From the Guidelines
Anti-Synthetase Syndrome Definition
Anti-synthetase syndrome is a rare autoimmune disorder characterized by the presence of autoantibodies against aminoacyl-tRNA synthetases, which are enzymes involved in protein synthesis, specifically presenting with fever, mechanic’s hands, Raynaud phenomenon, myositis, interstitial lung disease (ILD), and arthritis 1.
Clinical Features and Autoantibodies
The syndrome is associated with several autoantibodies, including Jo-1, PL-7, PL-12, OJ, EJ, KS, Ha, and Zo, with Jo-1 being the most common and the hallmark antibody for anti-synthetase syndrome, found in approximately 20% of adult patients with idiopathic inflammatory myopathy (IIM) 1. The frequencies of the other anti-tRNA synthetases range from 1% to 5% 1.
Diagnosis and Treatment
Diagnosis involves identifying the characteristic clinical features and the presence of specific autoantibodies. Treatment typically involves a combination of immunosuppressive medications to manage symptoms and prevent disease progression. The exact treatment regimen can vary based on the severity of the disease and the patient's response to therapy, often requiring long-term management to control symptoms and prevent disease progression.
Management and Monitoring
It is crucial to work closely with a healthcare provider to monitor the condition and adjust the treatment plan as needed to minimize side effects and optimize outcomes. Regular follow-up appointments and pulmonary function tests are essential to assess the effectiveness of the treatment and make any necessary adjustments 1.
From the Research
Definition and Characteristics of Anti-Synthetase Syndrome
- Anti-Synthetase Syndrome (AS) is a rare autoimmune disorder characterized by the presence of aminoacyl-transfer RNA synthetase antibodies in conjunction with clinical features such as interstitial lung disease (ILD), Raynaud's phenomenon, nonerosive arthritis, and myopathy 2.
- The syndrome is distinguished by its significant lung involvement and rapidly progressive interstitial lung disease (AS-ILD) 2.
- Anti-Synthetase Syndrome is also known as Antisynthetase Syndrome (ASS) or Anti-Synthetase Syndromes (ASS) 3, 4, 5, 6.
Clinical Features and Autoantibodies
- The clinical features of Anti-Synthetase Syndrome include interstitial lung disease, myositis, Raynaud's phenomenon, arthritis, mechanic's hands, and fever 3, 4, 5, 6.
- The syndrome is associated with the presence of autoantibodies targeting one of several aminoacyl t-RNA synthetases (aaRSs) 3.
- The most commonly described autoantibodies are histidyl (HisRS), threonyl (ThrRS), alanyl (AlaRS), glycyl (GlyRS), isoleucyl (IleRS), asparaginyl (AsnRS), phenylalanyl (PheRS), tyrosyl (TyrRS), lysyl (LysRS), glutaminyl (GlnRS), tryptophanyl (TrpRS), and seryl (SerRS) tRNA synthetases 3.
- Anti-Jo-1 is the most common autoantibody associated with Anti-Synthetase Syndrome, found in approximately 20-30% of patients with polymyositis (PM) or dermatomyositis (DM) 4.
Diagnosis, Treatment, and Prognosis
- The diagnosis of Anti-Synthetase Syndrome requires careful clinical, serologic, and radiologic assessment 2.
- Treatment with glucocorticoids is considered the mainstay of therapy, with additional immunosuppressive agents often required to achieve disease control 2.
- The prognosis of patients with Anti-Synthetase Syndrome is highly dependent on early diagnosis and symptom recognition, with a 5-year survival rate of approximately 90% for patients with the Jo-1 syndrome 6.