Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study

Objectives. Diagnosis and treatment of primary biliary cholangitis (PBC) are often complicated by hepatic and/or extrahepatic manifestations, which in turn affect the natural course and prognosis of PBC. This study evaluated the clinical characteristics and prognosis of PBC co-occurring with intrahe...

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Main Authors: Yuwei Liu, Kai Han, Chen Liu, Fangfang Duan, Jun Cheng, Song Yang
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Canadian Journal of Gastroenterology and Hepatology
Online Access:http://dx.doi.org/10.1155/2021/5557814
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author Yuwei Liu
Kai Han
Chen Liu
Fangfang Duan
Jun Cheng
Song Yang
author_facet Yuwei Liu
Kai Han
Chen Liu
Fangfang Duan
Jun Cheng
Song Yang
author_sort Yuwei Liu
collection DOAJ
description Objectives. Diagnosis and treatment of primary biliary cholangitis (PBC) are often complicated by hepatic and/or extrahepatic manifestations, which in turn affect the natural course and prognosis of PBC. This study evaluated the clinical characteristics and prognosis of PBC co-occurring with intrahepatic and extrahepatic autoimmune disease (AID). Methods. Clinical data of patients with PBC who were admitted to the Beijing Ditan Hospital from September 2008 to December 2014 were retrospectively reviewed, assessed for other autoimmune diseases, and analyzed statistically. All patients received ursodeoxycholic acid (UDCA) treatment. Results. Data from 505 patients were evaluated. Approximately 35.0% of patients had at least one additional AID. AIDs included Sjögren’s syndrome (SS; 26.3%), autoimmune hepatitis (AIH; 7.1%), rheumatoid arthritis (RA; 1.4%), hypothyroidism (0.8%), Graves’s thyroiditis (0.6%), systemic lupus erythematosus (SLE; 0.4%), and Hashimoto’s thyroiditis (0.2%). No differences in response rates of UDCA were found between the PBC group and the PBC-SS group or PBC complicated with AID group (both P>0.05). White blood cell (WBC, RR = 1.072, 95% CI: 1.016–1.130, P=0.011), platelet counts (PLT, RR = 0.995, 95% CI: 0.992–0.998, P=0.003), and prothrombin time and international normalized ratio (PT/INR, RR = 1.799, 95% CI: 1.010–3.206, P=0.046) were independent prognostic factors in patients with PBC. The overall survival time of patients in PBC-AIH and PBC-SS groups was shorter than that of those with PBC (P<0.001). Conclusions. AIH was the most common in hepatic comorbidity. SS was the most frequent extrahepatic comorbidity. WBC, PLT, and PT/INR were independent prognostic factors in patients with PBC. AID coexisted with PBC impaired patients’ survival.
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spelling doaj-art-e1000800306d4674b427bca4221bf3fd2025-08-20T03:26:00ZengWileyCanadian Journal of Gastroenterology and Hepatology2291-27892291-27972021-01-01202110.1155/2021/55578145557814Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective StudyYuwei Liu0Kai Han1Chen Liu2Fangfang Duan3Jun Cheng4Song Yang5Emergency Department, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaInstitute of Infectious Diseases, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaCenter of Hepatology, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaCenter of Hepatology, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaCenter of Hepatology, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaCenter of Hepatology, Beijing Ditan Hospital, Capital Medical University, Beijing 100015, ChinaObjectives. Diagnosis and treatment of primary biliary cholangitis (PBC) are often complicated by hepatic and/or extrahepatic manifestations, which in turn affect the natural course and prognosis of PBC. This study evaluated the clinical characteristics and prognosis of PBC co-occurring with intrahepatic and extrahepatic autoimmune disease (AID). Methods. Clinical data of patients with PBC who were admitted to the Beijing Ditan Hospital from September 2008 to December 2014 were retrospectively reviewed, assessed for other autoimmune diseases, and analyzed statistically. All patients received ursodeoxycholic acid (UDCA) treatment. Results. Data from 505 patients were evaluated. Approximately 35.0% of patients had at least one additional AID. AIDs included Sjögren’s syndrome (SS; 26.3%), autoimmune hepatitis (AIH; 7.1%), rheumatoid arthritis (RA; 1.4%), hypothyroidism (0.8%), Graves’s thyroiditis (0.6%), systemic lupus erythematosus (SLE; 0.4%), and Hashimoto’s thyroiditis (0.2%). No differences in response rates of UDCA were found between the PBC group and the PBC-SS group or PBC complicated with AID group (both P>0.05). White blood cell (WBC, RR = 1.072, 95% CI: 1.016–1.130, P=0.011), platelet counts (PLT, RR = 0.995, 95% CI: 0.992–0.998, P=0.003), and prothrombin time and international normalized ratio (PT/INR, RR = 1.799, 95% CI: 1.010–3.206, P=0.046) were independent prognostic factors in patients with PBC. The overall survival time of patients in PBC-AIH and PBC-SS groups was shorter than that of those with PBC (P<0.001). Conclusions. AIH was the most common in hepatic comorbidity. SS was the most frequent extrahepatic comorbidity. WBC, PLT, and PT/INR were independent prognostic factors in patients with PBC. AID coexisted with PBC impaired patients’ survival.http://dx.doi.org/10.1155/2021/5557814
spellingShingle Yuwei Liu
Kai Han
Chen Liu
Fangfang Duan
Jun Cheng
Song Yang
Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
Canadian Journal of Gastroenterology and Hepatology
title Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
title_full Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
title_fullStr Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
title_full_unstemmed Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
title_short Clinical Characteristics and Prognosis of Concomitant Primary Biliary Cholangitis and Autoimmune Diseases: A Retrospective Study
title_sort clinical characteristics and prognosis of concomitant primary biliary cholangitis and autoimmune diseases a retrospective study
url http://dx.doi.org/10.1155/2021/5557814
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