The Diagnosis and Treatment of Antisynthetase Syndrome

Abstract

Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud’s phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results. Patients with anti-synthetase syndrome often require multi-modality immunosuppressive therapy in order to control the muscle and/or pulmonary manifestations of their disease. The long-term care of these patients mandates careful attention to the adverse effects and complications of chronic immunosuppressive therapy, as well as disease-related sequelae that can include progressive interstitial lung disease necessitating lung transplantation, pulmonary hypertension, malignancy and decreased survival. It is hoped that greater awareness of the clinical features of this syndrome will allow for earlier diagnosis and appropriate treatment to improve outcomes in patients with anti-synthetase syndrome.

A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria

“…At our institution, our rheumatology colleagues have a heightened awareness of occult CTD presentations in patients with ILD and together we often discern subtle physical findings, such as mild mechanics hands. This more sensitive assignment of a diagnosis of an idiopathic inflammatory myositis in our patients aligns with our practice of aggressively treating ILD in patients with a “myositis” phenotype because of the possibility that ILD in this setting may respond to immunosuppressive treatment and may progress rapidly without such therapy [3, 4]. …”

Autoimmune Myopathies: Updates on Evaluation and Treatment

“…Myositis associated with ASS is a category of myositis associated with anti-aminoacyl-tRNA synthetase (ARS) antibodies, most commonly anti-Jo1 antibodies. These patients typically manifest with ILD, constitutional symptoms including fevers and weight loss, nonerosive arthritis, Raynaud's phenomenon, and skin changes known as Bmechanic's hands [34][35][36][37]. The co-occurrence of an erythematous rash can result in many patients being misdiagnosed with DM.…”

Aminoacyl-tRNA synthetases, therapeutic targets for infectious diseases

“…Specific auto-antibodies are found in the sera of patients with autoimmune disease. Antisynthetase syndrome is a chronic autoimmune condition caused by the production of auto-antibodies against eight different ARSs, of which anti-HRS is the most prevalent [117,118]. Antisynthetase syndrome is a form of idiopathic inflammatory myopathy characterized by myositis, interstitial lung disease, and arthritis [119].…”