anatomical & Severity Classifications

Anatomical & Severity Classifications

8 entries

WHO/ISN Lupus Nephritis Classes

Class	Pattern
1	Normal LM; minimal-change with Ig/complement deposits
2	Mesangial lupus nephritis
3	Focal proliferative GN (nephrotic risk)
4	Diffuse proliferative GN (nephrotic risk)
5	Membranous GN
6	Interstitial and tubular nephritis

Sacroiliitis X-ray Grading (axSpA)

Grade	Finding
0	Normal
I	Suspicious
II	Mild irregularity + sclerosis of articular surfaces, joint space preserved
III	Joint-space narrowing + intense irregularity + subchondral sclerosis
IV	Bilateral ankylosis (fusion)

Five Patterns of Psoriatic Arthritis

Pattern	Frequency
DIP-predominant (dactylitis "sausage finger")	55–70%
Asymmetrical oligoarthritis (<5 joints, large joints)	30–50%
Symmetrical polyarthritis (RA-like)	15–70%
Spondylitic (SI joints + spine)	5–33%
Arthritis mutilans (rare, deforming, destructive)	3–5%

Five Types of Cutaneous Lupus

Variant	Hallmark
Discoid lupus (DLE)	Photosensitive disfiguring lesion; ANA may be negative; 5% → SLE
Subacute cutaneous lupus (SCLE)	Photosensitive non-scarring; anti-Ro; mild illness; low renal
Systemic lupus (SLE)	Multisystem; ACR 4/11
Drug-induced lupus	Mild; anti-histone; resolves on drug cessation
Neonatal lupus	Born to anti-Ro+ mothers; transient rash; CHB 2%, mortality 15–25%

Five Ocular Manifestations of RA

Keratoconjunctivitis sicca (Sjögren's)
Episcleritis
Scleritis
Scleromalacia
Scleromalacia perforans (corneal melt — most severe)

Joint Distribution Percentages — RA

Joint group	% affected
MCPs, PIPs, MTPs	90%
Knees, wrists, ankles	80%
Hip, elbow	50%
Cervical spine	40%
TMJ, acromioclavicular	30%
Cricoarytenoid	10%

Vasculitis Classification by Vessel Size

Size	Disease
Large	Giant cell arteritis (GCA); Takayasu's
Medium	Polyarteritis nodosa (PAN); Kawasaki
Small/medium (ANCA-associated)	GPA (Wegener's); EGPA (Churg-Strauss); MPA
Small	HSP; Essential mixed cryoglobulinaemia
Variable	Behçet's

Joint-count Definitions (Acute Swollen Joint)

Term	Joints
Monoarthritis	1
Oligoarthritis	2–4
Polyarthritis	≥5
Acute	<14 days
Subacute	2–6 weeks
Chronic	>6 weeks

---

## D. Lab Pattern → Disease Pairings

Lab Pattern → Disease Pairings

5 entries

ANA Patterns → Associations + Next Test

Pattern	Disease association	Next test
Homogeneous	SLE, MCTD	Anti-dsDNA
Speckled (fine or coarse)	SLE, MCTD, Sjögren's	ENA (Ro, La, Sm, RNP)
Nucleolar	SLE, scleroderma	ENA (Scl-70)
Centromere	Scleroderma (limited / CREST)	No further
Cytoplasmic	Myositis	ENA (Jo-1)

ENA Antibody → Disease

Antibody	Disease
dsDNA, Sm	SLE
Ro, La	Sjögren's (Ro also SCLE + neonatal lupus)
U1-RNP	MCTD
Jo-1 (anti-histidyl-tRNA synthetase)	Polymyositis — ILD risk; anti-synthetase syndrome
Scl-70 (anti-topoisomerase I)	Diffuse cutaneous scleroderma
Anti-centromere	Limited cutaneous scleroderma (CREST)
Anti-PM/Scl	SSc-PM overlap
Anti-RNA polymerase	Scleroderma renal crisis (60%)
Lupus anticoagulant, anti-cardiolipin, anti-β2-GP	APS

Polarised Light Microscopy — Crystal Comparison

Feature	Gout	Pseudogout
Crystal	Monosodium urate (MSU)	Calcium pyrophosphate (CPPD)
Shape	Needle	Rhomboid
Birefringence	Strong	Weak
Colour parallel to axis	Yellow (negative birefringence)	Blue (positive birefringence)

ESR / CRP Patterns

Pattern	Suggests
ESR high + CRP high (rise together)	Active inflammation (RA, vasculitis, infection); drug reaction
ESR high + CRP normal	SLE; Sjögren's (polyclonal IgG); cancer
Both raised at presentation but CRP responds first to treatment	Acute-phase response (CRP > ESR for monitoring)

Vasculitis Antibody Signatures

Antibody	Disease
cANCA (anti-PR3)	GPA (Wegener's) — >95%
pANCA (anti-MPO)	MPA; EGPA
Cryoglobulins + low C4 + RF+	Essential mixed cryoglobulinaemia

---

## E. Lecture-cited Mnemonics

Lecture-cited Mnemonics

5 entries

CAN'T LEAP — Under-excretion Drugs Causing Hyperuricaemia

Letter	Drug
C	Cyclosporin
A	Alcohol
N	Nicotinic acid
T	Thiazides
L	Lasix (furosemide)
E	Ethambutol
A	Aspirin (low-dose)
P	Pyrazinamide

4 A's of Ankylosing Spondylitis

See A13.

CREST

See A12.

Reiter's Triad

See A8.

Wegener's / EGPA Triads

See A6, A7.

---

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Rheum Imaging — Erosive Arthropathies (RA · PsA · Gout · CPPD)

Rheumatoid arthritis

MARGINAL erosions at the bare area (ulnar styloid); symmetric MCP + PIP, DIP spared; juxta-articular osteopenia
Carpal collapse/amalgamation; arthritis mutilans; protrusio acetabuli; atlanto-axial subluxation (ADI ≤3 mm adults)

Psoriatic arthropathy

Pencil-in-cup; DIP involvement; large eccentric erosions; tuft resorption; periostitis; NORMAL mineralisation

Gout vs CPPD

Gout: punched-out erosions with OVERHANGING edges; PRESERVED joint space; NO osteopenia; asymmetric; soft-tissue tophi
Pseudogout/CPPD: chondrocalcinosis — linear calcification of menisci + TFCC of the wrist

Rheum Imaging — Spondyloarthropathy & Sacroiliitis

Ankylosing spondylitis

Syndesmophytes: vertical, thin, marginal, symmetric; bamboo spine; dagger sign (interspinous/supraspinous ligament ossification); Romanus shiny-corner (enthesitis of annulus fibrosus)

AS vs PsA

AS: bilateral symmetric SIJ + marginal symmetric syndesmophytes + more ligamentous ossification
PsA: unilateral/asymmetric SIJ + coarse non-marginal syndesmophytes + DIP disease

Sacroiliitis radiographic grading

I hazy margins (no erosion/sclerosis) · II erosions + subchondral sclerosis, normal joint width · III partial ankylosis · IV total ankylosis
Atlantodental interval (ADI): normal ≤3 mm adults / ≤5 mm children

Rheum Imaging — CTD, OA & Haemophilic

Connective tissue disease

SLE (Jaccoud's): REDUCIBLE, non-erosive ulnar deviation + swan-neck; no joint-space narrowing or erosions
Scleroderma: acro-osteolysis + subcutaneous/periarticular calcinosis; pulmonary fibrosis (basal reticular)
Dermatomyositis: INTRAMUSCULAR calcification (distinguishes from scleroderma's cutaneous); linear; children

Osteoarthritis

Kellgren–Lawrence grades 0–4; Heberden (DIP) / Bouchard (PIP) nodes; joint-space narrowing + osteophytes + subchondral sclerosis + cysts

Haemophilic arthropathy

Widened intercondylar notch + squared patella/condyles (Jordan's sign) + ballooned distal femur + joint-space narrowing

NSAIDs & Steroids

2 entries

NSAIDs

Mechanism. Block COX-1 and COX-2 → ↓ prostaglandins → ↓ inflammation, pain, fever. COX-1 also protects the stomach and supports platelets → GI + bleeding SEs.

Classes (know the classes, not individual drugs):

Acetic acid: diclofenac, indomethacin, ketorolac, sulindac, nabumetone
COX-2 inhibitors: celecoxib
Fenamates: mefenamic acid, meclofenamate
Oxicams: meloxicam, piroxicam
Propionic acids: ibuprofen, naproxen, ketoprofen, flurbiprofen
Salicylates: aspirin, diflunisal

Main indications. Inflammatory arthritis · osteoarthritis · crystal arthropathies (gout, pseudogout) · tendinitis / tenosynovitis · pleurisy · pericarditis.

Main side effects.

GI: dyspepsia, gastroduodenal ulcers, bleeding, perforation
CV: oedema, HTN, CHF, MI, stroke, thrombotic events
Renal: electrolyte imbalance, Na retention, ↓ GFR, nephrotic syndrome, acute interstitial nephritis, papillary necrosis, CKD

Prescribing rule: shortest duration, lowest effective dose, monitor GI/renal/CV.

Glucocorticoids

Mechanism. Genomic (change in gene expression) — most important in low-dose therapy. Transactivation drives most SEs; transrepression drives most anti-inflammatory effect.

Dose definitions (prednisone equivalent/day) — cross-ref B5.

Dose	mg/day
Low	≤7.5
Medium	>7.5 to ≤30
High	>30 to ≤100
Very high	>100
Pulse	≥250 × 1 day or a few days (life-threatening emergency — e.g. to avoid blindness in GCA)

Main indications.

CTDs: SLE, DM/PM, Sjögren's (high or low dose per indication)
Inflammatory arthritis: RA, JIA (mainly low dose)
Crystal arthropathies: acute attack OR low-dose prophylaxis (gout)
OA: mainly intra-articular
Sero-negative SpA: mainly for peripheral arthritis or uveitis

Routes. PO · IV pulse · IM · intra-articular.

Precautions / mandatory add-ons.

Lifestyle: no smoking, no excess alcohol, weight-bearing exercise
Monitor BP, blood sugar, lipid profile, CVS assessment
Dietary + supplemental Ca and vit D
Bisphosphonate for adults ≥40 yr on long-term GC at moderate/high fracture risk
Do NOT combine GC with NSAIDs → high peptic ulcer risk

Main side effects. Osteoporosis (bisphosphonate cover if >3 mo) · diabetes · HTN · dyslipidaemia · peptic ulcer (esp. with NSAIDs) · infection risk.

Conventional DMARDs

8 entries

T3a

Methotrexate (MTX) — first-line RA

Mechanism. DHFR inhibitor → ↓ purine synthesis → S-phase arrest → apoptosis.

Route. PO, SC, IM. Once weekly (long-acting).

Cover. Folic acid 1 mg/day (every day except injection day — if forgotten, could be fatal).

Indications.

RA — most effective DMARD (mono or combo)
JIA
Sero-negative SpA (peripheral arthritis only)
PM/DM
SLE — cutaneous + articular
ANCA-associated vasculitis
Uveitis

Main SEs. Nausea/vomiting (commonest) · hepatotoxicity · oral ulcers · cytopenias (leucopenia, thrombocytopenia, megaloblastic anaemia) · pneumonitis.

Precautions. Avoid if CrCl <30 ml/min · teratogenic — stop 3 mo before conception · baseline + 8–12 wk CBC/LFT/KFT.

T3b

Leflunomide (LEF)

Mechanism. Dihydroorotate dehydrogenase inhibitor → ↓ pyrimidine synthesis. Hits activated B cells > T cells.

Dose. PO 20 mg daily.

Indications. RA · JIA · PsA · anywhere MTX is used but not tolerated.

Main SEs. Hepatotoxicity (slightly less than MTX) · teratogenic — extremely long half-life, detectable for up to 2 yr; requires cholestyramine washout · N/V/diarrhoea · HTN · rare peripheral neuropathy.

T3c

Hydroxychloroquine (HCQ) — least toxic DMARD

Mechanism. Accumulates in lysosomes → ↑ lysosomal pH · ↓ IL-1/IL-6/IFN · ↓ lipids · ↓ platelet aggregation.

Indications.

SLE — very important for preventing flares
DLE + DM skin
RA + palindromic rheumatism
APS — anti-platelet effect
Sjögren's

Almost every SLE/RA patient takes it — except those with retinal disease.

Safe in pregnancy.

Main SEs. Retinal toxicity (maculopathy) — must do baseline + annual fundus exam · skin hyperpigmentation (gray-black) · hair bleaching · myopathy.

⚠ Do NOT give in psoriasis — worsens rash.

T3d

Sulfasalazine (SSZ / Salazopyrine)

Mechanism. Broken down by colonic bacteria → sulfapyridine (90% absorbed, active) + 5-ASA (stays in bowel).

Indications.

Sero-negative SpA — AS, PsA, ReA, EntA
RA
JIA

Safe in pregnancy + lactation (recently approved, but the lecturer avoids it — causes neonatal jaundice, crosses BBB → mental retardation + convulsions).

Main SEs. Skin rash · haemolysis in G6PD deficiency · neutropenia · azoospermia (careful in males) · pulmonary infiltrates · LFT rise.

Not for: SLE, APS, vasculitis.

T3e

Azathioprine (AZA)

Mechanism. Prodrug → 6-mercaptopurine → ↓ de novo purine synthesis → cytotoxicity.

Metabolised by xanthine oxidase + TPMT. Low TPMT activity → severe myelosuppression at 4–10 wk.

⚠ DO NOT combine with gout drugs (allopurinol / febuxostat) — XO inhibition raises AZA to fatal levels.

Indications.

SLE — maintenance in LN, serositis, cutaneous, haematological
Behçet's
ANCA-associated vasculitis
PM/DM
Mild ILD in systemic sclerosis
Steroid-sparing generally

Safe in pregnancy.

Main SEs. Bone marrow suppression · infections (esp. herpes zoster) · hepatotoxicity · N/V · rash · hypersensitivity (fever/rash/hepatitis/renal failure) · pancreatitis.

T3f

Mycophenolate mofetil (MMF)

Mechanism. Prodrug → MPA → ↓ B and T lymphocyte proliferation + migration.

Indications.

Lupus nephritis induction — diffuse proliferative + membranous
Cutaneous lupus (DLE + SCLE)
Small-vessel vasculitis
SSc ILD
Myositis
Uveitis

Main SEs. GI (esp. diarrhoea) · leucopenia · anaemia · infections · hepatotoxicity · teratogenic — stop 3 mo pre-conception.

T3g

Cyclophosphamide (CYC)

Mechanism. Alkylating agent → DNA breaks → apoptosis. Hits rapidly-dividing cells; B > T cells.

Dose. PO or IV 0.5–1 g/m² monthly, or 500 mg fortnightly × 6 doses. ↓30% if CrCl <30.

Indications.

SLE induction — LN focal/diffuse proliferative, CNS lupus
Systemic vasculitis
SSc ILD
Neuro-Behçet's (parenchymal)

Main SEs. Bone marrow suppression · infection · infertility (ovarian failure 30–70%; 100% if >30 yr — offer egg freezing) · haemorrhagic cystitis · bladder cancer · pneumonitis · pulmonary fibrosis · teratogenic.

T3h

Cyclosporine (CsA)

Mechanism. Inhibits T-cell activation via ↓ IL-2 transcription.

Indications. Uveitis · SLE (refractory LN, membranous) · PM/DM · psoriasis + PsA · RA.

Main SEs. ↑ creatinine + hyperkalaemia (commonest, reversible) · HTN · anaemia · hyperuricaemia + gout · headache · tremors.

Safe in pregnancy.

Pregnancy-safe DMARDs (summary): corticosteroids · HCQ · AZA · SSZ · CsA.

Biological DMARDs

5 entries

Used after failure of conventional DMARDs. Engineered to target specific cytokines or immune cells.

T4a

Anti-TNF agents (5 approved since 2000)

Infliximab (IV)
Adalimumab (SC)
Certolizumab pegol (SC)
Etanercept (SC) — fusion protein (TNF-R2 p75 + Fc IgG1); the rest are mAbs
Golimumab (SC)

Indications. RA · JIA · sero-negative SpA (PsA, AS) · Crohn's / UC · Behçet's (uveitis, vascular, neuro).

⚠ NOT for SLE — can cause drug-induced lupus.

Main SEs. Injection-site reactions · infection (esp. TB reactivation) · drug-induced lupus (anti-dsDNA + ANA) · LFT abnormalities · cytopenias · ↑ risk lymphoma + non-melanoma skin cancer.

Contraindications. Untreated TB · ILD (pulmonary fibrosis) · demyelinating disease · CHF NYHA III/IV.

Pre-start screen (mandatory). HBV + HCV serology + tuberculin skin test.

T4b

Anti-IL-1 — Anakinra

Mechanism. Recombinant IL-1 receptor antagonist. SC daily (IV possible).

Indications. RA · systemic-onset JIA · auto-inflammatory syndromes (FMF).

Main SEs. Injection-site reactions (daily use) · URT + viral infections · neutropenia.

T4c

Anti-IL-6 — Tocilizumab

Mechanism. Humanised anti-IL-6R mAb. IV monthly.

Indications. RA · systemic-onset JIA · auto-inflammatory syndromes (FMF) · steroid-sparing in GCA.

Main SEs. Infections (URTI, pharyngitis) · neutropenia · ↑ total cholesterol/LDL/HDL/TG · ↑ LFTs.

T4d

Abatacept — CTLA-4 fusion protein

Mechanism. Blocks co-stimulation (CD80/86 on APC ↔ CD28 on T-cell). IV.

Indications. RA · active PsA.

Main SEs. ↑ infection risk.

⚠ Contraindicated in COPD.

T4e

Rituximab (RTX) — anti-CD20

Mechanism. Depletes B cells → ↓ differentiation into plasma cells → ↓ autoantibodies.

Indications. ANCA-associated vasculitis · Sjögren's · seropositive RA · SLE · SSc ILD.

Main SEs. Infections (rare PML) · infusion reactions · HBV reactivation · rare vasculitis / psoriasis.

Targeted Synthetic DMARDs

1 entry

JAK inhibitors

Small oral molecules that block intracellular JAK-STAT signalling.

Examples.

Tofacitinib — JAK1/JAK3 (also JAK2, TYK2 to lesser extent)
Baricitinib — JAK1/JAK2
Upadacitinib — selective JAK1

Indications.

RA refractory to conventional DMARDs
Poly-articular JIA
Axial SpA
PsA
DM
Under investigation: SLE, SSc, Sjögren's

Main SEs / safety concerns.

Dyslipidaemia
Neutropenia, thrombocytopenia
Infections — esp. TB + herpes zoster
↑ VTE risk

EULAR 2022 — consider risk factors before prescribing:

Age >65
Current/past smoking
CV risk factors (DM, obesity, HTN)
Malignancy history (except successfully-treated non-melanoma skin cancer)
Thromboembolic risk

Step-wise Treatment Protocols

3 entries

T6a

RA — step-wise

Conventional DMARDs (MTX first)
Biological or targeted-synthetic DMARDs if failed

Low-dose GC + NSAIDs = bridging only (start of treatment or during flares).

T6b

SLE — step-wise

HCQ + sun protection for every patient (unless contraindicated).

Then organ-specific:

Arthritis → MTX, leflunomide
Myositis → MTX or AZA
Cutaneous → MMF, MTX
Severe (lupus nephritis, neuropsychiatric) → cyclophosphamide OR MMF

Biologicals if conventional therapy fails.

T6c

Lupus nephritis — induction & maintenance

Phase	Drug	Preference
Induction	Cyclophosphamide or MMF	MMF if ovarian reserve to preserve (age >30, single, no offspring)
Maintenance	Azathioprine or MMF	—