Abstract
BackgroundIn polymyositis/dermatomyositis (PM/DM), anti-aminoacyl-tRNA synthetase (ARS) antibodies are closely associated with interstitial lung disease (ILD), a frequent pulmonary complication. However, the clinical significance of anti-ARS antibodies is not well established.ObjectiveWe aimed to evaluate the clinical significance of anti-ARS antibodies in PM/DM-ILD patients.MethodsForty-eight consecutive PM/DM-ILD patients were studied retrospectively. Anti-ARS antibodies were screened by ELISA and confirmed by RNA immunoprecipitation test. Medical records, high-resolution computed tomography images, and surgical lung biopsy specimens were compared between ARS-positive (ARS group) and ARS-negative patients (non-ARS group).ResultsAnti-ARS antibodies were detected in 23 of 48 patients (48%). Radiologically, nonspecific interstitial pneumonia (NSIP) pattern was observed more frequently in the ARS group than in the non-ARS group (73.9% vs. 40%, P = 0.02). Pathologically, NSIP was the most frequent in both groups. Ten-year survival rate was also significantly higher in the ARS group than in the non-ARS group (91.6% vs. 58.7%, P = 0.02). Univariate Cox hazards analysis revealed that the presence of anti-ARS antibodies was associated with better prognosis (HR = 0.34, 95% CI 0.08–0.80; P = 0.01).ConclusionsThe presence of anti-ARS antibodies is a possible prognostic marker in patients with PM/DM-ILD.
Highlights
Idiopathic inflammatory myopathy (IIM) comprises a group of systemic autoimmune disorders, including polymyositis (PM) and dermatomyositis (DM), affecting skeletal muscles and other organs [1,2,3]
Univariate Cox hazards analysis revealed that the presence of anti-aminoacyl-tRNA synthetase (ARS) antibodies was associated with better prognosis (HR = 0.34, 95% CI 0.08–0.80; P = 0.01)
We previously described the clinical features of interstitial lung disease (ILD)-associated PM/DM (PM/DM-ILD) [7, 8] and identified the prognostic factors based on the clinical characteristics of a large series of PM/DM-ILD patients [9]
Summary
Idiopathic inflammatory myopathy (IIM) comprises a group of systemic autoimmune disorders, including polymyositis (PM) and dermatomyositis (DM), affecting skeletal muscles and other organs [1,2,3]. In patients with PM/DM, interstitial lung disease (ILD) is a common extramuscular involvement associated with poor prognosis [4,5,6]. Eight types of anti-ARS antibodies (Jo-1, PL-7, PL-12, EJ, OJ, KS, Zo, and Ha) have been identified [10, 16]. Patients with different types of anti-ARS antibodies show some unique clinical features and prognosis [17,18,19,20,21], these patient subgroups can present with similar clinical manifestations, such as ILD, myositis, arthritis, Raynaud’s phenomenon, and “mechanic’s hands” [ known as anti-synthetase syndrome (ASS)] [16, 17]. In polymyositis/dermatomyositis (PM/DM), anti-aminoacyl-tRNA synthetase (ARS) antibodies are closely associated with interstitial lung disease (ILD), a frequent pulmonary complication. The clinical significance of anti-ARS antibodies is not well established
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