ANTI-JO-1-ANTIBODIES - AUTOANTIBODIES SPE CIFIC FOR POLYMYOSITIS PATIENTS WITH INTERSTITIAL ALVEOLITIS - REPORT OF 2 CASES

Anti-Jo-1 antibodies are rare autoantibodies, which bind to and inhibi t the activity of histidyl-tRNA-synthetase. They are predominantly fou nd in a genetically and clinically distinct subset of myositis patient s, presenting with interstitial alveolitis. We describe the case of a 22-year-old woman with jo-1-syndrome with typical features of myositis , pulmonary fibrosis, nonerosive symmetric polyarthritis, Gottron's pa pules at the metacarpophalangeal joints and classic heliotrope discolo ration of the periorbital area with edema. The patient did not respond to chloroquine, azathioprine, intravenous immunoglobulins or cyclopho sphamide. Remission was finally achieved with oral methotrexate plus c orticosteroids. The second case describes a 34-year-old man with myosi tis, who rapidly developed respiratory insufficiency after suffering f rom joint pain and stiffness for about 10 weeks. He responded well to cyclophosphamide pulse therapy and high-dose corticosteroids. The seco nd case demonstrates that control of the interstitial alveolitis is mo st important for long-term outcome. Therefore, immunosuppresive drugs should be used along with corticosteroids as early as possible.