Much More than just a Blocked Nose

The ANCA-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). AAV can progress rapidly and lead to organ-threatening or life-threatening complications within a short time. The diseases are rare. However, they must be included in the differential diagnostic considerations in cases of unclear organ manifestations and elevated inflammation parameters. The diagnosis and treatment of the disease are explained using a case report of a girl with GPA.