Expansion
Large, medium and small vessel vasculitis, each with characteristic diseases
Mnemonic
Classified by vessel size, which predicts the presentation:
Large vessel
- Giant cell arteritis - over 50, headache, jaw claudication, visual loss, raised ESR
- Takayasu arteritis - young women, absent pulses, the “pulseless disease”
Medium vessel
- Polyarteritis nodosa - hepatitis B associated, spares the lungs, renal microaneurysms
- Kawasaki disease - children, coronary artery aneurysms
Small vessel, ANCA associated
- Granulomatosis with polyangiitis - cANCA / PR3. Upper airway, lungs, kidneys. Saddle nose
- Microscopic polyangiitis - pANCA / MPO. Lungs and kidneys, no granulomas
- Eosinophilic granulomatosis with polyangiitis - asthma, eosinophilia, pANCA
Small vessel, immune complex
- IgA vasculitis (Henoch-Schonlein) - children, purpuric rash on the buttocks and legs, arthralgia, abdominal pain, nephritis
- Cryoglobulinaemic, associated with hepatitis C
“Large vessel gives pulse and vision problems, medium gives aneurysms and infarcts, small gives purpura and glomerulonephritis.”
Expansion
Large vessel
- Giant cell (temporal) arteritis: over 50, headache, jaw claudication, visual loss, raised ESR, association with polymyalgia rheumatica. Skip lesions on biopsy
- Takayasu arteritis: young women, aortic arch, absent pulses
Medium vessel
- Polyarteritis nodosa: hepatitis B association, renal and gut involvement, microaneurysms on angiography, spares the lungs, ANCA negative
- Kawasaki disease: children, fever over 5 days, conjunctivitis, rash, cervical lymphadenopathy, strawberry tongue, and coronary artery aneurysms
Small vessel, ANCA associated
- Granulomatosis with polyangiitis: cANCA (PR3). Upper and lower respiratory tract plus glomerulonephritis; saddle nose deformity
- Eosinophilic granulomatosis with polyangiitis: pANCA (MPO), asthma, eosinophilia
- Microscopic polyangiitis: pANCA, pulmonary-renal syndrome without granulomas
Small vessel, immune complex
- IgA vasculitis (Henoch-Schonlein): children, purpuric rash on buttocks and legs, arthritis, abdominal pain, IgA nephropathy
- Cryoglobulinaemic vasculitis, associated with hepatitis C
Fibrinoid necrosis is the histological hallmark across the group.