Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis
Open accessAbstract
Backgrounds and aims: To evaluate the prognosis of primary biliary cirrhosis (PBC) together with systemic sclerosis (SSc), as this is unknown. Methods and results: A PBC database of 580 patients identified 43 with PBC and SSc: two patients with PBC alone were matched to each PBC-SSc patient for serum bilirubin concentration at the initial visit. Forty (93%) patients had limited cutaneous SSc. At diagnosis of PBC, median values were: 49.7 years, bilirubin 17 μmol/l, and albumin 40.5 g/l. Liver diagnosis occurred a median 4.9 years after SSc in 24 (56%) patients. In matched patients, median values at diagnosis were: 53.2 years, bilirubin 12 μmol/l, and albumin 41 g/l. Median follow up was similar: 3.16 years (PBC-SSc) and 4.8 years (PBC alone). The risk of transplantation or death from diagnosis, adjusting for sex, age, log bilirubin, and alkaline phosphatase was significantly lower in PBC-SSc (hazard ratio 0.116, p = 0.01) due to less transplantation (hazard ratio 0.068, p = 0.006). The rate of bilirubin increase was less in PBC-SSc (p = 0.04). Overall survival was similar (hazard ratio 1.11, p = 0.948); there were nine deaths (21%) in PBC-SSc (six SSc related and two liver related) and nine (11%) in PBC alone (six liver related). Conclusions: Liver disease has a slower progression in PBC-SSc compared with matched patients with PBC alone.
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Anti-centromere antibodies have been reported to have potential prognostic impact, with a portal hypertensive phenotype.
“Anti-centromere antibodies have also been reported to have potential prognostic impact (portal hypertensive phenotype) [79,80,78].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [79]
The ‘Patients with PBC and ACA positivity’ section reports survival, transplantation and portal-hypertension-related complications in 13 ACA-positive patients. It does not establish that ACA predicts prognosis or identifies a distinct portal hypertensive phenotype; the Discussion says this group behaved as if PBC was the major determinant of outcome. No page number is available in our copy. (LLM, conf=0.78)
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- Authors
- Rigamonti, C., Shand, L. M., Feudjo, M., Bunn, C. C., Black, C. M., Denton, C. P., & Burroughs, A. K.
- Affiliations
- Royal Free London NHS Foundation Trust (GB)
- London School of Hygiene & Tropical Medicine (GB)
- Corresponding author
- Burroughs, A K
- Venue
- Gut
- Publisher
- BMJ
- Published
- 2005-09-08
- Volume
- 55
- Issue
- 3
- Pages
- 388-394
- ISSN
- 0017-5749 (ISSN-L)
- 1468-3288
- PMC
- PMC1856066
- OpenAlex
- W2114160420
- Semantic Scholar
- 15a8d5ff5589e96d41a55b41aebd177e92f22583
- Cited by
- 194
- References
- 62
- Citations by year
- 2026: 9 · 2025: 7 · 2024: 11 · 2023: 12 · 2022: 6 · 2021: 8 · 2020: 12 · 2019: 8 · 2018: 9 · 2017: 15 · 2016: 10 · 2015: 9 · 2014: 8 · 2013: 9 · 2012: 9
- FWCI
- 3.75
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gut (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Hepatology › Liver Disease and Transplantation
- Keywords
- primary biliary cirrhosis, systemic sclerosis, limited cutaneous systemic sclerosis, prognosis, liver transplantation, mortality, bilirubin, disease progression, overall survival
- MeSH terms
- Adult, Aged, Aged, 80 and over, Antibodies, Antinuclear, Bilirubin, Centromere, Epidemiologic Methods, Female, Humans, Liver Cirrhosis, Biliary, Male, Middle Aged, Prognosis, Scleroderma, Systemic, Liver Transplantation, Disease Progression
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