Management of interstitial lung disease (ILD) in myositis syndromes: A practical guide for

Inflammatory myopathies are heterogeneous clinico-serological syndromes, with variable clinical manifestations. Interstitial lung disease (ILD) is a major cause of morbidity and mortality in pa-tients with myositis. The clinical manifestation of myositis-ILD is heterogeneous, e.g., with acute-on-chronic presentations, as well as the chronic aftermath of acute disease. Here, we have largely divided myositis-ILD into three main prognostic groups which require different treatment approaches: mild-moderate (sub-acute), severe or progressive (acute or subacute) and rapidly pro-gressive, life-threatening. In current clinical practice, the treatment of myositis-ILD involves immunomodulation in an induction -maintenance treatment paradigm. There is now an option to add antifibrotics to slow the progression of established fibrosis in selected cases with chronic progressive phenotype. Here, we describe current concepts in myositis-ILD and aim to provide a practical guide for clinicians on how to approach assessment, including early identification of ILD, phenotyping of patients according to clinical trajectory and likely prognosis and stratified management adopting multi-disciplinary cross-speciality exper-tise, with close collaboration between rheumatology and respira-tory physicians.(c) 2022 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY license (http://creativecommons. org/licenses/by/4.0/).

Automated summary

Inflammatory myopathies are a diverse set of clinical syndromes with variable manifestations, and interstitial lung disease (ILD) is a major contributor to morbidity and mortality in myositis patients. The treatment approach for myositis-ILD involves immunomodulation and the option to add antifibrotics to slow down fibrosis progression. Early identification and multidisciplinary collaboration are crucial for the assessment and management of myositis-ILD.