Connective tissue diseases in primary biliary cirrhosis: a population-based cohort study
Li Wang1, Feng-Chun Zhang, Hua Chen
1Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China.
World Journal of Gastroenterology
|August 22, 2013
Summary
Nearly half of Chinese primary biliary cirrhosis (PBC) patients have connective tissue diseases (CTDs). Overlapping CTDs, like Sjögren
Area of Science:
- Hepatology
- Rheumatology
- Autoimmune Diseases
Background:
- Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease.
- The association between PBC and connective tissue diseases (CTDs) is recognized but less studied in Chinese populations.
- Understanding CTD prevalence in Chinese PBC patients is crucial for comprehensive management.
Purpose of the Study:
- To determine the frequency of CTDs in Chinese patients with PBC.
- To characterize the clinical and laboratory features of CTD overlap in this cohort.
Main Methods:
- A cohort of 322 Chinese PBC patients was screened for CTDs.
- Systemic involvement and clinical/laboratory differences between PBC patients with and without CTDs were assessed.
- Statistical analyses included t-tests, Wilcoxon tests, and chi-squared/Fisher's exact tests.
Main Results:
- 46.6% of PBC patients had at least one CTD.
- Sjögren's syndrome was the most common CTD (36.2%), followed by systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and polymyositis.
- Specific clinical and laboratory features were associated with different CTD overlaps, including fever, elevated ESR, IgG, RF, ILD, renal, and myocardial involvement.
Conclusions:
- Systemic involvement and CTD overlaps are common in Chinese PBC patients.
- PBC patients with overlapping CTDs exhibit distinct clinical and laboratory profiles.
- These features may influence the prognosis of PBC.
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