Baydur A, Mongan E S, Slager U T
Chest. 1979 Apr;75(4):518-20. doi: 10.1378/chest.75.4.518.
A 28-year-old woman with a eight-year history of rheumatoid arthritis presented with a three-day history of dyspnea. Physical and electrocardiographic findings were consistent with pulmonary hypertension. Arterial blood gases revealed a ventilation-perfusion mismatch. Chest roentgenogram was normal. After transient improvement, she suddenly deteriorated and died. At autopsy, a necrotizing pulmonary panarteritis was found without parenchymal involvement by rheumatoid disease. The pulmonary arteries were the only vessels affected. Immunofluorescent staining revealed immunoproteins scattered throughout the vessel walls without localization to the basement membrane. The unique features of the case are discussed in relation to pulmonary hypertension and rheumatoid lung disease in which vascular lesions are usually associated with honeycomb lung. The association between the rheumatoid arthritis and pulmonary vasculitis was probably coincidental.
一名患有八年类风湿性关节炎病史的28岁女性，出现了三天的呼吸困难病史。体格检查和心电图检查结果与肺动脉高压相符。动脉血气分析显示通气-灌注不匹配。胸部X线片正常。经过短暂改善后，她突然病情恶化并死亡。尸检发现有坏死性肺全动脉炎，类风湿疾病未累及实质。肺动脉是唯一受累的血管。免疫荧光染色显示免疫蛋白散在分布于整个血管壁，未定位到基底膜。结合肺动脉高压和类风湿性肺病（其中血管病变通常与蜂窝肺相关）对该病例的独特特征进行了讨论。类风湿性关节炎与肺血管炎之间的关联可能是巧合。
