内科理论与实践 ›› 2022, Vol. 17 ›› Issue (01): 24-28.doi: 10.16138/j.1673-6087.2022.01.005
杨松, 李玥
收稿日期:2022-01-21
出版日期:2022-02-28
发布日期:2022-07-25
基金资助:
Received:2022-01-21
Online:2022-02-28
Published:2022-07-25
中图分类号:
杨松, 李玥. 原发性硬化性胆管炎的诊断与鉴别诊断[J]. 内科理论与实践, 2022, 17(01): 24-28.
图1
日本IgG4-SC根据胆管影像分型示意图[22]
表1
IgG4-SC诊断标准（2020日本标准）[22]
| 诊断指标和标准 |
|---|
| 诊断指标 Ⅰ. 存在肝内和（或）肝外胆管狭窄证据 |
| a. ERCP提示肝内和（或）肝外胆管狭窄 |
| b. MRCP提示肝内和（或）肝外胆管狭窄 |
| Ⅱ. 存在胆管壁增厚证据 |
| a. ESU/IDUS发现胆管壁增厚证据 |
| b. CT/MRI/超声发现胆管壁增厚证据 |
| Ⅲ. 血清学检测 |
| 血清IgG4水平升高≥1 350 mg/L |
| Ⅳ. 病理证据 |
| a. 病理发现（i）+（ii）+（v） |
| b. 病理发现（v） |
| c. 在上述（i）+（ii）+（v）基础上发现（iii）和（或）（iv） |
| （i）明显的淋巴细胞浆细胞浸润和纤维化 |
| （ii）每高倍视野IgG4阳性浆细胞>10个 |
| （iii）席纹状纤维化 |
| （iv）闭塞性静脉炎 |
| （v）未见肿瘤细胞 |
| Ⅴ. IgG4相关疾病中其他脏器受累证据 |
| a. Ⅰ型AIP b. IgG4相关泪腺炎/唾液腺炎（Mikulicz病）、IgG4相关腹膜 后纤维化、IgG4相关肾脏病变 |
| Ⅵ. 糖皮质激素治疗有效 一般建议激素治疗前应完善检查排除肿瘤性疾病,建议在 激素治疗后2周完善ERCP或MRCP评价病变缓解情况, 如胆管病变无缓解要再次排除肿瘤性病变 |
| 诊断标准 |
| 以下情况可以确诊IgG4-SC |
| 满足Ⅰa/b+Ⅱa/b+Ⅲ/Ⅵ+Ⅴa可确诊1或2型IgG4-SC 满足Ⅰa+Ⅱa+Ⅳb+Ⅲ/Ⅵ+Ⅴa可确诊3或4型IgG4-SC 满足Ⅰa+Ⅱa+Ⅲ+Ⅳa/Ⅵ可确诊1~4型IgG4-SC 另外,只要组织病理符合Ⅳc也可以确诊IgG4-SC |
| 以下情况考虑IgG4-SC可能性大 |
| 满足Ⅰa/b+Ⅱa/b+Ⅴa考虑1或2型IgG4-SC可能性大 满足Ⅰa+Ⅱa+Ⅳb+Ⅴa或Ⅰa/b+Ⅱb+Ⅵ+Ⅴa考虑3或4型 IgG4-SC可能性大 满足Ⅰa+Ⅱa+Ⅳa或Ⅰa+Ⅱa+Ⅲ+Ⅳb或Ⅰb+Ⅱa+Ⅲ+Ⅵ考 虑1~4型IgG4-SC可能性大 |
| 以下情况考虑IgG4-SC不能排除 满足Ⅰa/b+Ⅱa+Ⅴa或Ⅰb+Ⅱb+Ⅲ+Ⅴa不能排除3或4型 IgG4-SC 满足Ⅰa+Ⅱa+Ⅲ/Ⅴb/Ⅵ或Ⅰb+Ⅱb+Ⅲ+Ⅵ不能排除1~4型 IgG4-SC |
表2
SSC的病因分类
| 病因分类 | 常见病因 |
|---|---|
| 慢性胆 道梗阻 | 胆总管或肝内胆管结石 手术后胆管损伤（如胆囊切除术后胆管狭窄） 肝移植术后吻合口胆管狭窄 慢性胰腺炎 良、恶性肿瘤压迫胆管 门静脉高压性胆道病等 |
| 感染性 疾病 | 复发性化脓性胆管炎（细菌） 寄生虫（隐孢子虫、微孢子虫、蛔虫、华支睾吸虫） 巨细胞病毒感染 获得性免疫缺陷综合征相关性胆管病变 感染性休克等 |
| 免疫性 疾病 | IgG4-SC或IgG4相关AIP 嗜酸性粒细胞性胆管炎 肥大细胞性胆管病 滤泡性胆管炎 伴粒细胞上皮损害的硬化性胆管炎 淀粉样变性 骨髓移植后移植物抗宿主病等 |
| 肿瘤或炎 症等浸润 胆道系统 | 恶性肿瘤（如胆管癌等） 结节病 朗格汉斯细胞组织细胞增生症（组织细胞增生症X） |
| 缺血性胆 管疾病 | 肝动脉血栓形成（如肝移植术后） 肝移植排斥反应 放射性损伤 肝动脉化疗/栓塞 系统性血管炎 SSC-CIP |
| 药物、毒 物所致 | 手术暴露（甲醛、乙醇或高渗氯化钠溶液意外注入胆管） 药物性损伤（如氯胺酮、塞来昔布、七氟烷、阿莫西林克拉维酸、阿托伐他汀、英夫利西单抗、PD-1抑制剂等） |
| 遗传性 疾病 | 胆汁转运蛋白缺陷（如MDR3基因缺乏） 胆管板畸形/纤维多囊性肝脏疾病 囊性纤维化 CD40配体缺陷（X连锁高IgM综合征） |
| [1] | 中华医学会肝病学分会. 原发性硬化性胆管炎诊断及治疗指南(2021)[J]. 中华内科杂志, 2021, 60(12): 1050-1074. |
| [2] |
Karlsen TH, Folseraas T, Thorburn D, et al. Primary sclerosing cholangitis—a comprehensive review[J]. J Hepatol, 2017, 67(6): 1298-1323.
doi: S0168-8278(17)32196-7 pmid: 28802875 |
| [3] |
Mehta TI, Weissman S, Fung BM, et al. Global incidence, prevalence and features of primary sclerosing cholangitis: a systematic review and meta-analysis[J]. Liver Int, 2021, 41(10): 2418-2426.
doi: 10.1111/liv.15007 pmid: 34224208 |
| [4] | Trivedi PJ, Bowlus CL, Yimam KK, et al. Epidemiology, natural history, and outcomes of primary sclerosing cholangitis[J]. Clin Gastroenterol Hepatol, 2021.[Epub ahead of print]. |
| [5] | Mertz A, Nguyen NA, Katsanos KH, et al. Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence[J]. Ann Gastroenterol, 2019, 32(2): 124-133. |
| [6] |
Lundberg Båve AA, Bergquist A, Bottai M, et al. Increased risk of cancer in patients with primary sclerosing cholangitis[J]. Hepatol Int, 2021, 15(5): 1174-1182.
doi: 10.1007/s12072-021-10214-6 pmid: 34357546 |
| [7] |
Prokopič M, Beuers U. Management of primary sclerosing cholangitis and its complications: an algorithmic approach[J]. Hepatol Int, 2021, 15(1): 6-20.
doi: 10.1007/s12072-020-10118-x pmid: 33377990 |
| [8] |
Ponsioen CY, Assis DN, Boberg KM, et al. Defining primary sclerosing cholangitis: results from an International Primary Sclerosing Cholangitis Study Group Consensus Process[J]. Gastroenterology, 2021, 161(6): 1764-1775.
doi: 10.1053/j.gastro.2021.07.046 pmid: 34384749 |
| [9] |
Moiseev S, Cohen Tervaert JW, et al. 2020 international consensus on ANCA testing beyond systemic vasculitis[J]. Autoimmun Rev, 2020, 19(9): 102618.
doi: 10.1016/j.autrev.2020.102618 URL |
| [10] |
Burak KW, Angulo P, Lindor KD. Is there a role for liver biopsy in primary sclerosing cholangitis?[J]. Am J Gastroenterol, 2003, 98(5): 1155-1158.
doi: 10.1111/j.1572-0241.2003.07401.x URL |
| [11] |
Keihanian T, Barakat MT, Tejaswi S, et al. Role of endoscopic retrograde cholangiopancreatography in the diagnosis and management of cholestatic liver diseases[J]. Clin Liver Dis, 2022, 26(1): 51-67.
doi: 10.1016/j.cld.2021.08.006 pmid: 34802663 |
| [12] |
Lüth S, Kanzler S, Frenzel C, et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome[J]. J Clin Gastroenterol, 2009, 43(1): 75-80.
doi: 10.1097/MCG.0b013e318157c614 URL |
| [13] |
Aabakken L, Karlsen TH, Albert J, et al. Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline[J]. Endoscopy, 2017, 49(6): 588-608.
doi: 10.1055/s-0043-107029 pmid: 28420030 |
| [14] |
Chapman MH, Thorburn D, Hirschfield GM, et al. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis[J]. Gut, 2019, 68(8): 1356-1378.
doi: 10.1136/gutjnl-2018-317993 URL |
| [15] |
Isayama H, Tazuma S, Kokudo N, et al. Clinical guidelines for primary sclerosing cholangitis 2017[J]. J Gastroenterol, 2018, 53(9): 1006-1034.
doi: 10.1007/s00535-018-1484-9 URL |
| [16] |
de Vries EM, Verheij J, Hubscher SG, et al. Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis[J]. J Hepatol, 2015, 63(5): 1212-1219.
doi: 10.1016/j.jhep.2015.06.008 URL |
| [17] |
European Association for the Study of the Liver. EASL clinical practice guidelines: autoimmune hepatitis[J]. J Hepatol, 2015, 63(4): 971-1004.
doi: 10.1016/j.jhep.2015.06.030 URL |
| [18] | 中华医学会肝病学分会. 自身免疫性肝炎诊断和治疗指南(2021)[J]. 中华内科杂志, 2021, 60(12): 1038-1049. |
| [19] |
Tanaka A, Tazuma S, Okazaki K, et al. Clinical features, response to treatment, and outcomes of IgG4-related sclerosing cholangitis[J]. Clin Gastroenterol Hepatol, 2017, 15(6): 920-926.
doi: 10.1016/j.cgh.2016.12.038 URL |
| [20] | 张文, 董凌莉, 朱剑, 等. IgG 4相关性疾病诊治中国专家共识[J]. 中华内科杂志, 2021, 60(3): 192-206. |
| [21] |
Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis[J]. J Hepatobiliary Pancreat Sci, 2019, 26(1): 9-42.
doi: 10.1002/jhbp.596 URL |
| [22] |
Nakazawa T, Kamisawa T, Okazaki K, et al. Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020[J]. J Hepatobiliary Pancreat Sci, 2021, 28(3): 235-242.
doi: 10.1002/jhbp.913 URL |
| [23] |
Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-related disease[J]. Arthritis Rheumatol, 2020, 72(1): 7-19.
doi: 10.1002/art.41120 URL |
| [24] |
Manganis CD, Chapman RW, Culver EL. Review of primary sclerosing cholangitis with increased IgG4 levels[J]. World J Gastroenterol, 2020, 26(23): 3126-3144.
doi: 10.3748/wjg.v26.i23.3126 URL |
| [25] |
Lapidot Y, Amir A, Ben-Simon S, et al. Alterations of the salivary and fecal microbiome in patients with primary sclerosing cholangitis[J]. Hepatol Int, 2021, 15(1): 191-201.
doi: 10.1007/s12072-020-10089-z URL |
| [26] | Liu Q, Li B, Li Y, et al. Altered faecal microbiome and metabolome in IgG4-related sclerosing cholangitis and primary sclerosing cholangitis[J]. Gut, 2021.[Epub ahead of print]. |
| [27] |
Moon SH, Kim MH, Lee JK, et al. Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis[J]. J Gastroenterol, 2017, 52(4): 483-493.
doi: 10.1007/s00535-016-1246-5 URL |
| [28] |
Navaneethan U, Njei B, Venkatesh PG, et al. Fluorescence in situ hybridization for diagnosis of cholangiocarcinoma in primary sclerosing cholangitis: a systematic review and meta-analysis[J]. Gastrointest Endosc, 2014, 79(6): 943-950.
doi: 10.1016/j.gie.2013.11.001 pmid: 24360654 |
| [29] | Navaneethan U, Njei B, Venkatesh PG, et al. Endoscopic ultrasound in the diagnosis of cholangiocarcinoma as the etiology of biliary strictures: a systematic review and meta-analysis[J]. Gastroenterol Rep (Oxf), 2015, 3(3): 209-215. |
| [30] |
Weilert F, Bhat YM, Binmoeller KF, et al. EUS-FNA is superior to ERCP-based tissue sampling in suspected malignant biliary obstruction: results of a prospective, single-blind, comparative study[J]. Gastrointest Endosc, 2014, 80(1): 97-104.
doi: 10.1016/j.gie.2013.12.031 pmid: 24559784 |
| [31] | Scheid JF, Rosenbaum MW, Przybyszewski EM, et al. Next-generation sequencing in the evaluation of biliary strictures in patients with primary sclerosing cholangitis[J]. Cancer Cytopathol, 2021.[Epub ahead of print]. |
| [32] |
Onoyama T, Takeda Y, Yamashita T, et al. Programmed cell death-1 inhibitor-related sclerosing cholangitis: a systematic review[J]. World J Gastroenterol, 2020, 26(3): 353-365.
doi: 10.3748/wjg.v26.i3.353 URL |
| [33] | 郑卫萍. 危重患者继发性硬化性胆管炎的临床表现、胆管造影特征、自然病程及转归[J]. 实用器官移植电子杂志, 2016, 4(4): 247. |
| [34] |
Martins P, Verdelho Machado M. Secondary sclerosing cholangitis in critically ill patients[J]. GE Port J Gastroenterol, 2020, 27(2): 103-114.
doi: 10.1159/000501405 pmid: 32266307 |
| [35] | Klindt C, Jensen BE, Brandenburger T, et al. Secondary sclerosing cholangitis as a complication of severe COVID-19: a case report and review of the literature[J]. Clin Case Rep, 2021, 9(5): e04068. |
| [36] |
Durazo FA, Nicholas AA, Mahaffey JJ, et al. Post-covid-19 cholangiopathy: a new indication for liver transplantation[J]. Transplant Proc, 2021, 53(4): 1132-1137.
doi: 10.1016/j.transproceed.2021.03.007 URL |
| [1] | 杨玲, 范慧倩, 严胜琦. 胆汁淤积性肝病治疗进展[J]. 内科理论与实践, 2022, 17(01): 29-37. |
| [2] | 华静, 倪茜茜,. 胆汁淤积性肝病的药物治疗进展[J]. 内科理论与实践, 2018, 13(06): 334-338. |
| [3] | 马雄,. 自身免疫性肝病的诊断和治疗现状[J]. 内科理论与实践, 2009, 4(02): 104-107. |
| [4] | 邓国华,. 自身免疫性肝病临床思维及诊断评述[J]. 内科理论与实践, 2009, 4(02): 108-110. |
| 阅读次数 | ||||||
|
全文 |
|
|||||
|
摘要 |
|
|||||
