Shishido M, Ohtsuki Y, Ichiki H, Nishitani K
Department of Internal Medicine, Niihama Prefectural Hospital, Ehime, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1994 Oct;32(10):1026-31.
A 73-year-old woman admitted to the hospital with dyspnea on exertion. Chest radiography revealed a diffuse interstitial shadow; PaO2 was 72 Torr, and PaCO2 was 41 Torr. Laboratory examination results were compatible with idiopathic pulmonary fibrosis (IPF). Prednisolone relieved the dyspnea, but tapering of the drug led to a recurrence of this symptom. Pulse therapy was started and azathioprine was added to the corticosteroid. Over the course of 6 months of treatment, the patient's respiratory function remained fairly stable. Then respiratory distress was induced by an attack of atrial fibrillation, with relief provided by anti-arrhythmic drugs and large doses of corticosteroids. The patient died suddenly 3 weeks later. An autopsy revealed large thrombi in both pulmonary arteries with 90% stenosis. Parts of the thrombi were organized, which suggests that 2-3 weeks had elapsed since initial thrombus formation. Histological examination of lung tissue showed usual interstitial pneumonia. Pulmonary thromboembolism should be considered in patients with IPF if respiratory distress suddenly and unexpectedly worsens.
一名73岁女性因劳力性呼吸困难入院。胸部X线检查显示弥漫性间质阴影；动脉血氧分压（PaO2）为72托，动脉血二氧化碳分压（PaCO2）为41托。实验室检查结果符合特发性肺纤维化（IPF）。泼尼松龙缓解了呼吸困难，但药物减量导致该症状复发。开始脉冲治疗并在皮质类固醇基础上加用硫唑嘌呤。在6个月的治疗过程中，患者的呼吸功能保持相当稳定。随后房颤发作诱发呼吸窘迫，抗心律失常药物和大剂量皮质类固醇使其缓解。3周后患者突然死亡。尸检显示双侧肺动脉有大血栓，狭窄90%。部分血栓已机化，提示自最初血栓形成已过去2至3周。肺组织的组织学检查显示为普通型间质性肺炎。如果IPF患者的呼吸窘迫突然且意外地恶化，应考虑肺血栓栓塞。
