Acute respiratory distress syndrome in a polymyositis patient with the anti-Jo-1 antibody

We report a case of a 66-year-old caucasian woman suffering from polymyositis with the anti-Jo-1 antibody. Shortly after admission to our hospital, despite the fact that the patient was given intravenous pulse methylprednisolone (1000 mg/day for 3 consecutive days), she developed severe, ventilatory-dependent, acute respiratory distress syndrome. Evaluation for infectious or noninfectious aetiologies of acute respiratory distress syndrome was unrevealing. Bronchoalveolar lavage disclosed neutrophilic alveolitis. Histological examination of a transbronchial biopsy revealed an interstitial fibrosing process and cuboidalisation of the alveolar epithelium. In addition to high-dose methylprednisolone the patient was given intravenous pulse cyclophosphamide fortnightly for six weeks and afterwards every 4 weeks. All symptoms resolved and arterial blood gases returned to normal. Remission has been maintained with azathioprine. One year after onset, polymyositis is in complete remission. This is the first report of a patient suffering from polymyositis with the anti-Jo-1 antibody who survived such a complication. Intravenous pulse cyclophosphamide was probably a life saving therapy.