Hepatology
Signal score, how each part rates
ActionabilityPrevalenceStakesEvidence strengthNovelty
See the full rubric →Extrahepatic Autoimmune Disease in People With Primary Biliary Cholangitis: Nationwide Occurrence and Relation to Outcome.
Clinical takeawayBe aware that EHAIDs are common in PBC patients, but they do not appear to influence liver outcomes.
What it found27.3% of PBC patients had extrahepatic autoimmune diseases (EHAIDs), most commonly hypothyroidism (35.6%) and Sjögren's syndrome (18.3%), with 80.7% of EHAIDs present at baseline. No association was found between EHAIDs and liver transplant-free survival (aHR 0.81, 95% CI 0.63-1.05) or disease progression (aHR 0.92, 95% CI 0.65-1.32).
ContextConfirms EHAIDs are frequent in PBC but do not impact liver prognosis, aligning with prior evidence but providing robust nationwide data.
Reinforcessuggested applicable standard· American Association for the Study of Liver Diseases, 'Primary Biliary Cholangitis: 2021 Practice Guidance', 2021
Decision at stakethe presence of extrahepatic autoimmune diseases does not alter the management of primary biliary cholangitis
American Association for the Study of Liver Diseases, 'Primary Biliary Cholangitis: 2021 Practice Guidance', 2021 · reviewed 2026-07-21 ↗Treat with first-line ursodeoxycholic acid (UDCA) 13-15 mg/kg/d divided, and assess biochemical response at 12 months using Paris-II plus continuous risk scores (Globe, UK-PBC). Escalate to a second-line agent (obeticholic acid [contraindicated in cirrhosis with portal hypertension or prior decompensation], seladelpar, elafibranor, or a fibrate) when response is inadequate, guided by a Child-Pugh/portal-hypertension hard gate, and manage pruritus with a stepwise ladder (cholestyramine → rifampin → sertraline → naltrexone). Refer for transplant evaluation for decompensation, refractory pruritus, or rising bilirubin.
Weijsters GHX … Dutch PBC Study Group · Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association · IF 16.2 · PubMed ↗