Das Anti-Synthetase-Syndrom: Eine Sonderform der Myositis mit vielen Gesichtern

Antisynthetase syndrome (ASyS) encompasses a clinically heterogeneous and rare autoimmune disease characterized by the production of autoantibodies against aminoacyl-tRNA synthetases. Clinically, the disease presents with the triad of myositis, arthritis, and interstitial lung disease. We report the case of a 30-year-old man with recurrent episodes of fever, symmetric joint swelling, muscle symptoms, and progressive exertional dyspnea, in whom the diagnosis of ASyS was made based on the detection of anti-Jo‑1 antibodies. The combination therapy of glucocorticoids and azathioprine resulted in remission of the myositis and arthritis as well as regression of the ILD-associated changes in the chest CT. Early recognition of the syndrome and the assessment of myositis-specific antibodies are nowadays essential for an accurate nosological classification and definition of therapy strategies tailored to the severity of organ involvement.