Background:

Granulomatosis with polyangiitis (Wegener’s granulomatosis) is a rare, multisystem and autoimmune disease of unknown etiology. Its primary features include pauci-immune necrotizing granulomatous inflammation of small and medium vessel vasculitis; its prevalence is 1-9/100,000 and the typical age of onset is between 35 and 55 years. As a multisystem disease, it can affect any area of the body, with predilection for the lungs and kidneys.

Case report:

57-year-old female with one month of evolution characterized by clinical evidence of respiratory and renal failures requiring intubation and hemodialysis; she had a poor outcome upon her admission to the ICU, with positive c-ANCA. She was treated with rituximab, high doses of glucocorticoids and plasma exchange, achieving the recovery of her kidney and lung functions.

Conclusions:

Manifestations of granulomatosis with polyangiitis may occur in a fulminant way; 50% of chest imaging studies show cavitated nodules, which, coupled with crescentic necrotizing glomerulonephritis and the presence of c-ANCA (sensitivity of 96-98%), support the diagnosis, which is confirmed by biopsy. Current treatment with rituximab, corticosteroids and plasma exchange to induce remission increases five-year survival in 75% of the patients.