Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis
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Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis Tamara Vojinovic1 · Ilaria Cavazzana1 · Paolo Ceruti2 · Micaela Fredi1,3 · Denise Modina2 · Marialma Berlendis2 · Franco Franceschini1,3 Accepted: 21 October 2020 © The Author(s) 2020
Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511 Keywords Idiopathic inflammatory myositis (IIM) · Interstitial lung disease (ILD) · Anti-Ro52 antibodies · Anti-ku antibodies · Anti-synthetase syndrome
Introduction Tamara Vojinovic and Ilaria Cavazzana equally contributed to this work Electronic supplementary material The online version of this article (https://doi.org/10.1007/s12016-020-08814-5) contains supplementary material, which is available to authorized users. * Franco Franceschini [email protected] 1
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Piazzale Spedali Civili 1, 25123 Brescia, Italy
2
Pulmonology Unit, ASST Spedali Civili, Piazzale Spedali Civili 1, 25123 Brescia, Italy
3
Clinical and Experimental Science Department, University of Brescia, Piazza del Mercato 15, 25121 Brescia, Italy
Interstitial lung disease (ILD) represents one of the most frequent complaints in idiopathic inflammatory myositis (IIM) and could deeply mark the disease course, in terms of functional
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