Diagnostics of Granulomatosis with Polyangiitis

Abstract

Actuality: Granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis, granulomatous vasculitis associated with anti-neutrophil cytoplasmic antibody) is a rare disease of unknown aetiology with multisystemic failure characterized by necrotizing granulomatous inflammation and the signs of vasculitis of autoimmune genesis involving predominantly small vessels There are no exact data on the prevalence of the disease. GPA occurs at any age but mostly at 40-65. In Europe GPA occurs more often in men than in women with the ratio 1.5:1. Objective: the analysis of clinical course of the disease, its diagnostics and prоgnosis of GPA is presented in this review Materials and methods we present the major clinical features of its course, approaches to diagnostics, and prоgnosis of GPA based on the review of literature data. Results and discussion. The onset of the disease may be either subacute (the development of clinical symptoms lasts for several weeks) or primary-chronic. Upper respiratory tract involvement is found in 90% of patients with GPA. Often it is the first and the only manifestation of the disease for a long time along with general inflammatory symptoms (fever, loss of weight, increased ESR). These patients often present to the otolaryngologist with nose congestion, manifestations of “sinusitis”, ear pain and decrease of hearing. The diversity of GPA clinical variants are presented in the article. Stages of diagnostic search in suspected GPA including laboratory tests, visualization methods, biopsy and serologic methods of anti-neutrophil cytoplasmic antibody detection are presented. The article emphasizes important diagnostic criteria of GPA and discusses possible prognoses of the disease. Conclusions: Knowledge of diagnostic search directions is of great significance for doctors of various specialities as early GPA diagnostics and proper management of patients with this disease influences greatly its prognosis.