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ANCA-Associated Vasculitis (Comprehensive Management)

A spectrum of small-vessel necrotizing vasculitides — granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis — defined by anti-neutrophil cytoplasmic antibodies and treated with induction immunosuppression followed by maintenance and relapse prevention.

Written by: Saygı Hospital Health Guide Editorial Board
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This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is ANCA-Associated Vasculitis (Comprehensive Management)?

ANCA-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), characterized pathologically by pauci-immune necrotizing vasculitis of small to medium vessels.

The disease is mediated by anti-neutrophil cytoplasmic antibodies, predominantly directed against proteinase 3 (PR3-ANCA, often c-ANCA pattern, more common in GPA) or myeloperoxidase (MPO-ANCA, often p-ANCA pattern, more common in MPA and renal-limited disease).

Modern management is divided into induction (rituximab and/or cyclophosphamide combined with high-dose glucocorticoids and avacopan in severe disease) and maintenance therapy (rituximab or azathioprine), with risk stratification, ANCA monitoring, infection prophylaxis, and lifelong cardiovascular and bone-health follow-up.

Symptoms

Persistent rhinitis, recurrent sinusitis, nasal crusting, epistaxis, or saddle-nose deformity (especially in GPA)
Hemoptysis, pulmonary nodules or cavities, alveolar hemorrhage, or progressive dyspnea
Rapidly progressive glomerulonephritis: hematuria, proteinuria, red cell casts, and rising creatinine
Multifocal mononeuritis multiplex, palpable purpura, or non-healing skin ulcers
Constitutional symptoms: prolonged fever, weight loss, night sweats, arthralgia
Late-onset asthma with eosinophilia, cardiomyopathy, or peripheral neuropathy in EGPA

Risk Factors

Older age, peak incidence in the sixth and seventh decades
Genetic predisposition (HLA-DPB1*04:01 in PR3-ANCA, HLA-DQ in MPO-ANCA)
Silica exposure and occupational dust exposure (farming, mining)
Drug-induced AAV: hydralazine, propylthiouracil, levamisole-contaminated cocaine
Coexistence of asthma, eosinophilia, and atopy (EGPA)
Prior infection with Staphylococcus aureus (chronic nasal carriage in GPA)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained pulmonary-renal syndrome (hemoptysis with rising creatinine) — emergent nephrology and rheumatology evaluation
  • Persistent sinonasal symptoms (months) plus systemic features such as fever or arthralgia
  • New-onset asthma with eosinophilia plus mononeuritis multiplex or skin lesions
  • Active urinary sediment in a patient with constitutional symptoms — urgent kidney biopsy and ANCA testing
  • Suspected relapse on rituximab maintenance: rising ANCA titer plus new clinical symptoms

Treatment Methods

01
Induction with rituximab (preferred for relapse, organ-threatening disease, fertility preservation) or cyclophosphamide plus high-dose glucocorticoids
02
Avacopan, an oral C5a receptor antagonist, to reduce glucocorticoid burden and improve sustained renal recovery in severe disease
03
Plasma exchange in selected patients with severe alveolar hemorrhage or with overlapping anti-GBM disease
04
Maintenance immunosuppression with rituximab every 4 to 6 months or azathioprine, individualized by relapse risk
05
Adjunctive care: Pneumocystis prophylaxis, bone protection, vaccination, cardiovascular risk reduction, and infection surveillance

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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