TNF-α inhibitors in the management of refractory granulomatosis with polyangiitis: A report of two cases

Abstract

Granulomatosis with polyangiitis (GPA) is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that causes granulomatous necrotizing inflammation of small blood vessels. The goal of AAV treatment is to create a long-term remission. For inducing remission, the European League Against Rheumatism recommends using glucocorticoids (GCs) in combination with either cyclophosphamide (CYC) or rituximab (RTX). Despite this, in 10-40% the disease is resistant to initial treatment. In this report, we present two cases of refractory GPA that successfully achieved long-term remission using tumor necrosis factor-α inhibitors. The first case is a 27-year-old woman with GPA with involvement of upper airway, breast and central nervous system with active disease despite treatment with high dose GCs, CYC and RTX, successfully treated with infliximab and the disease was in complete remission for 54 months. The second case is a 35-year-old man with GPA with arthritis, lung cavitary lesions and nephritis resistant to treatment with high dose GCs, CYC and RTX, treated with adalimumab and the disease was in complete remission for 40 months.