Patients With Inflammatory Myopathies Require Monitoring for ILD Progression

Researchers were unable to identify independent predictors of the progressive fibrosing interstitial lung disease (PF-ILD) phenotype.

Almost 20% of patients with interstitial lung disease (ILD) developing from idiopathic inflammatory myopathies (IIMs) developed a progressive fibrosing ILD (PF-ILD) phenotype, according to study findings published in Rheumatic & Musculoskeletal Diseases Open.

Patients with IIMs may develop ILD, which may in turn lead to PF-ILD; however, there is currently no established monitoring strategy for ILD in patients with IIMs. Investigators therefore sought to assess the prevalence of PF-ILD in patients with IIMs as well as factors that could potentially predict this phenotype. The researchers also evaluated the radiological patterns and clinical/serological features associated with IIMs-ILD.

Investigators conducted a retrospective cohort study of 253 consecutive patients with IIMs seen between 2002 and 2020 at 3 centers in Italy and 1 center in France. IIMs was defined according to Bohan and Peter’s criteria, the European Neuromuscolar Center (ENMC) criteria, or 2017 EULAR/ACR classification criteria. PF-ILD was defined according to the 2022 American Thoracic Society (ATS), Japanese Respiratory Society (JRS), European Respiratory Society (ERS), and Latin American Thoracic Society (ALAT) guidelines.

Enrolled patients (median [interquartile range] age, 55 [46-66] years; 72% female) had pulmonary function tests at least once during a visit to a referral center. Predominant high-resolution computed tomography (HRCT) patterns were used to classify patients with ILD into nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), and organizing pneumonia (OP). Patients with juvenile IIMs, incomplete and/or unavailable clinical and/or serological baseline data were excluded.

All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD.

Among patients with IIMs (median follow-up [range] duration, 6 [3-10] years) almost half (125) had ILD (n=99 at IIMs diagnosis; n=26 during follow-up, most within 5 years). Factors independently associated with ILD and identified in multivariate analysis included anti-Ro52, anti-MDA5, antiJo-1, high score on manual muscle test, mechanic’s hands, and Raynaud’s phenomenon.

NSIP was noted as the predominant HRCT pattern (50% of patients), along with UIP (28%), and OP (22%). Almost one-fifth of patients with IIMs-ILD developed PF-ILD by the 1-year follow-up, but investigators were unable to identify independent predictors. Lung involvement was always detected before IIM diagnosis in the OP group vs NSIP group (P =.01) and in the OP group vs UIP group (P =.04).

AntiMDA5, heliotropic rash, xerostomia, and xerophthalmia predicted PF-ILD in univariate analysis.

Study limitations include unaccounted-for differences in patient management and follow-up, the retrospective design, and underpowered sample size of patients with PF-ILD.

“Patients with IIM should be carefully screened for ILD at IIMs diagnosis and yearly during follow-up,” investigators concluded. They wrote, “All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD.”

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.

This article originally appeared on Pulmonology Advisor


Zanatta E, Cocconcelli E, Castelli G, et al. Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: Data from a large multicentric cohort. RMD Open. August 2023;9(3):e003121. doi:10.1136/rmdopen-2023-003121