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 targeting autoantigens such as Jo-1, PL-7, PL-12, OJ, EJ, KS, Ha, and Zo 1.
Clinical Features
The condition typically presents with a combination of:
- Interstitial lung disease (ILD)
- Inflammatory myopathy
- Arthritis
- Fever
- And a skin rash known as mechanic's hands, with the anti-Jo-1 antibody being the most common and hallmark antibody for this syndrome, found in approximately 20% of adult patients with idiopathic inflammatory myopathy (IIM) 1.
Treatment Approach
Treatment for anti-synthetase syndrome usually involves a combination of immunosuppressive medications, with the goal of managing symptoms and preventing disease progression. The specific treatment regimen can vary based on the severity of the disease and the patient's response to therapy.
Key Considerations
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 to the medication regimen.
From the Research
Definition and Characteristics of Anti-Synthetase Syndrome
- Anti-synthetase syndrome (ASSD) is an autoimmune disease characterized by the presence of autoantibodies targeting one of several aminoacyl t-RNA synthetases (aaRSs) 2
- The syndrome is associated with clinical features including interstitial lung disease, myositis, Raynaud's phenomenon, arthritis, mechanic's hands, and fever 2, 3, 4, 5, 6
Autoantibodies and Clinical Manifestations
- Autoantibodies against aminoacyl t-RNA synthetases, such as 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, have been associated with ASSD 2
- The most commonly described autoantibodies associated with ASSD are anti-histidyl (anti-Jo1), anti-threonyl (anti-PL-7), anti-alanyl (anti-PL-12), anti-isoleucyl (anti-OJ), anti-glycyl (anti-EJ), anti-asparaginyl (anti-KS), anti-Wa, anti-tyrosil- (anti-YRS), and anti-phenylalanyl-transfer RNA synthetase (anti-Zo) 3, 4
Clinical Features and Prognosis
- Interstitial lung disease is a frequent and often predominant organ involvement in ASSD, responsible for morbidity and mortality 5
- The 5-year survival rate for patients with Jo-1 syndrome, a subtype of ASSD, is approximately 90% 5
- Treatment with selected immunosuppressive medication depends on the extent and severity of organ involvement 5, 6