Walter Dirk, Hartmann Sylvia, Herrmann Eva, Peveling-Oberhag Jan, Bechstein Wolf O, Zeuzem Stefan, Hansmann Martin-Leo, Friedrich-Rust Mireen, Albert Jörg G
Dirk Walter, Stefan Zeuzem, Mireen Friedrich-Rust Department of Internal Medicine I, Johann Wolfgang Goethe-University Hospital, 60590 Frankfurt, Germany.
World J Gastroenterol. 2017 Feb 14;23(6):1044-1050. doi: 10.3748/wjg.v23.i6.1044.
To investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS).
All patients with diagnosis of sclerosing cholangitis (SC) and histopathological samples such as biopsies or surgical specimens at University Hospital Frankfurt from 2005-2015 were included. Histopathological diagnoses as well as further clinical course were reviewed. Tissue samples of patients without definite diagnosis after complete diagnostic work-up were reviewed regarding presence of eosinophilic infiltration and IgG4 positive plasma cells. Eosinophilic infiltration was as well assessed in a control group of liver transplant donors and patients with primary sclerosing cholangitis.
one hundred and thirty-five patients with SC were included. In 10/135 (13.5%) patients, no potential cause of IBS could be identified after complete diagnostic work-up and further clinical course. After histopathological review, a post-hoc diagnosis of EC was established in three patients resulting in a prevalence of 2.2% (3/135) of all patients with SC as well as 30% (3/10) of patients, where no cause of IBS was identified. 2/3 patients with post-hoc diagnosis of EC underwent surgical resection with suspicion for malignancy. Diagnosis of IgG4-related cholangitis was observed in 7/135 patients (5.1%), whereas 3 cases were discovered in post-hoc analysis. 6/7 cases with IgG4-related cholangitis (85.7%) presented with eosinophilic infiltration in addition to IgG4 positive plasma cells. There was no patient with eosinophilic infiltration in the control group of liver transplant donors ( = 27) and patients with primary sclerosing cholangitis ( = 14).
EC is an underdiagnosed benign etiology of SC and IBS, which has to be considered in differential diagnosis of IBS.
研究嗜酸性胆管炎（EC）的存在情况及范围，以及不明原因胆管狭窄（IBS）患者中IgG4相关疾病的情况。
纳入2005年至2015年在法兰克福大学医院诊断为硬化性胆管炎（SC）且有活检或手术标本等组织病理学样本的所有患者。回顾组织病理学诊断及进一步的临床病程。对经过全面诊断评估后仍未明确诊断的患者的组织样本，就嗜酸性粒细胞浸润和IgG4阳性浆细胞的存在情况进行复查。在肝移植供体对照组和原发性硬化性胆管炎患者中也评估嗜酸性粒细胞浸润情况。
纳入135例SC患者。在10/135（7.4%）例患者中，经过全面诊断评估及进一步临床病程观察后，仍未发现IBS的潜在病因。经组织病理学复查，3例患者事后诊断为EC，在所有SC患者中的患病率为2.2%（3/135），在未发现IBS病因的患者中占30%（3/10）。2/3例事后诊断为EC的患者因怀疑恶性肿瘤而接受了手术切除。在135例患者中有7例（5.1%）诊断为IgG4相关胆管炎，其中3例是在事后分析中发现的。6/7例（85.7%）IgG4相关胆管炎患者除有IgG4阳性浆细胞外，还存在嗜酸性粒细胞浸润。肝移植供体对照组（n = 27）和原发性硬化性胆管炎患者（n = 14）中均无嗜酸性粒细胞浸润的患者。
EC是SC和IBS的一种诊断不足的良性病因，在IBS的鉴别诊断中必须予以考虑。
