Vnitr Lek 2025, 71(7):408-413 | DOI: 10.36290/vnl.2025.076

ANCA-associated vasculitides - diagnostic and therapeutic options

Zdenka Hrušková, Vladimír Tesař

Klinika nefrologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice v Praze

Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis are three rare diseases ranked among ANCA-associated vasculitides. The most commonly affected organs include the ENT area, lungs and kidneys. The diagnosis is based on the clinical picture, positivity of ANCA antibodies (directed against proteinase 3, PR3-ANCA, or myeloperoxidase, MPO-ANCA) and/or biopsy verification. Early recognition of the disease and prompt initiation of therapy are crucial for the patients´ prognosis. Nowadays, treatment usually consists of corticosteroids and either cyclophosphamide or rituximab, or both. In severe forms of the disease, the addition of plasma exchange can still be considered. A newer treatment option that has the potential to completely replace corticosteroids is avacopan, a complement C5a receptor inhibitor. In the treatment of EGPA, therapy directed against interleukin 5 may also be used.

Keywords: ANCA, avacopan, corticosteroids, cyclophosphamide, rituximab, vasculitis.

Accepted: October 16, 2025; Published: October 31, 2025  Show citation