A CLINICAL CASE OF IGG4-ASSOCIATED RESPIRATORY DAMAGE AND THE CHOICE OF THE OPTIMAL TREATMENT STRATEGY

Рекалов Д.Г., Данюк І.О.

Summary. Relevance. IgG4-associated disease is a group of rare diseases, characterized by inflammatory infiltration of various organs by mainly IgG4-positive plasmocytes, with the development of tissue fibrosis and increasing of IgG4 in blood. Many cases of different organs damage and tissues were described (mainly gastrointestinal damage). Aim: to describe a rare clinical case of IgG4-associated respiratory system damage. Materials and methods. The article presents a clinical case of IgG4-associated res­piratory system damage in 57-old years woman, who asked to rheumatology department of Zaporizhzhya regional clinical hospital. Clinical picture was manifested by persistent cough with expectoration of white sputum, slight hemoptysis, shortness of breath, fever up to 38 °C. Anamnesis was characterized by persistent urticaria. Laboratory changes included increase of C-reactive protein (30,7 mg/l), IgE (1872 IU/ml) and IgG4 (2080 mg/l) in serum. Using tomography were detected lymphadenopathy of the paratracheal and bifurcation lymph nodes, nodular changes in the lung parenchyma and fibrosis of the bronchial tree. Bronchus biopsy detected the metaplasmic flat epithelium, signs of granulation inflammation, lymphocytic-plasmacytic infiltration, fibrosis in the submucosal layer. Tuberculosis, sarcoidosis and systemic vasculitis were excluded. Results. Using the criteria of K. Okazaki et al. (2006) was diagnosed the IgG4-AD. Diagnosis was based on typical morphological changes in the respiratory system (subpleural nodule in the ½ segment of the left lung, paratracheal and bifurcation lymphadenopathy), increase in serum of IgG4 >135 mg/dl and typical histological picture in the affected bronchus (lymphocytic-plasmacytic infiltration, fibrosis in the submucosal layer). The case was characterized by the absence of multiple organ damage and a moderate degree of disease activity, so treatment strategy included only orally administration of corticosteroids and cytostatic. Induction of remission was achieved by the administration of prednisolone at a dose of 20 mg per day orally. To ensure a long-term control of the disease, the combination of prednisolone of 7.5 mg per day orally and methotrexate of 15 mg per week orally was optimal.