Objectives Data on interstitial lung disease (ILD) in Indian patients with idiopathic inflammatory myopathy (IIM) is limited. We aimed to determine the characteristics of ILD, and factors associated with the progression of ILD in Indian patients with IIM. Methods A prospective single centre cohort of 86 consecutive adult-onset IIM patients recruited between October 2020 and December 2025 were studied. The diagnosis of IIM related ILD (IIM-ILD) was made after high resolution CT Thorax. Progression of ILD was defined as per the 2022 ATS/ERS/JRS/ALAT guidelines for progressive pulmonary fibrosis (PPF). Multivariate logistic regression was done to identify independent factors associated IIM-ILD. Comparisons were made between patients with PPF and those without PPF. Results Of the 86 IIM patients, ILD was seen in 59.3% of patients (n = 51). The most common radiological patterns were non-specific interstitial pneumonia (58.8%), organizing pneumonia (21.6%), usual interstitial pneumonia (13.7%), and overlap of NSIP-OP (5.9%). The most common subsets were anti synthetase syndrome (66.6%, 34/51), dermatomyositis (23.5%, 12/51) and overlap myositis (7.8%, 4/51). The most common myositis specific autoantibody was anti-Jo1 (27.4%, 14/51) and the most common myositis associated autoantibody was anti-Ro52 (58.8%, 30/51). On multivariate logistic regression, heliotrope rash (OR 0.25, 95% CI 0.06–0.96, p = 0.04), arthritis (OR 3.57, 95% CI 1.15–11.07, p = 0.03) and anti-Ro52 (OR 4.37, 95% CI 1.40–13.40, p = 0.01) were associated with ILD. Of the 37 patients with IIM-ILD evaluated for progression, 18.9% (n = 7) developed PPF within 1 year of follow up. Amyopathic presentation and NSIP/OP pattern were associated with PPF (p = 0.02, p = 0.03 respectively). Conclusion The frequency of ILD is high in Indian patients with IIM (59.3%, 51/86) and of the IIM-ILD evaluated for progression, around one fifth developed PPF (18.9%, 7/37). A baseline screening for ILD is mandatory in IIM and close monitoring of IIM-ILD is required for early detection of PPF. <Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> Key Points •The frequency of ILD in Indian patients with IIM is high (59.3%, 51/86), higher than the global prevalence of ILD in IIM. •Arthritis, heliotrope rash and anti-Ro52 antibody were identified as significant factors associated with IIM-ILD. •Around 1/5th of evaluable IIM-ILD (18.9%, 7/37) developed progressive pulmonary fibrosis within 1 year of follow up. </entry> </row> </tbody> </tgroup> </Table>

High frequency of interstitial lung disease in Indian patients with adult- onset idiopathic inflammatory myopathy: Insights from a prospective observational cohort

期刊论文
发表日期：

2026年6月2日

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Harikrishnan Gangadharan,
Anusree Prasad Seetha,
Sajitha Musthafa,
Sandra Pulickal Babu,
Sandra Krishnan,
Sonia John,
Sajitha Krishnadas

摘要

Objectives

Data on interstitial lung disease (ILD) in Indian patients with idiopathic inflammatory myopathy (IIM) is limited. We aimed to determine the characteristics of ILD, and factors associated with the progression of ILD in Indian patients with IIM.

Methods

A prospective single centre cohort of 86 consecutive adult-onset IIM patients recruited between October 2020 and December 2025 were studied. The diagnosis of IIM related ILD (IIM-ILD) was made after high resolution CT Thorax. Progression of ILD was defined as per the 2022 ATS/ERS/JRS/ALAT guidelines for progressive pulmonary fibrosis (PPF). Multivariate logistic regression was done to identify independent factors associated IIM-ILD. Comparisons were made between patients with PPF and those without PPF.

Results

Of the 86 IIM patients, ILD was seen in 59.3% of patients (n = 51). The most common radiological patterns were non-specific interstitial pneumonia (58.8%), organizing pneumonia (21.6%), usual interstitial pneumonia (13.7%), and overlap of NSIP-OP (5.9%). The most common subsets were anti synthetase syndrome (66.6%, 34/51), dermatomyositis (23.5%, 12/51) and overlap myositis (7.8%, 4/51). The most common myositis specific autoantibody was anti-Jo1 (27.4%, 14/51) and the most common myositis associated autoantibody was anti-Ro52 (58.8%, 30/51). On multivariate logistic regression, heliotrope rash (OR 0.25, 95% CI 0.06–0.96, p = 0.04), arthritis (OR 3.57, 95% CI 1.15–11.07, p = 0.03) and anti-Ro52 (OR 4.37, 95% CI 1.40–13.40, p = 0.01) were associated with ILD. Of the 37 patients with IIM-ILD evaluated for progression, 18.9% (n = 7) developed PPF within 1 year of follow up. Amyopathic presentation and NSIP/OP pattern were associated with PPF (p = 0.02, p = 0.03 respectively).

Conclusion

The frequency of ILD is high in Indian patients with IIM (59.3%, 51/86) and of the IIM-ILD evaluated for progression, around one fifth developed PPF (18.9%, 7/37). A baseline screening for ILD is mandatory in IIM and close monitoring of IIM-ILD is required for early detection of PPF.

Key Points

•The frequency of ILD in Indian patients with IIM is high (59.3%, 51/86), higher than the global prevalence of ILD in IIM.

•Arthritis, heliotrope rash and anti-Ro52 antibody were identified as significant factors associated with IIM-ILD.

•Around 1/5th of evaluable IIM-ILD (18.9%, 7/37) developed progressive pulmonary fibrosis within 1 year of follow up.