Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy
Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy
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DOI：
10.1053/j.gastro.2007.12.009
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发表时间：
2008-03-01
期刊：
影响因子：
29.4
通讯作者：
Farnell, Michael B.
中科院分区：
文献类型：
--
作者：
Ghazale, Amaar;Chari, Suresh T.;Farnell, Michael B.
背景和目标：免疫球蛋白（IG）G4相关性胆管炎（IAC）是一种类固醇反应性多系统纤维炎性疾病的胆道表现，其中受累器官具有富含IgG 4阳性细胞的特征性淋巴浆细胞浸润。我们描述了IAC的临床特征、治疗反应和复发的预测因素，并比较了胰腺内和近端胆管狭窄IAC的复发率。方法：我们回顾了53例IAC患者的临床、血清学、影像学特征和治疗反应。结果如下：IAC患者通常为老年男性（平均年龄62岁）（85%），表现为梗阻性黄疸（77%），伴有自身免疫性胰腺炎（92%）、血清IgG 4水平升高（74%）和胆管活检标本中大量IgG 4阳性细胞（88%）。出现时，51%的胆管狭窄局限于胰内胆管; 49%累及近端肝外/肝内胆管。初始表现为类固醇治疗（n = 30;中位随访期，29.5个月），手术切除（n = 18;中位随访期，58个月），或保守治疗（n = 5;中位随访期，35个月）。53%的患者在停用类固醇后复发; 44%的患者在手术后复发并接受类固醇治疗。近端肝外/肝内狭窄的存在预示着复发。类固醇治疗使61%的肝酶水平恢复正常; 18例患者中有17例可以取出胆道支架。15名接受类固醇治疗的患者在类固醇停药后复发，有反应; 7名接受额外免疫调节药物的患者仍处于无类固醇缓解状态（中位随访期为6个月）。结论：在不明原因的胆管狭窄伴血清IgG 4升高和不明原因的胰腺疾病中应怀疑IAC。停用类固醇后复发很常见，尤其是近端狭窄。免疫调节药物对复发的作用有待进一步研究。
Background & Aims: Immunoglobulin (Ig)G4-associated cholangitis (IAC) is the biliary manifestation of a steroid-responsive multisystem fibroinflammatory disorder in which affected organs have a characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells. We describe clinical features, treatment response, and predictors of relapse in IAC and compare relapse rates in IAC with intrapancreatic vs proximal bile duct strictures. Methods: We reviewed clinical, serologic, and imaging characteristics and treatment response in 53 IAC patients. Results: IAC patients generally were older (mean age, 62 y) men (85%), presenting with obstructive jaundice (77%) associated with autoimmune pancreatitis (92%), increased serum IgG4 levels (74%), and abundant IgG4-positive cells in bile duct biopsy specimens (88%). At presentation, biliary strictures were confined to the intrapancreatic bile duct in 51%; the proximal extrahepatic/intrahepatic ducts were involved in 49%. Initial presentation was treated with steroids (n = 30; median follow-up period, 29.5 months), surgical resection (n = 18; median follow-up period, 58 months), or was conservative (n = 5; median follow-up period, 35 months). Relapses occurred in 53% after steroid withdrawal; 44% relapsed after surgery and were treated with steroids. The presence of proximal extrahepatic/intrahepatic strictures was predictive of relapse. Steroid therapy normalized liver enzyme levels in 61%; biliary stents could be removed in 17 of 18 patients. Fifteen patients treated with steroids for relapse after steroid withdrawal responded; 7 patients on additional immunomodulatory drugs remain in steroid-free remission (median follow-up period, 6 months). Conclusions: IAC should be suspected in unexplained biliary strictures associated with increased serum IgG4 and unexplained pancreatic disease. Relapses are common after steroid withdrawal, especially with proximal strictures. The role of immunomodulatory drugs for relapses needs further study.