[Polymyositis and Jo-1 syndrome]

Polymyositis is a primarily clinical diagnosis. In 30-40% of patients, however, the specific anti-Jo-1 antibody (anti-histidyl-tRNA-synthetase) can be found. These cases are often associated with interstitial lung disease and other typical clinical manifestations. This symptomatology is called Jo-1-syndrome. Prognosis depends on early diagnosis, since Jo-1 associated interstitial lung disease resulting in pulmonary fibrosis is favorably influenced by immunosuppressive therapy, especially with corticosteroids. The present report concerns a patient with Jo-1-syndrome and beginning pulmonary fibrosis who was treated with methotrexate and prednisolone with good initial response. After 1 year the patient's condition had deteriorated, so that the therapy was changed to cyclophosphamide. Longterm results under this therapy remain to be evaluated.