Large, medium, small vessel — by size, presentation, and ANCA status.
| Disease | Demographics | Features | Diagnosis | Treatment |
|---|---|---|---|---|
| Giant cell (temporal) arteritis | Women >50; polymyalgia rheumatica association | New headache + jaw claudication + scalp tenderness + visual loss (AION) | ESR >50, CRP↑; temporal artery biopsy (segmental — get long sample) | HIGH-DOSE prednisone IMMEDIATELY before biopsy (prevent blindness); add tocilizumab |
| Takayasu arteritis | Young Asian women <40 | 'Pulseless disease': diminished arm pulses + arm claudication + BP difference; aortic arch + branches | MRA/CTA | Glucocorticoids |
| Disease | Features | Associations | Treatment |
|---|---|---|---|
| Polyarteritis nodosa (PAN) | Constitutional + skin (livedo, ulcers, nodules) + peripheral neuropathy (mononeuritis multiplex) + GI + renal (HTN) + cardiac. SPARES lungs | Hepatitis B (30%); ANCA NEGATIVE; rosary bead aneurysms on angiogram | Steroids ± cyclophosphamide; treat HBV |
| Kawasaki | Child + fever ≥5 days + 4 of 5 (conjunctivitis, mucositis/strawberry tongue, rash, extremity changes, cervical LAD) | Coronary aneurysms if untreated | IVIG + high-dose aspirin within 10 days; echo monitoring |
| Buerger (thromboangiitis obliterans) | Heavy male smoker <45; distal extremity ischemia, ulcers, gangrene; superficial thrombophlebitis | SMOKING — strongly tied | STRICT smoking cessation (only treatment) |
| Disease | ANCA | Features | Treatment |
|---|---|---|---|
| GPA (Granulomatosis with polyangiitis / Wegener) | c-ANCA (anti-PR3) | Upper airway (sinusitis, saddle nose) + lower airway (cavitary nodules) + glomerulonephritis | Steroids + rituximab (or cyclophosphamide) |
| MPA (Microscopic polyangiitis) | p-ANCA (anti-MPO) | Pulmonary-renal syndrome WITHOUT upper airway; no granulomas | Steroids + rituximab/cyclophosphamide |
| EGPA (Eosinophilic granulomatosis / Churg-Strauss) | p-ANCA (anti-MPO) in 50% | Asthma + eosinophilia + sinusitis + neuropathy + skin | Steroids ± rituximab/mepolizumab |
| Disease | Features | Treatment |
|---|---|---|
| IgA vasculitis (Henoch-Schönlein purpura) | Child after URI; palpable purpura on buttocks/legs + arthralgias + abdominal pain (intussusception) + IgA nephritis | Supportive; steroids for severe abdominal pain or renal involvement |
| Cryoglobulinemic vasculitis | Hepatitis C; palpable purpura + arthralgia + neuropathy + glomerulonephritis | Treat HCV (DAAs); rituximab if severe |
| Behçet disease | Recurrent oral + genital ulcers + uveitis + erythema nodosum + pathergy (papule at needle stick site) | Steroids; immunosuppression |