Modern concepts of the prevalence, pathomorphology and diagnosis of granulomatosis with polyangiitis with pulmonary involvement (literature review)

Abstract

Granulomatosis with polyangiitis (GPA) (Wegener's granulomatosis) is a rare systemic autoimmune disease from the group of vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). GPA is proceeding with granulomatous inflammation, mainly of the upper and lower respiratory tract and low-immune necrotizing vasculitis that affects small- and medium-size vessels in lungs, kidneys, skin. The disease is an extremely rare long-term systemic disorder, is rarely the object of research, the literature data are few and unsystematic.Purpose. The purpose of the literature review was to study the published materials, analyze the received information and define tasks for further study of this pathology, primarily from the standpoint of radiation diagnostics.Materials and methods. The search was carried out in bibliographic databases MedLine, PubMed, if necessary, links to external full-text sources were used. 36 of the most significant publications were selected and worked out, including 8 domestic and 28 foreign. One of the selection criteria for articles was the availability of information about the radiological diagnosis of GPA in them.Results. Most authors focus their attention on clinical manifestations and features of treatment, not paying sufficient attention to diagnostic methods. Many studies have been carried out on a small sample of patients or are limited to individual clinical observations; there is practically no information on changes in the computed tomographic picture of the lungs in GPA in dynamics. The most complete semiotics of lung damage according to CT data is presented in the article by F. Martinez et al. (2012).Conclusions. It was found that in case of lung lesions by GPA, nodules, nodes and masses are more often determined, in half of cases – with cavities of decay, less often areas of ground-glass opacity and consolidation are detected. However, at present there is no unified approach to the interpretation of the radiological semiotics of the disease and the determination of the diagnostic value of individual computed tomographic patterns of lung damage. This requires their further detailed study for the correct and timely diagnosis of such a rare, but complex and potentially life-threatening disease.