Difficulties of differential diagnosis in practice of pulmonary medicine: granulomatosis with polyangiitis
Demko I.V. ^1,2, Gordeeva N.V.^1,2, Mamaeva M.G.², Matveeva I.V.², Kraposhina A.Yu.¹, Soloveva I.A.^1,2, Sobko E.A.^1,2, Zeleniy S.V.2, Loktionova M.M.<sup>1

1</sup> Krasnoyarsk State Medical University named after professor V.F. Voyno-Yasenetsky
² Regional Clinical Hospital, Krasnoyarsk

Granulomatosis with polyangiitis (Wegener's) (GPA) is systemic vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA), severe multiple organ damage. The prognosis is largely determined by speed of diagnosis and correct induction therapy. The hallmark of GPA is a combination of vasculitis necrotizing vasculitis (pathogenetically associated with ANCA) of mainly small and medium vessels of various localization and concomitant granulomatous inflammation, especially in respiratory organs. GPA is one of the most severe and prognostically unfavorable variants of systemic vasculitis. Recently the tendency to increase the number of patients with this pathology has been observed. GPA can have various masks at manifestation and during developed clinical picture, that complicates the diagnosis.
The paper presents a clinical case study, caused some difficulties in the diagnosis and verification of GPA diagnosis. Through careful analysis of clinical picture, symptoms, diagnostic finding with evaluation of specific GPA immunological markers, joint discussion with experts from different fields, the correct diagnosis was established timely, and adequate therapy was prescribed, that improved the prognosis of this severe disease.

Key words: granulomatosis, polyangiitis, systemic vasculitis, airway, alveolar infiltration.
For citation: Demko I.V., Gordeeva N.V., Mamaeva M.G. et al. Difficulties of differential diagnosis in practice of pulmonary medicine: granulomatosis with polyangiitis // RMJ. 2017. № 3. P. 211–213.