Interstitial lung disease associated with inflammatory myositis: Autoantibodies, clinical phenotypes, and progressive fibrosis

Abstract

Progressive pulmonary fibrosis is generally diagnosed when interstitial lung disease progression occurs in the absence of any other cause, and a subset of patients with myositis and associated interstitial lung disease may develop progressive pulmonary fibrosis. Numerous autoantibodies (e.g., against tRNA-synthetase, MDA5, Ro52) increase the risk of this clinical feature in myositis and we speculate that serum biomarkers, sought using the most sensitive laboratory techniques available (i.e., immunoprecipitation) may predict pulmonary involvement and allow the early identification of progressive pulmonary fibrosis. We herein provide a narrative review of the literature and also present original data on pulmonary fibrosis in a cohort of patients with myositis and serum anti-Ro52 with interstitial lung disease. Our results fit into the previous evidence and support the association between anti-Ro52 and signs of pulmonary fibrosis in patients with inflammatory myositis. We believe that the combination of available and real-life data has significant clinical relevance as a paradigm of serum autoantibodies that prove useful in determining precision medicine in rare connective tissue diseases.

Progressive pulmonary fibrosis in myositis-specific antibody-positive interstitial pneumonia: a retrospective cohort study

“…Myositis-specific antibodies (MSAs) have gained increasing attention for their role in distinguishing various types of IIMs and their association with the risk of developing ILD ( 3 , 4 ). Different MSAs, including anti-aminoacyl tRNA synthetase (anti-ARS) antibodies, anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and other rare antibodies, have been identified.…”

“…Different MSAs, including anti-aminoacyl tRNA synthetase (anti-ARS) antibodies, anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and other rare antibodies, have been identified. MSAs are relevant to the pathogenesis and prognosis of patients with IIM, with specific antibodies linked to clinical subtypes and increased risk of ILD ( 3 , 4 ). In cases where patients with interstitial pneumonia exhibit features suggestive of underlying autoimmune conditions but do not meet diagnostic criteria for any specific CTD, a diagnosis of interstitial pneumonia with autoimmune features may be considered ( 5 ).…”