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一例晚期硬化性胆管炎合并自身免疫性胰腺炎,对类固醇治疗无反应。

A case of advanced-stage sclerosing cholangitis with autoimmune pancreatitis not responsive to steroid therapy.

作者信息

Nakazawa Takahiro, Naitoh Itaru, Ando Tomoaki, Hayashi Kazuki, Okumura Fumihiro, Miyabe Katsuyuki, Yoshida Michihiro, Ohara Hirotaka, Joh Takashi

机构信息

Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.

出版信息

JOP. 2010 Jan 8;11(1):58-60.

PMID:20065555
Abstract

CONTEXT

Autoimmune pancreatitis and sclerosing cholangitis associated with autoimmune pancreatitis respond well to steroid therapy. Some criteria used for the differential diagnosis of autoimmune pancreatitis and sclerosing cholangitis with autoimmune pancreatitis include the response to a steroid trial.

CASE REPORT

A 68-year-old woman was diagnosed as having type 3 sclerosing cholangitis with autoimmune pancreatitis four years after clinical onset. Seven years after clinical onset, imaging findings revealed multiple pancreatic stones in an atrophic pancreas, stenosis of the main pancreatic duct in the head of the pancreas and upstream dilatation and a longer stretch of stenosis in the hilar hepatic region. We tried steroid therapy in an attempt to ameliorate stenosis of both the bile duct and the pancreatic duct and prevent further progression. Neither lesion responded to steroid therapy.

CONCLUSION

Advanced-stage sclerosing cholangitis with autoimmune pancreatitis may sometimes be unresponsive to steroid therapy, and this should be borne in mind when attempting a steroid trial for the diagnosis of sclerosing cholangitis with autoimmune pancreatitis. Early administration of steroid is important for the prevention of disease progression.

摘要

背景

自身免疫性胰腺炎以及与自身免疫性胰腺炎相关的硬化性胆管炎对类固醇疗法反应良好。用于自身免疫性胰腺炎与自身免疫性胰腺炎相关的硬化性胆管炎鉴别诊断的一些标准包括对类固醇试验的反应。

病例报告

一名68岁女性在临床发病四年后被诊断为3型硬化性胆管炎合并自身免疫性胰腺炎。临床发病七年后,影像学检查发现萎缩胰腺内有多个胰石,胰头主胰管狭窄及上游扩张,肝门区狭窄范围更长。我们尝试使用类固醇疗法以改善胆管和胰管狭窄并防止病情进一步发展。但两个病变对类固醇疗法均无反应。

结论

晚期自身免疫性胰腺炎相关的硬化性胆管炎有时可能对类固醇疗法无反应,在尝试使用类固醇试验诊断自身免疫性胰腺炎相关的硬化性胆管炎时应牢记这一点。早期给予类固醇对于预防疾病进展很重要。

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