Interstitial Lung Disease in Dermatomyositis Without Myositis-Specific and Myositis-Associated Autoantibodies: Study of a Series of 72 Patients From a Single Cohort

• Published in: Frontiers in immunology 2022-05, Vol.13, p.879266-879266
• Main Authors: Chen, Fang, Wang, Jinping, Zhang, Puli, Zuo, Yu, Ye, Lifang, Wang, Guochun, Shu, Xiaoming
• Format: Article
• Language: English
• Subjects: dermatomyositis; Immunology; interstitial lung disease; myositis associated autoantibody; myositis specific autoantibody; negative myositis autoantibody; RPILD
• ISSN: 1664-3224; 1664-3224
• DOI: 10.3389/fimmu.2022.879266

The clinical features of interstitial lung disease (ILD) in patients with dermatomyositis (DM) and negative myositis autoantibodies had not been exactly demonstrated previously. This study aimed to describe and expand the phenotype of interstitial lung disease (ILD) in this cohort of patients. A total of 1125 consecutive Chinese patients with idiopathic inflammatory myopathies (IIM) between 2006 and 2020 were screened retrospectively. All proven cases of isolated ILD with both negative myositis-specific autoantibodies (MSA) and negative myositis-associated autoantibodies (MAA) were selected for inclusion. The clinical features and outcome among this group, MDA5 DM (DM patients with positive anti-MDA5 antibody) and ASS (patients with positive anti-aminoacyl tRNA synthetases antibodies were recorded and compared. Of 1125 IIM patients with an average follow-up of 6 years, 154 DM patients with negative MSA and MAA (MSA/MAA) were identified, with an ILD incidence of 46.8%. DM-ILD Patients with negative MSA/MAA presented younger age at onset (p