{ "schemaVersion": 2, "eyebrow": "Hepatology", "title": "Primary Biliary Cholangitis", "summary": "Primary biliary cholangitis is usually diagnosed serologically in the setting of cholestatic liver tests. Management centers on prompt ursodeoxycholic acid, biochemical risk reassessment, escalation for inadequate response or intolerance, symptom-directed care, fibrosis assessment, and surveillance for complications.", "seoDescription": "Physician guide to diagnosing, risk stratifying, and managing primary biliary cholangitis, including UDCA, second-line therapies, symptoms, and monitoring.", "clinicalQuestion": "How should physicians confirm, risk stratify, treat, and monitor adults with primary biliary cholangitis?", "specialty": "Hepatology", "audience": "U.S. physicians and medical trainees", "tags": [ "primary biliary cholangitis", "PBC", "ursodeoxycholic acid", "alkaline phosphatase", "antimitochondrial antibody", "cholestatic pruritus", "elafibranor", "obeticholic acid" ], "keyTakeaways": [ "In most patients, a cholestatic liver-test pattern plus a PBC-specific autoantibody, typically antimitochondrial antibody, establishes PBC without liver biopsy. [13]", "Initiate ursodeoxycholic acid promptly and reassess biochemical response after at least 12 months; approximately 40% of patients do not meet response criteria. [18][19]", "Persistent biochemical activity, particularly incomplete alkaline phosphatase normalization, identifies residual risk and should prompt structured reassessment and consideration of approved second-line therapy. [18][23]", "Elafibranor is FDA-approved under accelerated approval with UDCA for inadequate responders or alone when UDCA is intolerable; survival benefit and prevention of decompensation have not been demonstrated. [2]", "Obeticholic acid is contraindicated in advanced cirrhosis, defined by current or prior decompensation or portal-hypertension features; discontinue permanently if cirrhosis progresses to advanced disease or clinically significant liver-related adverse reactions occur. [5][6]" ], "sections": [ { "id": "diagnostic-approach", "eyebrow": "Diagnosis", "heading": "Confirm PBC and exclude biliary obstruction", "intro": "Diagnosis is generally noninvasive, but discordant presentations require a broader cholestasis evaluation.", "paragraphs": [ "For chronic cholestatic liver tests, obtain PBC serology, particularly antimitochondrial antibody, and abdominal ultrasound to assess for biliary obstruction. The combination of cholestatic alkaline phosphatase and/or gamma-glutamyl transferase elevation with a PBC-specific autoantibody is sufficient for diagnosis in most patients and usually obviates biopsy. [13][17]", "Do not infer disease severity from symptom burden. Pruritus and fatigue may substantially impair quality of life but do not correlate with disease stage. [9][10]", "When autoimmune hepatitis overlap is suspected, liver biopsy with expert clinicopathologic review is recommended by the BSG/UK-PBC guideline because true overlap appears uncommon and histology guides management. [9]" ], "bullets": [ "Obtain abdominal ultrasound during initial evaluation to evaluate obstruction rather than attributing all cholestasis to PBC. [17]", "Assess and document pruritus and fatigue at diagnosis and longitudinally, separately from biochemical risk. [9]", "Refer patients with advanced disease, clinically significant events, uncertain diagnosis, or suspected overlap for hepatology-level assessment. [10]" ], "subsections": [], "table": { "caption": "Diagnostic decisions in suspected PBC. [9][13][17]", "columns": [ "Clinical situation", "Action", "Interpretation or next step" ], "rows": [ [ "Cholestatic liver tests", "Order antimitochondrial antibody and abdominal ultrasound. [13][17]", "Cholestatic tests plus a PBC-specific autoantibody establish PBC in most patients. [13]" ], [ "Imaging suggests obstruction", "Evaluate the obstructive process rather than assuming isolated PBC. [17]", "Biliary obstruction is an essential alternative explanation for cholestasis. [17]" ], [ "Suspected autoimmune hepatitis overlap", "Obtain liver biopsy with expert clinicopathologic review. [9]", "Biopsy helps establish overlap and guide treatment. [9]" ] ] } }, { "id": "risk-stratification", "eyebrow": "Prognosis", "heading": "Use biochemical response and fibrosis assessment to identify residual risk", "intro": "Risk assessment should be repeated rather than limited to the diagnostic visit.", "paragraphs": [ "PBC has heterogeneous progression: some patients progress slowly enough that disease may have limited clinical consequence, whereas others develop progressive biliary injury, fibrosis, cirrhosis, and end-stage liver disease. [13][10] Response to UDCA and longitudinal cholestatic biochemistry are central to risk stratification. [10][22]", "ALP normalization is an important treatment objective. Patients with an insufficient UDCA response, including those whose results remain in an historically acceptable range but are not normalized, have higher risk of liver transplantation or death than those with normalized ALP. [18] A Global PBC Study Group analysis cited a bilirubin threshold of 0.6 times the upper limit of normal as necessary to detect risk discrimination. [23]", "The BSG/UK-PBC guidance recommends annual serum liver tests and repeat documented risk assessment every 3 years. [21] In U.S. practice, monitoring frequency should also reflect treatment changes, biochemical activity, fibrosis stage, and decompensation risk." ], "bullets": [ "Track ALP and bilirubin longitudinally rather than treating a single value as definitive risk classification. [18][23]", "Reassess risk after an adequate UDCA trial and periodically thereafter; annual liver tests and a documented risk reassessment every 3 years are supported by BSG/UK-PBC guidance. [21]", "Evaluate for cirrhosis and portal-hypertension features before selecting obeticholic acid. [5][6]" ], "subsections": [], "table": { "caption": "Risk-stratification findings that change management. [5][18][21][23]", "columns": [ "Finding", "Clinical implication", "Management consequence" ], "rows": [ [ "ALP not normalized on therapy", "Residual transplant or mortality risk remains higher than with normalized ALP. [18]", "Confirm adherence, reassess disease activity and fibrosis, and consider approved escalation when UDCA response is inadequate. [2][18]" ], [ "Bilirubin approaching or above 0.6 times ULN", "May identify increased risk in Global PBC Study Group analyses. [23]", "Prompt closer clinical assessment and risk reassessment. [21][23]" ], [ "Current or prior decompensation or portal hypertension", "Meets FDA definition of advanced cirrhosis for obeticholic acid restriction. [5]", "Do not use obeticholic acid. [5][6]" ] ] } }, { "id": "disease-modifying-treatment", "eyebrow": "Pharmacotherapy", "heading": "Start UDCA, then escalate inadequate biochemical response or intolerance", "intro": "Disease-modifying therapy and symptom control are related but distinct treatment tracks.", "paragraphs": [ "UDCA is first-line therapy and slows disease progression. [13] Contemporary treatment pathways use UDCA for a minimum of 12 months before formal biochemical response assessment. [19] The supplied evidence does not provide a source-supported U.S. dose; use current prescribing information and hepatology guidance when prescribing.", "For adults with inadequate response to UDCA, elafibranor is FDA-approved in combination with UDCA; it is also approved as monotherapy when UDCA cannot be tolerated. Its 2024 approval is accelerated and based on ALP reduction; improvement in survival or prevention of liver decompensation has not been demonstrated. [2]", "Obeticholic acid improved ALP in the pivotal placebo-controlled trial and received accelerated approval based on biochemical improvement. [4][12] However, FDA safety actions substantially constrain selection: it is contraindicated in advanced cirrhosis, including cirrhosis with current or prior decompensation or portal-hypertension features such as ascites, gastroesophageal varices, or persistent thrombocytopenia. [5] FDA also reports serious liver injury in treated patients without cirrhosis, reinforcing the need for clinical and laboratory monitoring. [4]" ], "bullets": [ "Begin UDCA after diagnosis unless contraindicated or not tolerated; assess biochemical response after at least 12 months. [13][19]", "For inadequate UDCA response or UDCA intolerance, elafibranor is an FDA-approved option; counsel that its approval is based on ALP reduction and that clinical-outcome benefit remains unproven. [2]", "Before obeticholic acid, determine whether advanced cirrhosis is present; it is contraindicated if there is current or prior decompensation or portal hypertension. [5][6]", "During obeticholic acid therapy, monitor for disease progression and liver-related adverse reactions; permanently discontinue for progression to advanced cirrhosis or clinically significant liver-related adverse reactions. [6]" ], "subsections": [ { "heading": "Second-line selection and safety", "paragraphs": [ "Elafibranor and obeticholic acid have different regulatory and safety considerations. Elafibranor is approved under accelerated approval for inadequate UDCA response or UDCA intolerance, whereas obeticholic acid requires particular caution because of serious liver injury and its contraindication in advanced cirrhosis. [2][5][6]" ], "bullets": [ "Do not equate ALP reduction with demonstrated survival or decompensation prevention for elafibranor; the FDA label explicitly states these outcomes have not been demonstrated. [2]", "Obeticholic acid exposure rises several-fold in moderate and severe hepatic impairment in the FDA review, supporting dose adjustment and close monitoring in hepatic impairment; use current labeling for exact dosing. [1]" ] } ], "table": { "caption": "Disease-modifying therapies supported by supplied sources. [1][2][4][5][6][12][13][19]", "columns": [ "Therapy", "Role", "Evidence and regulatory status", "Key safety or monitoring issue" ], "rows": [ [ "Ursodeoxycholic acid", "First-line disease-modifying therapy. [13]", "Slows progression; treatment pathways assess response after at least 12 months. [13][19]", "Follow cholestatic tests and biochemical response longitudinally. [18][21]" ], [ "Elafibranor", "With UDCA for inadequate UDCA response; monotherapy if UDCA is not tolerated. [2]", "FDA accelerated approval in 2024 based on ALP reduction. [2]", "Survival benefit and prevention of decompensation have not been demonstrated. [2]" ], [ "Obeticholic acid", "Biochemical second-line option in appropriately selected patients. [4][12]", "Accelerated approval was supported by ALP improvement. [4]", "Contraindicated in advanced cirrhosis; monitor for progression and liver-related adverse reactions. [5][6]" ] ] } }, { "id": "symptoms-and-complications", "eyebrow": "Longitudinal care", "heading": "Treat symptoms independently of biochemical disease control", "intro": "Symptom burden requires active management even when fibrosis risk appears low.", "paragraphs": [ "Pruritus, fatigue, sicca symptoms, abdominal discomfort, and arthralgias or bone pain are part of the PBC morbidity burden. [10] Disease-modifying therapies and symptom-directed therapies should not be assumed to have the same effects: available review evidence notes that treatments modifying progression often do not improve symptoms, and symptom therapies do not substitute for biochemical disease control. [13]", "Linerixibat received FDA orphan-product listing approval for cholestatic pruritus associated with PBC in adults, with an approval date of March 17, 2026 in the supplied FDA listing. [3] Because the supplied source does not include prescribing information, dosing, contraindications, drug interactions, and monitoring parameters cannot be specified here; verify the current FDA label before use." ], "bullets": [ "At each follow-up, ask specifically about itch and fatigue; their severity does not stage PBC. [9][10]", "Address quality-of-life symptoms alongside biochemical monitoring rather than waiting for advanced fibrosis. [13]", "For linerixibat, verify current U.S. labeling before prescribing because the supplied source supports indication and approval status but not use details. [3]" ], "subsections": [], "table": { "caption": "Symptom-focused care principles in PBC. [3][9][10][13]", "columns": [ "Issue", "Clinical interpretation", "Action" ], "rows": [ [ "Pruritus", "Can be clinically important regardless of disease stage. [9][10]", "Assess routinely and use symptom-directed therapy; linerixibat is FDA-listed for adult cholestatic pruritus associated with PBC. [3]" ], [ "Fatigue", "May markedly reduce quality of life but does not correlate with PBC stage. [9][10]", "Document burden and evaluate/manage contributors separately from biochemical risk. [9][13]" ], [ "Biochemical improvement without symptom relief", "Disease-modifying and symptom-directed treatment effects may diverge. [13]", "Continue risk-based therapy while adding symptom-focused management. [13]" ] ] } }, { "id": "follow-up", "eyebrow": "Monitoring", "heading": "Build follow-up around treatment response, fibrosis risk, and drug safety", "intro": "Longitudinal care should detect biochemical nonresponse, clinical progression, and therapy-specific harm.", "paragraphs": [ "Use serial liver tests to establish trajectory after initiating or changing therapy. BSG/UK-PBC guidance recommends annual serum liver tests and documented repeat risk assessment every 3 years; more frequent evaluation is reasonable when biochemical activity persists, treatment is adjusted, or cirrhosis is present. [21]", "For patients receiving obeticholic acid, FDA directs clinicians to monitor routinely for PBC progression using laboratory and clinical assessments and to assess for acute-on-chronic liver disease manifestations, including nausea, vomiting, diarrhea, jaundice, scleral icterus, or dark urine. Permanently discontinue for progression to advanced cirrhosis or development of these clinically significant liver-related adverse reactions. [6]" ], "bullets": [ "Review ALP and bilirubin trends after the initial UDCA assessment period and after any treatment escalation. [18][19][23]", "Reevaluate for portal-hypertension or decompensation features before and during obeticholic acid exposure. [5][6]", "Use clinical symptoms to identify quality-of-life needs, not as a surrogate for fibrosis stage. [9][10]" ], "subsections": [], "table": { "caption": "Practical follow-up framework supported by available sources. [5][6][19][21]", "columns": [ "Time point", "Assess", "Action triggered" ], "rows": [ [ "After at least 12 months of UDCA", "Biochemical response. [19]", "If response is inadequate, reassess risk and consider an approved second-line option. [2][18]" ], [ "At least annually", "Serum liver tests. [21]", "Use trends to identify persistent disease activity or progression. [18][21]" ], [ "Every 3 years", "Documented repeat risk assessment. [21]", "Update prognosis and follow-up intensity. [21]" ], [ "During obeticholic acid therapy", "Clinical and laboratory evidence of progression or liver-related adverse reactions. [6]", "Permanently discontinue for advanced-cirrhosis progression or clinically significant liver-related adverse reactions. [6]" ] ] } } ], "faq": [ { "question": "Is liver biopsy required to diagnose primary biliary cholangitis?", "answer": "Usually not. Cholestatic liver tests plus a PBC-specific autoantibody, typically antimitochondrial antibody, are sufficient in most patients. Biopsy is most useful when autoimmune hepatitis overlap is suspected or the presentation remains diagnostically discordant. [9][13]" }, { "question": "When should response to UDCA be assessed?", "answer": "Current step-up treatment pathways assess biochemical response after a minimum of 12 months of UDCA. Persistent biochemical activity or failure to normalize ALP should prompt repeat risk assessment and consideration of escalation when appropriate. [18][19]" }, { "question": "Can obeticholic acid be used in cirrhosis?", "answer": "It must not be used in advanced cirrhosis. FDA defines advanced cirrhosis as cirrhosis with current or prior decompensation or portal-hypertension features, including ascites, gastroesophageal varices, or persistent thrombocytopenia. [5][6]" }, { "question": "Does biochemical improvement reliably improve pruritus or fatigue?", "answer": "No. Disease-modifying therapies may not materially improve symptoms, while symptom-directed therapies do not replace biochemical risk reduction. Assess and manage pruritus and fatigue independently. [9][13]" } ], "references": [ { "number": 1, "title": "207999Orig1s000 - accessdata.fda.gov", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/nda/2016/207999Orig1s000CrossR.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov" }, { "number": 2, "title": "highlights of prescribing information", "detail": "www.fda.gov", "url": "https://www.fda.gov/media/180873/download", "authors": "www.fda.gov", "host": "www.fda.gov" }, { "number": 3, "title": "Search Orphan Drug Designations and Approvals", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/scripts/opdlisting/oopd/detailedIndex.cfm?cfgridkey=701919", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov" }, { "number": 4, "title": "Serious liver injury being observed in patients without cirrhosis taking Ocaliva (obeticholic acid) to treat primary biliary cholangitis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/drug-safety-communications/serious-liver-injury-being-observed-patients-without-cirrhosis-taking-ocaliva-obeticholic-acid-treat", "authors": "www.fda.gov", "host": "www.fda.gov" }, { "number": 5, "title": "Due to risk of serious liver injury, FDA restricts use of Ocaliva (obeticholic acid) in primary biliary cholangitis (PBC) patients with advanced cirrhosis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/drug-safety-and-availability/due-risk-serious-liver-injury-fda-restricts-use-ocaliva-obeticholic-acid-primary-biliary-cholangitis", "authors": "www.fda.gov", "host": "www.fda.gov" }, { "number": 6, "title": "Due to risk of serious liver injury, FDA restricts use of obeticholic acid (Ocaliva) in primary biliary cholangitis (PBC) patients with advanced cirrhosis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/fda-drug-safety-podcasts/due-risk-serious-liver-injury-fda-restricts-use-obeticholic-acid-ocaliva-primary-biliary-cholangitis", "authors": "www.fda.gov", "host": "www.fda.gov" }, { "number": 7, "title": "This label may not be the latest approved by FDA. For current ...", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/050708s050%2C050709s042%2C210115s002lbl.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov" }, { "number": 8, "title": "prescribing information - accessdata.fda.gov", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/label/2022/020702Orig1s079correctedlbl.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov" }, { "number": 9, "title": "PBC primary biliary cholangitis treatment and management ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/gutjnl/early/2018/03/28/gutjnl-2017-315259.full.pdf", "authors": "gut.bmj.com", "host": "gut.bmj.com" }, { "number": 10, "title": "The British Society of Gastroenterology/UK-PBC primary ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/67/9/1568", "authors": "gut.bmj.com", "host": "gut.bmj.com" }, { "number": 11, "title": "British Society of Gastroenterology and UK-PSC guidelines ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/68/8/1356", "authors": "gut.bmj.com", "host": "gut.bmj.com" }, { "number": 12, "title": "A Placebo-Controlled Trial of Obeticholic Acid in Primary ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa1509840", "authors": "www.nejm.org", "host": "www.nejm.org" }, { "number": 13, "title": "Primary biliary cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/344", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com" }, { "number": 14, "title": "Primary sclerosing cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice US", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/847", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com" }, { "number": 15, "title": "P187 Are we identifying and treating PBC appropriately? 5 ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/70/Suppl_1/A140", "authors": "gut.bmj.com", "host": "gut.bmj.com" }, { "number": 16, "title": "A change of paradigm in PBC: Pursuing normal alkaline ...", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/ebiom/article/PIIS2352-3964(22)00331-0/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com" }, { "number": 17, "title": "Primary biliary cholangitis", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(24)01303-5/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com" }, { "number": 18, "title": "Challenges facing the PBC community: insights from the PBC International Summit | Frontline Gastroenterology", "detail": "fg.bmj.com", "url": "https://fg.bmj.com/content/early/2025/11/26/flgastro-2025-103262", "authors": "fg.bmj.com", "host": "fg.bmj.com" }, { "number": 19, "title": "Optimising Primary thErapy in pRimAry biliary cholangitis ...", "detail": "bmjopen.bmj.com", "url": "https://bmjopen.bmj.com/content/16/3/e113812", "authors": "bmjopen.bmj.com", "host": "bmjopen.bmj.com" }, { "number": 20, "title": "Acute cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice US", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/345", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com" }, { "number": 21, "title": "British Society of Gastroenterology/UK-PBC Primary Biliary ...", "detail": "fg.bmj.com", "url": "https://fg.bmj.com/content/10/3/316", "authors": "fg.bmj.com", "host": "fg.bmj.com" }, { "number": 22, "title": "development and validation of the UDCA Response Score", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/langas/article/PIIS2468-1253(18)30163-8/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com" }, { "number": 23, "title": "The relationship between disease activity and UDCA ...", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/ebiom/article/PIIS2352-3964(22)00249-3/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com" }, { "number": 24, "title": "Assessment of adherence to guidelines for biochemical ...", "detail": "bmjopengastro.bmj.com", "url": "https://bmjopengastro.bmj.com/content/12/1/e001899", "authors": "bmjopengastro.bmj.com", "host": "bmjopengastro.bmj.com" } ], "editorialNote": "Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.", "citations": [ { "number": 1, "title": "207999Orig1s000 - accessdata.fda.gov", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/nda/2016/207999Orig1s000CrossR.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov", "snippet": "The clinical studies conducted in patients with PBC consist of two phase 2 and one pivotal phase 3 studies. The phase 2 studies evaluated dosing of 10, 25 and 50 mg QD dosing. The phase 3 study evaluated 10 mg QD and a titration arm (5 mg QD for 6 months followed by up-titration to 10 mg QD based on", "score": 0.6453216 }, { "number": 2, "title": "highlights of prescribing information", "detail": "www.fda.gov", "url": "https://www.fda.gov/media/180873/download", "authors": "www.fda.gov", "host": "www.fda.gov", "snippet": "INFORMATION Sections or subsections omitted from the full prescribing information are not listed. Reference ID: 5395349 FULL PRESCRIBING INFORMATION 1 INDICATIONS AND USAGE IQIRVO is indicated for the treatment of primary biliary cholangitis (PBC) in combination with ursodeoxycholic acid (UDCA) in ", "score": 0.61168414 }, { "number": 3, "title": "Search Orphan Drug Designations and Approvals", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/scripts/opdlisting/oopd/detailedIndex.cfm?cfgridkey=701919", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov", "snippet": "Decrease font size\nReturn font size to normal\nIncrease font size\nU.S. Department of Health and Human Services\nFDA, U.S. Food and Drug Administration\nSearch\n\n# Search Orphan Drug Designations and Approvals\n\nPrint\nShare\nE-mail\n\n| | |\n --- |\n| Generic Name: | linerixibat |\n| Trade Name: | Lynavoy |\n|", "score": 0.5775198 }, { "number": 4, "title": "Serious liver injury being observed in patients without cirrhosis taking Ocaliva (obeticholic acid) to treat primary biliary cholangitis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/drug-safety-communications/serious-liver-injury-being-observed-patients-without-cirrhosis-taking-ocaliva-obeticholic-acid-treat", "authors": "www.fda.gov", "host": "www.fda.gov", "snippet": "Ocaliva is a prescription medicine approved in May 2016 that has been shown to improve a certain liver test called alkaline phosphatase (ALP) in patients with PBC who have not responded well enough to another medicine called ursodeoxycholic acid (UDCA). The original clinical trial showed a decrease ", "score": 0.5009527 }, { "number": 5, "title": "Due to risk of serious liver injury, FDA restricts use of Ocaliva (obeticholic acid) in primary biliary cholangitis (PBC) patients with advanced cirrhosis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/drug-safety-and-availability/due-risk-serious-liver-injury-fda-restricts-use-ocaliva-obeticholic-acid-primary-biliary-cholangitis", "authors": "www.fda.gov", "host": "www.fda.gov", "snippet": "## References\n\n## Related Information\n\n## Contact FDA\n\nFor More Info \n855-543-DRUG (3784) and press 4 \ndruginfo@fda.hhs.gov\n\nReport a Serious Problem to MedWatch \nComplete and submit the report Online. \nDownload form\") or call 1-800-332-1088 to request a reporting form, then complete and return ", "score": 0.4993414 }, { "number": 6, "title": "Due to risk of serious liver injury, FDA restricts use of obeticholic acid (Ocaliva) in primary biliary cholangitis (PBC) patients with advanced cirrhosis | FDA", "detail": "www.fda.gov", "url": "https://www.fda.gov/drugs/fda-drug-safety-podcasts/due-risk-serious-liver-injury-fda-restricts-use-obeticholic-acid-ocaliva-primary-biliary-cholangitis", "authors": "www.fda.gov", "host": "www.fda.gov", "snippet": "Based on the original clinical trials, FDA believes the benefits of Ocaliva outweigh the risks for PBC patients who do not have advanced cirrhosis. We will continue to monitor and evaluate the clinical benefit and adverse events of Ocaliva and will communicate any new information to the public if it", "score": 0.49275014 }, { "number": 7, "title": "This label may not be the latest approved by FDA. For current ...", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/050708s050%2C050709s042%2C210115s002lbl.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov", "snippet": "oral formulations (capsules or suspension). (2.1, 2.2) • Administer capsules or suspension consistently with or without food. (2.1) • Therapeutic drug monitoring is recommended. (2.1, 2.6) • Avoid eating grapefruit or drinking grapefruit juice. (2.1) • See dosing adjustments for African-American pat", "score": 0.462061 }, { "number": 8, "title": "prescribing information - accessdata.fda.gov", "detail": "www.accessdata.fda.gov", "url": "https://www.accessdata.fda.gov/drugsatfda_docs/label/2022/020702Orig1s079correctedlbl.pdf", "authors": "www.accessdata.fda.gov", "host": "www.accessdata.fda.gov", "snippet": "colchicine, and ledipasvir plus sofosbuvir. Consider if the benefit of use of these products outweighs the increased risk of myopathy and rhabdomyolysis [see Drug Interactions (7.1)]. Concomitant intake of large quantities, more than 1.2 liters daily, of grapefruit juice is not recommended in patien", "score": 0.3542772 }, { "number": 9, "title": "PBC primary biliary cholangitis treatment and management ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/gutjnl/early/2018/03/28/gutjnl-2017-315259.full.pdf", "authors": "gut.bmj.com", "host": "gut.bmj.com", "snippet": "Guideline development process These guidelines are designed primarily with the hospital physi-cian in mind. They nevertheless underpin the management of PBC across all specialities and between primary and hospital care. The guidelines have been produced as a consensus docu-ment of the BSG Liver Sect", "score": 0.7707586 }, { "number": 10, "title": "The British Society of Gastroenterology/UK-PBC primary ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/67/9/1568", "authors": "gut.bmj.com", "host": "gut.bmj.com", "snippet": "Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC), is a life-long autoimmune cholestatic liver disease that is a rare but important cause of chronic liver disease. More than 15 000 individuals in the UK live with the risks and consequences of chronic biliary inflammation", "score": 0.531672 }, { "number": 11, "title": "British Society of Gastroenterology and UK-PSC guidelines ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/68/8/1356", "authors": "gut.bmj.com", "host": "gut.bmj.com", "snippet": "Similar to clinical outcomes in primary biliary cholangitis (PBC), recent data from retrospective studies support the use of falling ALP (normalisation or <1.5 x upper limit of normal (ULN)) as a stratifier for improved outcome in patients with PSC, independent of the therapeutic modality used23–25 ", "score": 0.30090013 }, { "number": 12, "title": "A Placebo-Controlled Trial of Obeticholic Acid in Primary ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa1509840", "authors": "www.nejm.org", "host": "www.nejm.org", "snippet": "by F Nevens · 2016 · Cited by 1466 — The only approved treatment for primary biliary cholangitis was ursodiol, which decreases liver biochemical values and delays the time to liver", "score": 0.22369951 }, { "number": 13, "title": "Primary biliary cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/344", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com", "snippet": "open menu\n\nWhen viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.\n\n# Primary biliary cholangitis\n\nEpidemiology\n\nAetiology\n\nCase history\n\nApproach\n\nHistory and exam\n\nInvestigation", "score": 0.5752307 }, { "number": 14, "title": "Primary sclerosing cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice US", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/847", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com", "snippet": "open menu\n\nAo visualizar este tópico em um idioma diferente, você poderá notar algumas diferenças na forma como o conteúdo é estruturado, mas ele ainda refletirá as orientações baseadas em evidências mais recentes.\n\n# Primary sclerosing cholangitis\n\nEpidemiology\n\nEtiology\n\nCase history\n\nApproach\n\nHi", "score": 0.39089483 }, { "number": 15, "title": "P187 Are we identifying and treating PBC appropriately? 5 ...", "detail": "gut.bmj.com", "url": "https://gut.bmj.com/content/70/Suppl_1/A140", "authors": "gut.bmj.com", "host": "gut.bmj.com", "snippet": "25/29 (86%) of PBC patients were treated with UDCA, which was adequately dosed in 23/25 (92%). 15/19 (79%) of patients who had completed at least one year of", "score": 0.3539422 }, { "number": 16, "title": "A change of paradigm in PBC: Pursuing normal alkaline ...", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/ebiom/article/PIIS2352-3964(22)00331-0/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com", "snippet": "by A Lleo · 2022 · Cited by 5 — Primary biliary cholangitis (PBC) is an autoimmune liver disease characterised by a chronic and destructive lymphocytic cholangitis of the small", "score": 0.33196434 }, { "number": 17, "title": "Primary biliary cholangitis", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(24)01303-5/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com", "snippet": "by A Tanaka · 2024 · Cited by 203 — A thorough history and physical examination, serological test, and abdominal ultrasound to assess for biliary obstruction are essential.", "score": 0.33056894 }, { "number": 18, "title": "Challenges facing the PBC community: insights from the PBC International Summit | Frontline Gastroenterology", "detail": "fg.bmj.com", "url": "https://fg.bmj.com/content/early/2025/11/26/flgastro-2025-103262", "authors": "fg.bmj.com", "host": "fg.bmj.com", "snippet": "## \n\n## Introduction\n\nPrimary biliary cholangitis (PBC) is a chronic, progressive autoimmune liver disease, characterised by widespread inflammation and scarring in the liver, accompanied by damage to and loss of the intrahepatic bile ducts.1 2 Over the past decade, there have been significant advan", "score": 0.32075796 }, { "number": 19, "title": "Optimising Primary thErapy in pRimAry biliary cholangitis ...", "detail": "bmjopen.bmj.com", "url": "https://bmjopen.bmj.com/content/16/3/e113812", "authors": "bmjopen.bmj.com", "host": "bmjopen.bmj.com", "snippet": "The current treatment approach is a step-up model, wherein first-line therapy, ursodeoxycholic acid (UDCA), is given for a minimum of 12 months before the", "score": 0.30631998 }, { "number": 20, "title": "Acute cholangitis - Symptoms, diagnosis and treatment | BMJ Best Practice US", "detail": "bestpractice.bmj.com", "url": "https://bestpractice.bmj.com/topics/en-us/345", "authors": "bestpractice.bmj.com", "host": "bestpractice.bmj.com", "snippet": "open menu\n\n# Acute cholangitis\n\nEpidemiology\n\nEtiology\n\nCase history\n\nRecommendations\n\nHistory and exam\n\nTests\n\nDifferentials\n\nCriteria\n\nRecommendations\n\nTreatment algorithm\n\nEmerging\n\nPrevention\n\nPatient discussions\n\nMonitoring\n\nComplications\n\nPrognosis\n\nGuidelines\n\nImages and videos\n\nReferences\n\nP", "score": 0.2871432 }, { "number": 21, "title": "British Society of Gastroenterology/UK-PBC Primary Biliary ...", "detail": "fg.bmj.com", "url": "https://fg.bmj.com/content/10/3/316", "authors": "fg.bmj.com", "host": "fg.bmj.com", "snippet": "by JC Goet · 2019 · Cited by 16 — The guidelines recommend annual serum liver tests and documented repeat risk assessment every 3 years. PBC is a lifelong disease, with a slow natural history", "score": 0.28175429 }, { "number": 22, "title": "development and validation of the UDCA Response Score", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/langas/article/PIIS2468-1253(18)30163-8/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com", "snippet": "by M Carbone · 2018 · Cited by 189 — Treatment guidelines recommend a stepwise approach to primary biliary cholangitis: all patients begin treatment with ursodeoxycholic acid", "score": 0.2229373 }, { "number": 23, "title": "The relationship between disease activity and UDCA ...", "detail": "www.thelancet.com", "url": "https://www.thelancet.com/journals/ebiom/article/PIIS2352-3964(22)00249-3/fulltext", "authors": "www.thelancet.com", "host": "www.thelancet.com", "snippet": "by DEJ Jones · 2022 · Cited by 26 — The Global PBC Study Group suggested that using a threshold for bilirubin values of 0.6 times the upper limit of normal was necessary to see", "score": 0.10787861 }, { "number": 24, "title": "Assessment of adherence to guidelines for biochemical ...", "detail": "bmjopengastro.bmj.com", "url": "https://bmjopengastro.bmj.com/content/12/1/e001899", "authors": "bmjopengastro.bmj.com", "host": "bmjopengastro.bmj.com", "snippet": "by SC Gordon · 2025 · Cited by 2 — patients with primary biliary cholangitis (PBC) to monitor disease progression … routine monitoring every 6–9 months thereafter.", "score": 0.6554468 } ], "publishedAt": "2026-08-21T00:30:01.460998+00:00", "updatedAt": "2026-08-21T00:30:01.460998+00:00", "readingMinutes": 5, "slug": "primary-biliary-cholangitis" }