<p>The antisynthetase syndrome (ASS) is an extremely rare autoimmune disease characterized by the presence of aminoacyl-tRNA synthetase antibodies in combination with a&#xa0;symptom triad of myositis, interstitial lung disease (ILD) and/or arthritis. In addition to myositis, ILD is a&#xa0;frequent and often dominant organ involvement that essentially contributes to the morbidity and mortality. Other pulmonary manifestations include pulmonary hypertension and respiratory muscle involvement. The Jo‑1 antibody is by far the most frequently detected antibody in ASS. The treatment should be individualized depending on the organs affected and the severity of the disease.</p>

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Antisynthetasesyndrom mit Lungenbeteiligung: eine nicht zu unterschätzende Organmanifestation

  • Andreea Florea,
  • Hanns-Martin Lorenz,
  • Panagiota Xanthouli

摘要

The antisynthetase syndrome (ASS) is an extremely rare autoimmune disease characterized by the presence of aminoacyl-tRNA synthetase antibodies in combination with a symptom triad of myositis, interstitial lung disease (ILD) and/or arthritis. In addition to myositis, ILD is a frequent and often dominant organ involvement that essentially contributes to the morbidity and mortality. Other pulmonary manifestations include pulmonary hypertension and respiratory muscle involvement. The Jo‑1 antibody is by far the most frequently detected antibody in ASS. The treatment should be individualized depending on the organs affected and the severity of the disease.