Geoepidemiology of Primary Biliary Cholangitis: Lessons from Switzerland
No data on primary biliary cholangitis (PBC) are available in Switzerland. We established a national patient cohort to obtain information on PBC phenotypes and disease course in Switzerland. Local databases in all university hospitals and in two large secondary centers were searched for case finding...
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| Published in: | Clinical reviews in allergy & immunology Vol. 54; no. 2; pp. 295 - 306 |
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| Main Authors: | , , , , , , , , , , , , , , , |
| Format: | Journal Article |
| Language: | English |
| Published: |
New York
Springer US
01.04.2018
Springer Springer Nature B.V |
| Subjects: | |
| ISSN: | 1080-0549, 1559-0267, 1559-0267 |
| Online Access: | Get full text |
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| Summary: | No data on primary biliary cholangitis (PBC) are available in Switzerland. We established a national patient cohort to obtain information on PBC phenotypes and disease course in Switzerland. Local databases in all university hospitals and in two large secondary centers were searched for case finding. In addition, all primary care physicians, gastroenterologists, rheumatologists, and dermatologists were invited to contribute patients from their own medical records. PBC diagnosis was centrally reviewed. Five hundred one PBC patients were identified, 474 were included in data analysis, and 449 of them were enrolled by tertiary centers. The catchment area accounts for approximately one third of the Swiss population or approximately 2.8 million inhabitants. The median age at diagnosis was 53 years, 84% were women, and 86% were anti-mitochondrial antibody positive. The median follow-up was 5.4 years, 12.6% experienced a liver-related endpoint. Splenomegaly was present at diagnosis in one quarter of patients and in half of male patients. Approximately one third were non-responders to ursodeoxycholic acid (UDCA). The median transplant-free survival at 10 years was 85%. The following variables were independently associated with poor outcome: low platelet count at baseline (HR = 0.99,
p
< 0.0001), elevated alkaline phosphatase at baseline (HR = 1.36,
p
< 0.0001), elevated bilirubin at baseline (HR = 1.11,
p
= 0.001), and elevated alanine aminotransaminase (HR = 1.35,
p
= 0.04) after 12 months of UDCA therapy. The AUROC for the
UK-PBC risk score
at 5, 10, and 15 years was 0.82. The AUROC for the
Globe score
at 5, 10, and 15 years was 0.77. Patients included in this study are currently being enrolled in a prospective nationwide registry with biobank, taking advantage of the collaboration network generated by this study. Our study provides the first snapshot of PBC in Switzerland, describing a diagnostic delay with one quarter of patients diagnosed when already in the cirrhotic stage. We were also able to externally validate the UK-PBC risk score and the Globe score. The ongoing nationwide prospective registry will be fundamental to improve disease awareness and interdisciplinary collaborations and will serve as a platform for clinical and translational research.
Trial registration number:
clinicaltrials.gov
: NCT02846896; SNCTP000001870 |
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| Bibliography: | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 ObjectType-Article-2 ObjectType-Feature-1 content type line 23 |
| ISSN: | 1080-0549 1559-0267 1559-0267 |
| DOI: | 10.1007/s12016-017-8656-x |