Hasegawa Y, Shimokata K, Ichiyama S, Saito H
First Dept of Internal Medicine, Nagoya University School of Medicine, Japan.
Eur Respir J. 1999 Apr;13(4):934-7. doi: 10.1034/j.1399-3003.1999.13d39.x.
Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the disease often remains unknown. This study reports on the case of a 38 yr-old female with constrictive bronchiolitis obliterans and paraneoplastic pemphigus associated with malignant lymphoma. The patient developed progressive obstructive lung disease. The chest radiograph showed almost normal lungs. Paraneoplastic pemphigus is a newly described syndrome in which patients have autoantibodies binding to some epithelia, including in the respiratory tract. The disease develops in association with non-Hodgkin's lymphomas or other malignant neoplasms. The case presented here suggests that constrictive bronchiolitis obliterans associated with paraneoplastic pemphigus may be one of the facets of autoimmune responses in this context.
闭塞性细支气管炎伴机化性肺炎较为罕见，其发病机制通常仍不明确。本研究报告了一例38岁女性患者，患有闭塞性细支气管炎伴机化性肺炎及与恶性淋巴瘤相关的副肿瘤性天疱疮。该患者出现进行性阻塞性肺病。胸部X线片显示肺部基本正常。副肿瘤性天疱疮是一种新描述的综合征，患者体内存在与包括呼吸道上皮在内的某些上皮结合的自身抗体。该疾病与非霍奇金淋巴瘤或其他恶性肿瘤相关。此处报告的病例提示，与副肿瘤性天疱疮相关的闭塞性细支气管炎伴机化性肺炎可能是这种情况下自身免疫反应的一个方面。
