Small vessel vasculitis (ANCA-associated, IgA vasculitis, anti-GBM)

Condition

Features

Treatment

Granulomatosis with polyangiitis (GPA)

Upper and lower respiratory and renal disease; c-ANCA (PR3)

A: Prednisolone 1 mg/kg/day (max 60–80 mg) AND S: cyclophosphamide 2 mg/kg/day PO or 0.5–1 g/m² IV every 2–4 weeks, OR S: rituximab 375 mg/m² weekly × 4 ± plasma exchange

Microscopic polyangiitis (MPA)

Pulmonary capillaritis, RPGN; p-ANCA (MPO)

Prednisolone 1 mg/kg/day AND cyclophosphamide OR rituximab as for GPA ± plasma exchange

Eosinophilic GPA (EGPA)

Asthma, eosinophilia, neuropathy ± renal disease

Prednisolone 1 mg/kg/day then taper AND cyclophosphamide, OR rituximab for severe disease

IgA vasculitis (HSP)

Palpable purpura, abdominal pain, arthralgia, IgA deposits

A: Prednisolone 1–2 mg/kg/day (max 60 mg) for severe GI or renal disease ± methylprednisolone IV 500–1000 mg daily × 3 (severe nephritis)

Cryoglobulinaemic vasculitis

Purpura, neuropathy, renal disease, low complement

Prednisolone 1 mg/kg/day AND S: rituximab 375 mg/m² weekly × 4 or 1 g on days 1 and 15 ± cyclophosphamide ± plasma exchange

Anti-GBM disease (Goodpasture)

RPGN, pulmonary haemorrhage, anti-GBM antibodies

D: Methylprednisolone IV 500–1000 mg daily × 3, then prednisolone 1 mg/kg/day AND cyclophosphamide AND plasma exchange daily or alternate days for 10–14 sessions

Hypocomplementaemic urticarial vasculitis

Urticarial rash, low complement, nephritis

Prednisolone 0.5–1 mg/kg/day AND methotrexate 15–25 mg weekly OR cyclophosphamide