Mitry E, Algayres J P, Bili H, Coutant G, Gros P, Nizou J Y, Castagne C, Vergeau B, Daly J P
Service de Clinique Médicale, Hôpital d'Instruction des Armées du Val-de-Grâce, Paris.
Gastroenterol Clin Biol. 1998 Jan;22(1):94-7.
Autoimmune cholangitis is a rare cause of chronic liver disease which has recently been described and associates the clinical, biological, and histological patterns of primary biliary cirrhosis without serum anti-mitochondrial antibodies. We report a case of this disease in a 67-year-old female. The patient presented with jaundice and marked biological cholestasis associated with pulmonary fibrosis and salivary and lacrymal sicca syndrome. Serum anti-smooth muscle antibodies were found without anti-mitochondrial antibodies. Corticotherapy resulted in rapid improvement of clinical and hepatic abnormalities, as well as of pulmonary lesions. The patient was still healthy 18 months later, with low dose corticotherapy. This report emphasizes the possible effectiveness of corticotherapy in autoimmune cholangitis.
自身免疫性胆管炎是一种罕见的慢性肝病病因，最近才被描述，它具有原发性胆汁性肝硬化的临床、生物学和组织学特征，但血清中无抗线粒体抗体。我们报告一例67岁女性患此病的病例。该患者出现黄疸和明显的生物学胆汁淤积，伴有肺纤维化以及唾液腺和泪腺干燥综合征。血清中发现抗平滑肌抗体，但无抗线粒体抗体。皮质激素治疗使临床和肝脏异常以及肺部病变迅速改善。18个月后，患者在低剂量皮质激素治疗下仍健康。本报告强调了皮质激素治疗在自身免疫性胆管炎中可能的有效性。
