[原发性节段性硬化性胆管炎合并干燥综合征]

[Primary segmental sclerosing cholangitis associated with Sjögren's syndrome].

作者信息

Guerrero P, Martín M, Conde J M, Castro M, Rodríguez M C, Castilla L, Guillén P

机构信息

Sección de Anatomía Patológica, Hospital Universitario de Valme, Sevilla.

出版信息

Rev Esp Enferm Dig. 1991 May;79(5):363-6.

Abstract

We report the case of a patient with primary sclerosing cholangitis associated with Sjögren's syndrome and Raynaud's phenomenon, an uncommon association. The presence of periportal lymph nodes with features similar to those described in autoimmune diseases suggests an immune pathogenesis of the disease. The localized and segmentary involvement of the common duct is also an uncommon finding. The pathogenetic and therapeutic aspects are discussed.

摘要

我们报告了一例原发性硬化性胆管炎合并干燥综合征和雷诺现象的患者，这种关联并不常见。肝门周围淋巴结的存在，其特征与自身免疫性疾病中所描述的相似，提示了该疾病的免疫发病机制。胆总管的局限性和节段性受累也是一个不常见的发现。本文对其发病机制和治疗方面进行了讨论。

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