A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Abstract

Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

Keywords: IIM; anti-Jo-1 antibody; antisynthetase antibodies; antisynthetase syndrome; idiopathic inflammatory myopathy; interstitial lung disease.

Figure 1

Chest CT scan of lower lung fields.

Figure 2

Chest CT scans with different ASAbs. (A): Bilateral, scattered, peribronchovascular ground-glass opacities; (B): bilateral, right side predominant, dense consolidative opacities; (C): diffuse, lower lobe predominant round-glass opacities with traction bronchiectasis; and (D): lower lobe predominant, scattered ground-glass opacities.

Figure 3

Mechanic’s hands (courtesy of C. Oddis).