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Journal: NAUKA MOLODYKH (Eruditio Juvenium) (Vol.6, No. 2)

Publication Date:

Authors : ;

Page : 285-297

Keywords : vasculitis; polyangiitis; Wegener’s granulomatosis; antineutrophil cytoplasmic antibodies; rapidly progressing glomerulonephritis;

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In the article modern data about clinical manifestations, diagnosis, differential diagnosis, treatment and prognosis of granulomatosis with polyangiitis are given. Granulomatosis with polyangiitis is a rather rare disease with poor prognosis in a rapidly progressing course. Etiology and pathogenesis of the disease are not completely understood. Due to the presence of antineutrophil cytoplasmic antibodies (ANCAs) the disease is referred to the group of systemic ANCAs-associated necrotizing vasculitis. The clinical picture is characterized by an extremely high polymorphism with probable involvement of practically any organs and systems into the pathological process. Most commonly involved are ENT-organs, lungs, kidneys. In modern diagnosis an important role is assigned to antineutrophil cytoplasmic antibodies. Morphological confirmation of the diagnosis relies on biopsy of the affected organ. Differential diagnosis consists in ruling out diseases that also run with pulmonary-renal syndrome. The aim of therapy is to suppress immunopathological processes that form the basis of the disease. A clinical case of identification of granulomatosis with polyangiitis in a man of 61 years old is given. The case illustrates manifestations of granulomatosis with polyangiitis in the form of a severe damage to the upper airways and kidneys with difficulties in diagnosis due to rare incidence of the disease and polyorganic character of pathology. Thus, timely diagnosis of granulomatosis with polyangiitis presents a difficult clinical task for doctors of different specialties due to rare incidence of the disease and varieity of its clinical manifestations. Early determination of ANCAs increases the rate of correct diagnosis, biopsy of the affected organs enables morphological verification of diagnosis. Only timely diagnosis permits to start treatment as early as possible and to avoid disability of patients.

Last modified: 2019-01-09 17:17:38