Diminished survival in asymptomatic primary biliary cirrhosis
Open accessAbstract
Data from 73 asymptomatic patients with primary biliary cirrhosis were analyzed to determine clinical course and long-term survival. Of these, 44 entered a D-penicillamine treatment trial; 29 qualified but chose not to participate. Median follow-up was 7.6 yr (range, 2.8-12.2 yr). Liver biopsy at the initial visit showed advanced disease (fibrosis, cirrhosis) in 61% of the patients. During prospective clinical follow-up, which was available for 37 of the 44 study patients, one or more symptoms of liver disease developed in 33 (89%); esophageal varices were found in 15 (41%), and histologic progression to cirrhosis was found in 20 (67%) of the 30 precirrhotic patients. Significant (p less than 0.01) biochemical progression was reflected by a decrease in mean serum albumin concentrations and an increase in mean serum bilirubin levels in 32 patients followed for 4-6 yr. Survival data were available for all 73 patients; 17 died (11 secondary to liver failure), and 1 underwent liver transplantation. These patients had a 4-fold increase in mortality rate (p less than 0.001) compared with the U.S. population matched for age, race, and sex.
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Survival of both symptomatic and asymptomatic patients with PBC is significantly less than that of the general population.
“More recent studies with longer follow-up indicate that, although asymptomatic disease tends to progress at a much slower rate than symptomatic disease, survival of both symptomatic and asymptomatic patients with PBC is significantly less than that of the general population.125 126”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [125]
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- Authors
- Balasubramaniam, K., Grambsch, P. M., Wiesner, R. H., Lindor, K. D., & Dickson, E. R.
- Affiliations
- Mayo Clinic (US)
- ORCID
- Lindor, Keith D.
- Venue
- Gastroenterology
- Publisher
- Elsevier BV
- Published
- 1990-06
- Volume
- 98
- Issue
- 6
- Pages
- 1567-1571
- ISSN
- 0016-5085 (ISSN-L)
- OpenAlex
- W140612521
- Cited by
- 141
- References
- 24
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 2 · 2023: 1 · 2022: 1 · 2021: 2 · 2020: 1 · 2018: 7 · 2017: 5 · 2016: 1 · 2014: 1 · 2013: 1 · 2012: 3
- FWCI
- 4.06
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gastroenterology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (DK34238 02, R01DK034238)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Hepatology › Liver Disease and Transplantation
- Keywords
- primary biliary cirrhosis, asymptomatic patients, long-term survival, mortality, D-penicillamine, disease progression, esophageal varices, liver failure, serum albumin, serum bilirubin, liver transplantation
- MeSH terms
- Adult, Aged, Bilirubin, Female, Follow-Up Studies, Humans, Liver, Liver Cirrhosis, Biliary, Male, Middle Aged, Penicillamine, Prospective Studies, Serum Albumin, Survival Rate
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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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