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[伴有多发性大疱和肺结节性淀粉样变的干燥综合征]

[Sjögren's syndrome with multiple bullae and pulmonary nodular amyloidosis].

作者信息

Teruuchi S, Bando M, Hironaka M, Ohno S, Sugiyama Y

机构信息

Department of Pulmonary Medicine, Jichi Medical School, 3311-1 Yakushiji Minami, Kawachi-machi, Kawachi-gun, Tochigi, Japan.

出版信息

Nihon Kokyuki Gakkai Zasshi. 2000 Dec;38(12):918-22.

Abstract

We report a case of Sjögren's syndrome with pulmonary involvement diagnosed by open lung biopsy. The patient was a 62-year-old woman with antiphospholipid antibody syndrome. Her chest radiograph and CT scan showed multiple bullae diffusely scattered throughout the lung. The open lung biopsy specimens revealed marked inflammatory mononuclear cell infiltration and nodular amyloid deposits in the bronchiolar walls. The mechanism of bulla formation appeared to be the check valve mechanism caused by the narrowing of the airway by the bronchiolitis. The patient was treated with oral corticosteroids, and her symptoms and laboratory findings became stable.

摘要

我们报告一例通过开胸肺活检诊断为合并肺部受累的干燥综合征病例。患者为一名62岁患有抗磷脂抗体综合征的女性。她的胸部X线片和CT扫描显示双肺弥漫性散在多个肺大疱。开胸肺活检标本显示细支气管壁有明显的炎性单核细胞浸润和结节状淀粉样沉积物。肺大疱形成的机制似乎是细支气管炎导致气道狭窄引起的单向活瓣机制。患者接受了口服糖皮质激素治疗,其症状和实验室检查结果趋于稳定。

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