Myositis-Associated Interstitial Lung Disease: Focusing on Anti-synthetase Syndrome and MDA5-Positive Dermatomyositis
DOI:
https://doi.org/10.53350/pjmhs02026207.3Keywords:
Myositis-associated ILD, Anti-synthetase syndrome, MDA5-positive dermatomyositis, Pulmonary function, HRCT, Mortality.Abstract
Background: Myositis-related interstitial lung disease (ILD) is a devastating presentation of idiopathic inflammatory myopathies, and not all patients respond in a similar way to this condition. Anti-synthetase syndrome and MDA5-positive dermatomyositis are identified subtypes that have an independent pulmonary involvement. The purpose of the study was to compare clinical, functional, radiologic and outcome characteristics of these two subgroups.
Methods: A retrospective cohort study was carried out in a tertiary care center(POF Hospital Wah Cantt) in the time frame of January 2010 to December 2022. Adult patients with positive anti-synthetase syndrome or MDA5-positive dermatomyositis were taken into account. Medical records were reviewed to extract demographic information, clinical characteristics, pulmonary function tests (FVC and DLCO) and high-resolution computer tomography (HRCT) severity scores, and mortality outcomes. The SPSS version 26 was used to analyse the data and p < 0.05 was taken to be statistically significant.
Findings: 120 patients were studied (50 anti-synthetase syndrome, 70 MDA5-positive dermatomyositis). There was no difference in mean age (50.96 vs. 51.47 years). Both groups had restrictive lung disease and had a poor diffusion capacity (mean FVC: 71.91% vs. 66.53%; mean DLCO: 53.92% vs. 56.05%). The severity of HRCT was similar, but with slightly higher proportions of patients with severe involvement in MDA5-positive dermatomyositis. The MDA5-positive dermatomyositis group had a higher mortality (29% vs. 16%). The overall gender balance was equal with slight female dominance in anti-synthetase syndrome and male dominance in MDA5-positive dermatomyositis.
Conclusions: MDA5-positive dermatomyositis and both anti-synthetase syndrome are characterized by high restrictive lung disease and diffusion impairment. Nonetheless, MDA5-positive dermatomyositis has a greater severity and mortality of HRCT, implying a more aggressive ILD phenotype. These results highlight the issue of early detection and surveillance of pulmonary involvement in myositis patients especially when they are MDA5 positive.
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Copyright (c) 2026 Ibadullah Tahir, Hunain Shahbaz, Maria Islam, Abdul Rehman Shahid, Zubair Shahid, Malik Muhammad Kabir
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