Prompt identification of extrahepatic conditions (EHCs) is of key importance for the precise, timely diagnosis of primary biliary cholangitis (PBC), according to findings from a systematic review and meta-analysis published in the journal Clinics and Research in Hepatology and Gastroenterology.
Among patients with the chronic autoimmune disorder PBC, cholestasis is often reported, which can lead to hepatic fibrosis and cirrhosis, and eventually to a fatal outcome. PBC exhibits a female predominance. Over the last few decades, the prognosis of individuals with the disease has been facilitated by the use of ursodeoxycholic acid, leading to symptom improvement, prevention of end-stage liver disease, and amelioration in transplant-free survival.
Recognizing that although many studies have evaluated the prevalence of EHCs among patients with PBC, great heterogeneity exists across the various studies, the researchers sought to perform a systematic review and meta-analysis to establish the prevalence of EHC and its relationship with PBC. All reviews and analyses were performed based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method.
Study inclusion criteria were English-language publications, which comprised cross-sectional, observational, or case-control studies that reported on the frequency, prevalence, and/or risk for EHCs in patients with PBC. A total of 5370 potentially relevant articles were identified, with 158 publications deemed eligible for full-text review. Ultimately, 129 articles fulfilled the study criteria, which involved 133 studies—69 cohort studies, 40 case-control studies, and 24 cross-sectional studies.
Read more about PBC clinical features
The most common systematic autoimmune rheumatic diseases (SARDs) reported among patients with PBC were the following:
- Sjögren’s syndrome (SjS) : 68 studies
- Rheumatoid arthritis (RA): 41 studies
- Systemic sclerosis (SSc): 40 studies
- Raynaud’s syndrome (RS): 30 studies
- Systemic lupus erythematosus (SLE): 20 studies
- Myositis: 15 studies
- Mixed/uncertain connective disease: 6 studies
- Vasculitis: 6 studies
- Ankylosing spondylitis: 3 studies
- Antiphospholipid syndrome: 2 studies
The studies linked to SjS included 5620 cases among 80,622 patients with PBC. This translated to a pooled prevalence of 21.4% (95% CI, 19.2%-23.5%) of SjS in patients with PBC, compared with 3.0% in those without PBC.
Further, the rates of the following conditions were also increased among individuals with PBC compared with those without the disorder:
- Overall thyroid diseases: 11.3%
- Autoimmune thyroid diseases: 9.9%
- Osteoporosis: 21.1%
- Celiac disease: 1.0%
- Chronic bronchitis: 4.6%
“[I]n the present study, not only did we evaluate the prevalence of various EHCs among . . . patients [with PBC] as comprehensively as possible, but we also investigated whether PBC can increase the prevalence of these EHCs by comparing it between . . . patients [with PBC] and non-PBC individuals,” the authors explained. “This is the first exhaustive study on the old theme about EHC of PBC,” they concluded.
