Characteristics of patients with fibrosing sarcoidosis in four medical centers In Russia

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Introduction. Sarcoidosis is considered a disease that can lead to pulmonary fibrosis, but the incidence of progressive fibrosis and the need for antifibrotic therapy have not been fully determined. Аim. To evaluate the condition of patients with sarcoidosis at the time of development of pulmonary fibrosis and during the following two years. Mat erials and methods. Retrospective analysis of data of 65 patients with verified diagnosis of sarcoidosis from medical centers of Kazan, Moscow, St. Petersburg and Irkutsk. Data were recorded at four points: upon detection of sarcoidosis, upon detection of fibrosis and one and two years later. The analysis was performed using SPSS-18 software (IBM, USA). Differences were considered significant at p < 0.05. Results. Upon detection, stage I was diagnosed in 7 (10.8%), stage II – in 41 (63.1%), stage III – in 15 (23.1%) and stage IV – in 2 (3.0%). Löfgren’s syndrome was present in 2 (3.1%) patients. According to the international Delphi consensus, the most common fibrosis was type A – 25 (38.5%), type C and type D – 14 each (21.5%), and type B – 12 (18.5%). The decrease in FVC was significant upon detection of fibrosis from 80.0 ± 3.7% to 69.9 ± 3.3% (p = 0.0001), after a year 72.3 ± 3.7% (p = 0.015) and after 2 – 68.3 ± 3.5% (p = 0.0001), after the formation of fibrosis there was no decrease. HRCT signs did not change in 58.5%, increased in 30.2%, and decreased in 11.3%. DLco, like FVC, significantly decreased relative to the initial value, but did not decrease after the formation of fibrosis. Only in 50% of cases of sarcoidosis, the treatment tactics corresponded to federal clinical recommendations. Systemic GCS were received by 66.2% before the formation of fibrosis, and then by 43.1% and 34.1%. Among the drugs that affected fibrosis, nintedanib (13.6%), pirfenidone (4.6%), N-acetylcysteine (6.8%) and longidaza (3.1%) were used, none of them significantly affected all the indicators of progressive fibrosis. In a small sample, no advantages were found for their use in either stable or progressive fibrosis in patients with sarcoidosis.

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