Overview of ANCA-associated vasculitides

Yates, 2016

Antineutrophil cytoplasmic antibodies are well known by the acronym ANCA. ANCA-associated vasculitides are a group of small-vessel vasculitides with some similarities in clinical features and management. The 3 ANCA-associated vasculitides are:

Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss vasculitis)
Granulomatosis with polyangiitis (GPA; formerly known as Wegener granulomatosis)
Microscopic polyangiitis (MPA).

These conditions are described in detail in individual sections below. Clinical features of systemic vasculitides lists their clinical features.

ANCA-associated vasculitides are uncommon, variable, unpredictable and life-threatening conditions that require specialist management. Seek urgent specialist advice if there is a high index of suspicion for ANCA-associated vasculitis to determine if immediate intensive induction therapy is required. Rheumatologists strongly encourage direct contact by general practitioners to expedite referral or obtain advice on treatment (eg before starting oral corticosteroids).

Note: Seek urgent specialist advice if there is a high index of suspicion for ANCA-associated vasculitis to determine if immediate treatment is required.