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ANCA-associated vasculitis
ANCA-associated vasculitis is a rare inflammatory disease of the blood vessels that can compromise tissue perfusion and damage multiple organs
Prevalence
~40–50 / 100,000
US Estimated
~10–30 / 100,000
Europe Estimated
Age of Onset
Adults
ICD-10
I77.82
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
ANCA-Associated Vasculitis (AAV) comprises a group of autoimmune diseases affecting small blood vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA)
FACT
Onset typically occurs in middle age, with a peak incidence between 50 to 70 years old
FACT
Symptoms may include constitutional symptoms like fatigue, fever, weight loss, and general malaise
FACT
Common clinical manifestations involve the respiratory tract (sinusitis, pulmonary infiltrates), kidneys (hematuria, proteinuria), and systemic involvement (nerve damage, skin lesions)
FACT
Diagnosis involves clinical evaluation, serological testing for ANCA antibodies, imaging studies, and biopsy to confirm vasculitis
Interest over time
Google searches
Common signs & symptoms
Constitutional
- Fever, weight loss, fatigue
ENT involvement
- Chronic sinusitis, otitis media, nasal crusting or epistaxis
Pulmonary manifestations
- Cough, dyspnea, pulmonary infiltrates, alveolar hemorrhage (subtype-dependent)
Renal involvement
- Hematuria, proteinuria, rapidly progressive glomerulonephritis
Skin findings
- Palpable purpura, livedo, ulcers
Neurologic involvement
- Mononeuritis multiplex or peripheral neuropathy
Clinical presentation varies across GPA, MPA, and EGPA
Current treatments
Induction therapy
Systemic glucocorticoids combined with rituximab or cyclophosphamide, depending on disease severity and organ involvement
Steroid-sparing therapy
Avacopan (C5a receptor inhibitor) in selected patients, with guideline-recommended limited duration
Maintenance therapy
Rituximab or conventional immunosuppressive agents to reduce relapse risk