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proLékaře.cz / Journals / Czech Rheumatology / 2022 - 3

Interstitial lung disease in systemic connective tissue diseases – risk factors, diagnostic and treatment

Czech version

Authors: P. Horák 1;  M. Žurková 2;  A. Skoumalová 1;  M. Žurek 1;  M. Skácelová 1
Authors‘ workplace: III. interní klinika – nefrologická, revmatologická a endokrinologická LF UP a FN, Olomouc 1;  Klinika plicních nemocí a tuberkulózy LF UP a FN, Olomouc 2
Published in: Čes. Revmatol., 30, 2022, No. 3, p. 121-133.
Category: Review Article

Overview

The tissues of the airways and lungs are among the frequently affected structures in systemic connective tissue diseases. Interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and fibrosis of the lung parenchyma. They are classified based on histopathological, radiological, and clinical parameters. The distinction between idiopathic pulmonary fibrosis and fibrosis accompanying systemic connective tissue diseases is important from the point of view of prognosis, as secondary forms have a more favorable course, although their impact is also serious. Interstitial lung involvement occurs most often in systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and rheumatoid arthritis, less often in systemic lupus erythematosus and Sjögren's syndrome. It also occurs frequently in microscopic polyangiitis. The prevalence, histopathological type, and mortality of these conditions vary significantly between rheumatic diseases. The cooperation of a rheumatologist, pulmonologist, and radiologist as well as screening strategies play a vital role in early diagnosis. Screening strategies are important for early diagnosis. Functional lung examination and imaging methods, especially high-resolution computed tomography, are irreplaceable examination methods. A number of anti-inflammatory drugs are available for therapy. For inflammatory forms of the disorders, it is possible to use glucocorticoids, cyclophosphamide, mycophenolate mofetil, azathioprine, calcineurin inhibitors, B-depletion therapy, or tocilizumab. The effect of these drugs is insufficient in predominantly fibrotic forms, where the use of antifibrotic drugs is an emerging therapy. Nintedanib is a drug newly registered for the indication of progressive interstitial lung involvement in systemic connective tissue diseases with broad efficacy evidence based on randomized, double-blinded, placebo-controlled trials.

Keywords:

interstitial lung disease – Connective tissue – immunosuppression – antifibrotic therapy

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Dermatology & STDs Paediatric rheumatology Rheumatology
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Prof. MUDr. Eduard Cmunt – průkopník české revmatologie
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