Wegener's granulomatosis: difficulties in diagnosis and treatment

Abstract

Granulomatosis with polyangiitis (Wegener's granulomatosis, GPA) is a rare disease of unknown etiology with multiple organ lesions, characterized by the development of necrotizing granulomatous inflammation and signs of autoimmune vasculitis, predominantly affecting small and medium-sized vessels. In the above clinical case, the patient has a classic triad of symptoms (damage to the upper respiratory tract, lungs, kidneys), the diagnosis was confirmed morphologically. The peculiarity of the described clinical case is its rare prevalence, the difficulty in making a diagnosis due to the multiorganism of the lesion, the absence of specific signs at the onset of the disease, which required a differential diagnosis. Early detection of antineutrophil cytoplasmic antibodies (ANCA) in the blood increases the frequency of GPA diagnosis, and timely diagnosis allows prescribing therapy as soon as possible and avoiding disability in patients.