Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

Li LI; Hui LIU; Wenyan SONG

doi:10.3969/j.issn.1001-5256.2021.08.026

Volume 37 Issue 8

Aug. 2021

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Article Navigation > Journal of Clinical Hepatology > 2021 > 37(8): 1883-1887

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Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

DOI: 10.3969/j.issn.1001-5256.2021.08.026

Li LI^a,
Hui LIU^b,
Wenyan SONG^{c
,
,}

a.
Integrated Traditional Chinese and Western Medicine Treatment Center, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
b.
Department of Pathology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
c.
Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China

Research funding:

Major Scientific and Technological Projects for Prevention and Treatment of Major Infectious Diseases Such as AIDS and Viral Hepatitis (2018ZX10725504);

Key Medical Professional Projects of "Sailing Plan" (ZYLX201819)

Received Date: 2020-12-27
Accepted Date: 2021-01-20
Published Date: 2021-08-20

Abstract

Abstract

Objective To investigate the clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis (IgG4-SC). Methods A retrospective analysis was performed for the clinical data of 25 patients who were diagnosed with IgG4-SC in Beijing YouAn Hospital, Capital Medical University, from January 2014 to September 2020, and an electronic medical record system was used to collect general information, medical history, diagnosis and treatment processes, laboratory examination, imaging data, and pathological data. Clinical features, process of confirmed diagnosis, and cause of misdiagnosis were analyzed. Normally distributed continuous data were expressed as mean±SD, and the non-normally distributed continuous data were expressed as M(P_{minimum value}-P_{maximum value}). Results Among the 25 patients with IgG4-SC, there were 21 (84%) male patients, and the mean age was 57.61±9.73 years. Common initial symptoms included jaundice and/or yellow urine in 18 patients (78.26%) and weakness in 9 patients (39.13%). As for imaging classification, 16 patients (64%) had type Ⅰ IgG4-SC, no patient had type Ⅱa IgG4-SC, 4 (16%) had IIb IgG4-SC, 1 (4%) had type Ⅲ IgG4-SC, and 4 (16%) had type Ⅳ IgG4-SC. Pancreatic involvement was observed in 22 patients (88%). Glucocorticoids were the main treatment method, and total bilirubin achieved a mean reduction of 67.48% within 2 weeks. Among the 22 patients with a confirmed diagnosis in our hospital, 15 (68.18%) were suspected of "space-occupying lesions in the pancreatic and biliary system" and 5 (22.73%) were suspected of obstructive jaundice at the outpatient service. Understanding of IgG4-SC was achieved by postoperative pathology in the early stage, then imaging doctors gradually became aware of this disease, and finally multidisciplinary consultation made more physicians understand the disease, which helped to make a confirmed diagnosis earlier. Conclusion For patients with obstructive jaundice, clinical physicians need to identify IgG4-SC based on clinical manifestations and especially imaging features. Multidisciplinary consultation involving imaging and pathology plays an important role in helping clinicians understand this disease and making a confirmed diagnosis.
- Immunoglobulin G,
- Cholangitis, Sclerosing,
- Diagnosis, Differential,
- Therapeutics

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Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

DOI: 10.3969/j.issn.1001-5256.2021.08.026