Table 1.

Primary Vasculitis: Summary of main features and treatments

Condition	Size of affected vessel	Symptoms	Treatments
Behcet's syndrome	Small	Mouth and genital ulcers with scarring, erythema nodosum, folliculitis and cutaneous pustular vasculitis. Uveitis and pathergy present. Age 20s–30s	Triamcinolone acetonide cream, NSAIDs, colchicine, steroids, azathioprine, mycophenolate, methotrexate. Anti‐TNF and rituxumab
Henoch Schönlein purpura	Small	Abdominal pain, arthritis, purpura on buttocks, legs and feet. Haematuria	Hydration, rest and analgesics; majority of cases are self‐limiting. Steroids. Azathioprine. Cyclophosphamide. Plasmapheresis
Cryoglobulinaemia	Small	Purpura on lower extremities, polyarthritis, weakness, associated with hepatitis B/C and myeloma, arthritis, weakness and neuropathy	Steroids +/− cyclophosphamide or rituximab. Treat any underlying cause: anti‐viral therapy for hepatitis C or anti‐retroviral therapy for HIV. Consider plasma exchange
Microscopic polyangiitis	Small/medium	Fever, purpura and LR. Proteinura and haematuria. Haemoptysis and fever	Steroids with methotrexate. Cyclophosphamide or rituximab 9.
Buerger's disease (Thromboangiitis obliterans)	Small/medium	Extremities affected with pain and ulcers. Specific geographical distribution being more prevalent in Asia	Smoking cessation is the definitive treatment
Eosinophilic granulomatosis with polyangiitis (Churg‐Strauss)	Small/medium	Cutaneous granulomata along elbows and fingers ‘Churg Strauss nodules’. Asthma, allergic rhinitis, nasal polyps neuropathy	Steroids, cyclophosphamide. Azathioprine, methotrexate, leflunomide, mycophenolate mofetil, IV immunoglobulins, hydroxyurea, rituximab, interferon‐alpha 9
Granulomatosis with polyangiitis (Wegener's granulomatosis)	Small/medium	Palpable purpura, necrotic papules, LR, pyoderma gangrenosum. Epistaxis, painless oral ulcers, chronic sinusitis, nasal stuffiness. Haematuria or red cell casts. Neuropathy	Steroid, cyclophosphamide, plasma exchange, rituximab 10
Polyarteritis nodosa	Medium	Purpura, ‘punched out’ skin ulcers in the lower extremities, myalgia and arthritis, abdominal haemorrhage, fever, weight loss, renal infarction	Steroids, cyclophosphamide, azathioprine, methotrexate 10
Kawasaki disease	Medium	Erythema of palms and soles with desquamation, macular‐papular rash, purpura. Persistent fever, bilateral conjunctival injection, anterior uveitis, strawberry tongue.	Intravenous immunoglobulin, aspirin
Giant cell arteritis	Large/medium	Age >50, tender scalp, jaw claudication, absent neck and arm pulses, blurred or double vision, blindness. Associated with PMR	Steroids, methotrexate
Takayasu's arteritis	Large	Aorta, young women. Numb and cold extremities, decreased or absent pulses, hypertension, headaches and visual disturbances	Steroids, methotrexate, leflunomide, mycophenolate mofetil, tocilizumab/anti‐TNF agents, azathioprine, cyclophosphamide. Treatment of hypertension, heart failure or angioplasty for aortic stenosis

HIV, human immunodeficiency virus; LR, livedo reticularis; NSAIDs, Non‐Steroidal Anti‐Inflammatories.