Disease Directory ANCA-Associated Vasculitis
Immune

ANCA-Associated Vasculitis

Also known as: AAV, granulomatosis with polyangiitis, GPA, MPA, EGPA, Wegener granulomatosis

Prevalence

150-300 in 1,000,000

Onset

Middle to older adulthood; peak in 6th-7th decade

Type

Small vessel systemic vasculitis (ANCA mediated)

About ANCA-Associated Vasculitis

ANCA-Associated Vasculitis encompasses granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), unified by neutrophil-activating autoantibodies targeting proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA), causing necrotizing inflammation of small to medium vessels. Renal involvement with rapidly progressive glomerulonephritis and pulmonary manifestations including alveolar hemorrhage are the most immediately life-threatening features. Rituximab and cyclophosphamide have equivalent efficacy for induction, while avacopan (a C5a receptor inhibitor) has received approval as a steroid-sparing agent.

Common Clinical Features

Rapidly progressive glomerulonephritis with hematuria and casts Pulmonary capillaritis with alveolar hemorrhage Sino-nasal destruction (saddle-nose, chronic otitis in GPA) Peripheral neuropathy (mononeuritis multiplex) Orbital pseudotumor and proptosis (GPA) Asthma and peripheral eosinophilia (EGPA) Purpura and cutaneous ulceration

Clinical Trial Eligibility Tips

What to know before applying to ANCA-Associated Vasculitis trials.

ANCA serotype (PR3 vs MPO) and AAV subtype (GPA, MPA, EGPA) determine trial eligibility; many trials enroll specific subtypes only — know your ANCA titer and subtype before applying

Avacopan and other complement-targeting trials require documented active disease with BVAS score confirmation; inactive or remission-phase patients are not eligible for induction trials

Renal function trajectory (eGFR trend, dialysis dependence) critically affects eligibility; dialysis-dependent patients may be excluded from some trials while specifically enrolled in others

Patient Resources

Patient Organization

Vasculitis Foundation

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Natural History Registry

VCRC Patient Contact Registry

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Orphanet

European reference resource for rare diseases (ORPHA:156152)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting ANCA-Associated Vasculitis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for ANCA-Associated Vasculitis. Updated daily.

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