Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis

Tamara Vojinovic,Franco Franceschini,Ilaria Cavazzana,Denise Modina,Marialma Berlendis,Micaela Fredi,Paolo Ceruti

doi:10.1007/s12016-020-08814-5

Tamara Vojinovic, Franco Franceschini + Show 5 more

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https://doi.org/10.1007/s12016-020-08814-5

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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: non-specific 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

Highlights

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 limitation, treatment strategy, and mortality [1]
Most of IIM were represented by polymyositis (PM) (52 cases), DM (55 cases), or overlap IIM (24 cases), according to the Bohan and Peter criteria [14]; ARS [15] were found in 31 patients, while inclusion body myositis (IBM) [16] and necrotizing autoimmune myositis (NAM) [17] were diagnosed in 2 and 1 patients, respectively
We report here a cohort of largely prevalent Caucasian patients affected from IIM from a single center

Summary

Introduction

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 limitation, treatment strategy, and mortality [1]. ILD is one of the clinical hallmarks of anti-synthetase syndrome (ARS) [3], characterized by the triad of myositis, arthritis, and ILD, in which ILD occurs in about 60–80% of cases [4]. A severe ILD, named rapidly progressive ILD (RPILD), was described, especially in Asian cohorts, in hypo- or amyopathic dermatomyositis (DM), associated with anti-MDA-5 antibodies, characterized by a rapid decline of functional status and a high mortality rate [6,7,8,9,10,11]

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