Perm Medical JournalPerm Medical Journal0136-14492687-1408Eco-Vector1334010.17816/pmj36482-87Research ArticleClinical case of granulomatosis with polyangiitis (Wegener’s) with isolated pulmonary lesionObukhovaOksana V.obukhova.o@list.ruKhlynovaOlga V.<p><span style="color: #00000a;"><span style="font-family: Times New Roman, serif;"><span style="font-size: large;"><span lang="en-US"> </span></span></span></span></p>olgakhlynova@mail.ruE.A. Vagner Perm State Medical UniversityPerm Regional Clinical Hospital17102019364828710062019Copyright © 2019, Obukhova O.V., Khlynova O.V.2019<p>Pulmonary vasculites include geterogenic group of diseases, characterized by destruction of pulmonary vessels with inflammation and tissue necrosis. From the clinical point of view, pulmonary vasculites are still difficult to diagnose and treat.</p>
<p>Pulmonary lesion is one of typical features of granulomatosis with polyangiitis (GPA) along with upper respiratory tract and renal pathologies, composing classical triad of this disease.</p>
<p>In case of GPA, pulmonary lesion is diagnosed in 6085 % of patients. As a rule, it is combined with involvement of other organs, but in 9 % of patients, it can be the main manifestation of disease. The paper presents a case of GPA with isolated pulmonary lesion.</p>Pulmonary vasculitisgranulomatosis with polyangiitisANCA-associated vasculitisЛегочный васкулитгранулематоз с полиангиитомАНЦА-ассоциированный васкулит[Бекетова Т.В. Алгоритм диагностики системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами. Терапевтический архив 2018; 05: 13–21.][Бекетова Т.В. Гранулематоз с полиангиитом, патогенетически связанный с антинейтрофильными цитоплазматическими антителами: особенности клинического течения. Научно-практическая ревматология 2012; 50 (6): 19–28.][Flossmann O., Berden A., de Goot K., Hagen C., Harper L., Heijl C., Jayne D., Luqmani R., Mahr A., Mukhtyar C., Pusey C., Rasmussen N., Stegeman C., Walsh M., Westman K. European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70 (3): 488–494.][Flossmann O. Risks of treatments and long-term outcomes of systemic ANCA- associated vasculitis. Presse Med 2015; 44 (6Pt.2): e251–e257.][Frankel S.K., Cosgrove G.P., Brown K.K. Small vessel vasculitis of the lung. Chron Respir Dis 2005; 2 (2): 75–84.][Hoffman G.S., Kerr G.S., Leavitt R.Y., et al. Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med.1992; 116 (6): 488–498. DOI: http://dx.doi.org/10.7326/0003-4819-116-6-488][Jennette J.C., Falk R.J., Bacon P.A., Basu N., Cid M.C., Fertario F., Flores-Suarez L.F., Gross W.L., Guillevin L., Hagen E.C., Hoffman G.S., Sayne D.R., Kallenberg C.G.M, Lamprecht P., Langford C.A., Luqmani R.A., Mahr A.D., Matteson E.L., Merkel P.A., Ozen S., Pusey C.D., Rasmussen N., Rees A.S., Seott D.G.I., Specks U., Stone J.H., Takahashi K., Watts R.A. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65 (1): 1–11. DOI: 10.1002/art.37715]