Tomsic M, Sifrer F
Department of Rheumatology, University Medical Centre Ljubljana, Slovenia.
Wien Klin Wochenschr. 2000 Aug 25;112(15-16):728-31.
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.
我们报告一例66岁患有抗Jo - 1抗体的白种裔女性多肌炎患者。在入院后不久，尽管患者接受了静脉注射甲泼尼龙冲击治疗（连续3天，每日1000毫克），她仍发展为严重的、依赖通气的急性呼吸窘迫综合征。对急性呼吸窘迫综合征的感染性或非感染性病因评估未发现异常。支气管肺泡灌洗显示中性粒细胞性肺泡炎。经支气管活检的组织学检查显示为间质性纤维化过程和肺泡上皮立方化。除了大剂量甲泼尼龙外，患者每两周接受一次静脉注射环磷酰胺冲击治疗，持续六周，之后每4周一次。所有症状均得到缓解，动脉血气恢复正常。使用硫唑嘌呤维持缓解状态。发病一年后，多肌炎完全缓解。这是首例患有抗Jo - 1抗体的多肌炎患者在发生此类并发症后存活的报告。静脉注射环磷酰胺可能是挽救生命的治疗方法。
