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Symptom development and prognosis in primary biliary cirrhosis: A study in two centers
Open accessAbstract
The prognosis of patients with asymptomatic primary biliary cirrhosis has been uncertain. Cases of primary biliary cirrhosis in 95 patients from two centers are presented: 70 patients from a regional referral center (Freeman Hospital, FH) and 25 from an international tertiary referral center (King's College Hospital, KCH) with similar mean age at diagnosis and duration of follow-up (median, 12 months). During follow-up, 19 of 70 FH patients and 15 of 25 KCH patients developed symptoms (P less than 0.001); the mean time to symptom appearance was 43 months at FH and 35 months at KCH (P = 0.0012). Twenty-five of 95 patients died, 15 of 34 subjects who had developed symptoms (all liver-related deaths) and 10 of 61 who remained asymptomatic (all non-liver-related deaths) (P less than 0.001). Hepatic mortality was worse than among the normal population (P less than 0.05). A comparison of those patients developing symptoms with 111 at FH and 165 at KCH who presented with symptoms suggests no difference in survival once hepatic symptoms appear. In both centers, hepatic mortality was strongly related to symptom appearance in asymptomatic primary biliary cirrhosis: patients developing symptoms resemble those with symptoms at presentation. Other differences between the centers may reflect different referral patterns.
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A substantial proportion of asymptomatic PBC patients later develop symptoms, with 36% to 89% becoming symptomatic over average follow-up of 4.5 to 17.8 years.
“All of these studies provide evidence of progressive disease in a substantial proportion of patients, with between 36% and 89% becoming symptomatic during average follow-up periods ranging from 4.5 to 17.8 years and a median time from diagnosis to the appearance of symptoms between 2 and 4.2 years.”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [46]
The Abstract reports that 19 of 70 patients at Freeman Hospital and 15 of 25 at King's College Hospital developed symptoms—34 of 95 patients, or about 36%. The Results give a median follow-up of 69 months for initially asymptomatic patients. This study supports the lower symptom-development figure, but does not itself report 89% or average follow-up spanning 4.5 to 17.8 years; those figures in the citing sentence refer to the studies collectively. (LLM, conf=0.88)
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Metadata
- Authors
- Mitchison, H. C., Lucey, M. R., Kelly, P. J., Neuberger, J. M., Williams, R., & James, O. F. W.
- Affiliations
- University of Michigan (US)
- Venue
- Gastroenterology
- Publisher
- Elsevier BV
- Published
- 1990-09
- Volume
- 99
- Issue
- 3
- Pages
- 778-784
- ISSN
- 0016-5085 (ISSN-L)
- OpenAlex
- W1799876023
- Cited by
- 74
- References
- 16
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 1 · 2023: 2 · 2021: 1 · 2020: 2 · 2018: 4 · 2017: 2 · 2014: 2
- FWCI
- 1.90
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gastroenterology (publishedVersion)
- PubMed (publishedVersion)
- 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 disease, symptom development, prognosis, survival, time to symptom onset, referral patterns, liver-related mortality
- MeSH terms
- Adult, Aged, Female, Follow-Up Studies, Humans, Liver Cirrhosis, Biliary, Liver Function Tests, Male, Middle Aged, Prognosis, Retrospective Studies, Survival Rate
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