Myositis Specific Antibodies and Interstitial Lung Disease: A Single Center Retrospective Study
Recommended Citation
Major J, Konja J, Thavarajah K, Calo SM, Abu Sayf A, Martirosov AL, Abdul Hameed AM. Myositis Specific Antibodies and Interstitial Lung Disease: A Single Center Retrospective Study. Am J Respir Crit Care Med 2025; 211:A3535.
Document Type
Conference Proceeding
Publication Date
5-20-2025
Publication Title
Am J Respir Crit Care Med
Abstract
Rationale: Myositis-specific antibodies (MSA) are commonly present in patients with myositis. Interstitial lung disease (ILD) is a common extra-skeletal manifestation of myositis that often precedes muscular or skin involvement. Many individuals with MSA and ILD never progress to develop typical features of myositis. This study aims to describe the characteristics of patients with ILD and a positive MSA. Methods: A retrospective cohort study of patients evaluated by a provider of the Henry Ford Health (HFH) ILD program and an MSA antibody was performed. MSA included Jo-1, PL7, PL12, EJ, OJ, MDA5, TIFF, NXP, Mi2 and SRP autoantibodies. Demographic data including gender, age at diagnosis, and diagnostic makers such as serology results, pulmonary function tests (PFT), and High-resolution computed tomography (CT) findings at the time of initial evaluation were collected. Evaluations by sub-specialties including rheumatology, dermatology, and neurology were reviewed for a formal myositis diagnosis. Descriptive statistics were performed. CT findings and PFTs at the time of diagnosis were analyzed for statistical differences between patients with a confirmed diagnosis of myositis-associated ILD (M-ILD) and idiopathic ILD with a positive MSA (I-ILD w/MSA) Results: This study included 127 patients of whom 62% were female. 69.3% were evaluated by rheumatology, 42.4% by dermatology, 47.2% by neurology, and 40.9% were confirmed to have a diagnosed with myositis. MSA included 47 Jo-1, 8 PL7, 14 PL12, 1 EJ, 2 OJ, 15 MDA5, 11 TIFF, 18 NXP, 9 MI2, and 7 SRP. The mean percentage of predicted Forced Vital Capacity (FVC) was 68.4% with a standard deviation of 19.6. Mean Diffusion Capacity for Carbon Monoxide (DLCO) was 46.9% with a standard deviation of 19.3. Imaging demonstrated 70.9% with ground glass opacities, 15.7% consolidation, 42.5% reticulation, 55.1% bronchiectasis, and 16.5% honeycombing. Statistical significance was found for M-ILD to present with consolidation on imaging, whereas I-ILD w/MSA was more likely to present with honeycombing. Conclusions: This cohort represents a female predominance in those with ILD and positive MSA. Furthermore, those with I-ILD w/MSA more frequently presented with honeycombing on imaging suggesting advanced disease at the time of presentation compared to M-ILD. Ultimately, given only 40.9% of this population possessed a formal diagnosis of myositis, further investigation regarding the relationship of MSA and ILD is warranted. Future studies should elicit disease course and response to treatment between these two groups. Figure 1: Characteristics of our patient population. Standard deviation for PFT's within brackets. Significant P values are bolded.
Volume
211
First Page
A3535
