Systemic vasculites is a heterogeneous group of diseases in which inflammation of the vascular wall is the main morphological feature, and clinical manifestations depend on type, size, localization of the affected vessels and systemic inflammation activity. Vasculites accompanied by the appearance of anti-neutrophil cytoplasmic antibodies (ANCA-associated vasculites, AAVs) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic granulomatous polyangiitis. These variants differ in clinical manifestations, treatment approaches and prognosis. The article describes a clinical case of GPA manifested from lung damage with acute onset of the disease and rapid progression with minimal signs of the kidney and upper respiratory tract lesion and in the absence of symptoms typical for AAVs.