Primary biliary cholangitis

2020 ◽  
pp. 3127-3135
Author(s):  
Jessica K. Dyson ◽  
David E.J. Jones
Keyword(s):  
Alkaline Phosphatase ◽  
Liver Disease ◽  
Serum Alkaline Phosphatase ◽  
Disease Diagnosis ◽  
Immunosuppressive Drug ◽  
Primary Biliary Cholangitis ◽  
Line Treatment ◽  
Antimitochondrial Antibodies ◽  
First Line ◽  
Intrahepatic Bile Ducts

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a chronic, cholestatic liver disease in which the biliary epithelial cells lining the small intrahepatic bile ducts are the target for immune-mediated damage, leading to progressive ductopenia and cholestasis. The cause is unknown but presumed to be autoimmune. The disorder affects women (>90% of cases) and usually has an insidious onset in middle age. Younger patients are less common but have a more aggressive disease course. Fatigue and pruritus are the most common presenting symptoms. Findings on examination vary widely, ranging from no abnormality to jaundice with hyperpigmentation, scratch marks, and rarely the features of advanced liver disease. Diagnosis of PBC is based on three criteria: (1) cholestatic liver function tests, with increases in serum alkaline phosphatase and γ‎-glutamyl transferase, (2) presence of serum antimitochondrial antibodies (found in more than 95% of cases), and (3) compatible liver histology. Many asymptomatic patients are recognized following the incidental discovery of antimitochondrial antibodies or elevated levels of serum alkaline phosphatase. First-line treatment is with ursodeoxycholic acid which can lead to significant improvement in liver biochemical values. Second-line treatment is with obeticholic acid. No immunosuppressive drug regimen has been proven effective. Progression may be slow, but eventually patients can develop cirrhosis. Cholestyramine is used as first line to treat pruritus. There is no recognized treatment for fatigue. Liver transplantation is indicated in some cases.

scholarly journals Immediate hypersensitivity reaction followed by successful oral desensitization to ursodiol

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Erika Yue Lee ◽  
Christine Song
Keyword(s):  
Hypersensitivity Reaction ◽  
Primary Biliary Cholangitis ◽  
Line Treatment ◽  
Second Line ◽  
Immediate Hypersensitivity ◽  
First Line ◽  
Case Presentation ◽  
Desensitization Protocol ◽  
First Line Treatment ◽  
Oral Desensitization

Abstract Background Immediate hypersensitivity reaction to ursodiol is rare and there is no previously published protocol on ursodiol desensitization. Case presentation A 59-year-old woman with primary biliary cholangitis (PBC) developed an immediate hypersensitivity reaction to ursodiol—the first-line treatment for PBC. When she switched to a second-line treatment, her PBC continued to progress. As such, she completed a novel 12-step desensitization protocol to oral ursodiol. She experienced recurrent pruritus after each dose following desensitization, which subsided after a month of being on daily ursodiol. Conclusion Immediate hypersensitivity reaction to ursodiol is uncommon. Our case demonstrated that this novel desensitization protocol to ursodiol could be safely implemented when alternative options are not available or have proven inferior in efficacy.

Serum Gamma-Glutamyl Transpeptidase Activity as an Indicator of Disease of Liver, Pancreas, or Bone

1972 ◽  
Vol 18 (4) ◽  
pp. 358-362 ◽  
Author(s):  
Gifford Lum ◽  
S Raymond Gambino
Keyword(s):  
Alkaline Phosphatase ◽  
Liver Disease ◽  
Viral Hepatitis ◽  
Bone Disease ◽  
Leucine Aminopeptidase ◽  
Serum Alkaline Phosphatase ◽  
Metastatic Carcinoma ◽  
Gamma Glutamyl Transpeptidase ◽  
Serum Alkaline Phosphatase Activity ◽  
Glutamyl Transpeptidase

Abstract Serum γ-glutamyl transpeptidase (GGT), leucine aminopeptidase, alkaline phosphatase, alanine aminotransferase, and aspartate aminotransferase activities were assayed in controls and in patients with liver, pancreatic, or bone disease. GGT activity was above normal in all forms of liver disease studied (viral hepatitis, cirrhosis, cholecystitis, metastatic carcinoma to liver, pancreatic carcinoma, liver granuloma, and acute pancreatitis). GGT more sensitively indicated hepatic disease than did alkaline phosphatase, much more so than did leucine aminopeptidase. GGT was disproportionately more active in relation to the transaminases in cases of intraor extrahepatic biliary obstruction; the reverse was true in cases of viral hepatitis. GGT activity was normal in children, adolescents, and pregnant women, and in cases of bone disease and renal failure. Kinetic measurement of GGT activity offers a simple, sensitive, and direct means for distinguishing whether bone or liver is the source of increased serum alkaline phosphatase activity. Activity was highest in obstructive liver disease.

scholarly journals Practical strategies for pruritus management in the obeticholic acid-treated patient with PBC: proceedings from the 2018 expert panel

2019 ◽  
Vol 6 (1) ◽  
pp. e000256 ◽  
Author(s):  
Jennifer Pate ◽  
Juilo A Gutierrez ◽  
Catherine T Frenette ◽  
Aparna Goel ◽  
Sonal Kumar ◽  
...  
Keyword(s):  
Liver Disease ◽  
Patient Adherence ◽  
Treated Patient ◽  
Management Strategies ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Common Symptom ◽  
Obeticholic Acid ◽  
Intrahepatic Bile Ducts ◽  
Patient Health

Background and aimsThis article provides expert guidance on the management of pruritus symptoms in patients receiving obeticholic acid (OCA) as treatment for primary biliary cholangitis (PBC). PBC is a chronic, autoimmune cholestatic liver disease that affects intrahepatic bile ducts. If not adequately treated, PBC can lead to cholestasis and end-stage liver disease, which may require transplant. Timely treatment is therefore vital to patient health. Pruritus is a common symptom in patients with PBC. Additionally, the use of OCA to treat PBC can contribute to increased pruritus severity in some patients, adding to patient discomfort, decreasing patient quality of life (QoL), and potentially affecting patient adherence to OCA treatment.MethodsIn May 2018, a group of physician experts from the fields of gastroenterology, hepatology, and psychiatry met to discuss the management of pruritus in OCA-treated patients with PBC. Recognizing the importance of optimizing treatment for PBC, these experts developed recommendations for managing pruritus symptoms in the OCA-treated PBC patient based on their experience in clinical practice.ResultsThese recommendations include a comprehensive list of management strategies (including over-the-counter, prescription, and alternative therapies), guidance on titration of OCA to minimize pruritus severity, and an algorithm that outlines a practical approach to follow up with patients receiving OCA, to better assess and manage pruritus symptoms.ConclusionsPruritus associated with OCA therapy is dose dependent and often manageable, and with the proper education and tools, most pruritus cases can be effectively managed to minimize treatment discontinuation.

Alcoholic liver disease presenting with marked elevation of serum alkaline phosphatase

1978 ◽  
Vol 23 (12) ◽  
pp. 1061-1066 ◽  
Author(s):  
Robert P. Perrillo ◽  
Ronald Griffin ◽  
Katherine DeSchryver-Kecskemeti ◽  
Jerrold J. Lander ◽  
Gary R. Zuckerman
Keyword(s):  
Alkaline Phosphatase ◽  
Liver Disease ◽  
Alcoholic Liver Disease ◽  
Serum Alkaline Phosphatase ◽  
Marked Elevation

scholarly journals Immediate Hypersensitivity Reaction Followed by Successful Oral Desensitization to Ursodiol

2021 ◽  
Author(s):  
Erika Yue Lee ◽  
Christine Song
Keyword(s):  
Hypersensitivity Reaction ◽  
Primary Biliary Cholangitis ◽  
Line Treatment ◽  
Second Line ◽  
Immediate Hypersensitivity ◽  
First Line ◽  
Case Presentation ◽  
Desensitization Protocol ◽  
First Line Treatment ◽  
Oral Desensitization

Abstract Background: Immediate hypersensitivity reaction to ursodiol is rare and there is no previously published protocol on ursodiol desensitization. Case presentation: A 59-year-old woman with primary biliary cholangitis (PBC) developed an immediate hypersensitivity reaction to ursodiol – the first-line treatment for PBC. When she switched to a second-line treatment, her PBC continued to progress. As such, she completed a novel 12-step desensitization protocol to oral ursodiol. She experienced recurrent pruritus after each dose following desensitization, which subsided after a month of being on daily ursodiol.Conclusion: Immediate hypersensitivity reaction to ursodiol is uncommon. Our case demonstrated that this novel desensitization protocol to ursodiol could be safely implemented when alternative options are not available or have proven inferior in efficacy.

scholarly journals Primary Biliary Cholangitis and Bile Acid Farnesoid X Receptor Agonists

Diseases ◽  
2020 ◽  
Vol 8 (2) ◽  
pp. 20
Author(s):  
Ludovico Abenavoli ◽  
Anna Caterina Procopio ◽  
Sharmila Fagoonee ◽  
Rinaldo Pellicano ◽  
Marco Carbone ◽  
...  
Keyword(s):  
Farnesoid X Receptor ◽  
Drop Out ◽  
Future Perspective ◽  
Primary Biliary Cholangitis ◽  
First Line ◽  
Obeticholic Acid ◽  
Intrahepatic Bile Ducts ◽  
Protective Actions ◽  
Line Drug

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by the progressive destruction of the intrahepatic bile ducts. Currently, the first line drug for PBC is ursodeoxycholic acid (UDCA) characterized by anti-apoptotic, anti-inflammatory and protective actions on cholangiocytes. Despite its recognized therapeutic action, 30–40% of PBC patients only partially benefit from UDCA therapy. This has led to the identification of the role of the farnesoid x receptor (FXR) in cholestatic liver diseases and, consequently, to the development of obeticholic acid (OCA), a steroid FXR agonist that has been recently approved for the treatment of PBC. OCA though is not effective in all patients and can cause itch, which eventually induces treatment drop out. Therefore, the search for new therapeutic strategies for PBC has begun. This review, in addition to summarizing the current treatments for PBC, provides overview of the chemical characteristics of new steroid FXR agonist candidates that could represent a future perspective for the treatment of PBC.

Primary biliary cholangitis: assessment and management strategies

2021 ◽  
Vol 19 (Sup4) ◽  
pp. S1-S24
Author(s):  
S Nadir Abbas ◽  
David Jones ◽  
Yiannis Kallis ◽  
Laura Maher ◽  
Imran Patanwala
Keyword(s):  
Bile Ducts ◽  
Management Strategies ◽  
Patient Treatment ◽  
Primary Biliary Cholangitis ◽  
Line Treatment ◽  
First Line ◽  
Treatment Pathways ◽  
National Guidance ◽  
First Line Treatment

Primary biliary cholangitis (PBC) is an autoimmune disease resulting in permanent damage to the bile ducts in the liver, often leading to symptoms including fatigue and itch that significantly impair patient quality of life. PBC is often diagnosed in its early stages and can generally be treated effectively with first-line treatment. Patients who do not respond to this should be referred to the hub centre of their local operational delivery network for assessment and prescription of second-line treatment. These patient treatment pathways are based on national guidance. This supplement summarises the key components of the guidance on the assessment, diagnosis and treatment of patients with PBC.

scholarly journals Real-World Clinical Management of Patients with Primary Biliary Cholangitis—A Retrospective Multicenter Study from Germany

2021 ◽  
Vol 10 (5) ◽  
pp. 1061
Author(s):  
Anne-Christin Beatrice Wilde ◽  
Charlotte Lieb ◽  
Elise Leicht ◽  
Lena Maria Greverath ◽  
Lara Marleen Steinhagen ◽  
...  
Keyword(s):  
Multicenter Study ◽  
Response Rates ◽  
Primary Biliary Cholangitis ◽  
Inadequate Response ◽  
Line Treatment ◽  
Second Line ◽  
First Line ◽  
Treatment Modification ◽  
Treatment Regimens ◽  
Retrospective Multicenter Study

Background: Clinical practice guidelines for patients with primary biliary cholangitis (PBC) have been recently revised and implemented for well-established response criteria to standard first-line ursodeoxycholic acid (UDCA) therapy at 12 months after treatment initiation for the early identification of high-risk patients with inadequate treatment responses who may require treatment modification. However, there are only very limited data concerning the real-world clinical management of patients with PBC in Germany. Objective: The aim of this retrospective multicenter study was to evaluate response rates to standard first-line UDCA therapy and subsequent Second-line treatment regimens in a large cohort of well-characterized patients with PBC from 10 independent hepatological referral centers in Germany prior to the introduction of obeticholic acid as a licensed second-line treatment option. Methods: Diagnostic confirmation of PBC, standard first-line UDCA treatment regimens and response rates at 12 months according to Paris-I, Paris-II, and Barcelona criteria, the follow-up cut-off alkaline phosphatase (ALP) ≤ 1.67 x upper limit of normal (ULN) and the normalization of bilirubin (bilirubin ≤ 1 × ULN) were retrospectively examined between June 1986 and March 2017. The management and hitherto applied second-line treatment regimens in patients with an inadequate response to UDCA and subsequent response rates at 12 months were also evaluated. Results: Overall, 480 PBC patients were included in this study. The median UDCA dosage was 13.2 mg UDCA/kg bodyweight (BW)/d. Adequate UDCA treatment response rates according to Paris-I, Paris-II, and Barcelona criteria were observed in 91, 71.3, and 61.3% of patients, respectively. In 83.8% of patients, ALP ≤ 1.67 x ULN were achieved. A total of 116 patients (24.2%) showed an inadequate response to UDCA according to at least one criterion. The diverse second-line treatment regimens applied led to significantly higher response rates according to Paris-II (35 vs. 60%, p = 0.005), Barcelona (13 vs. 34%, p = 0.0005), ALP ≤ 1.67 x ULN and bilirubin ≤ 1 x ULN (52.1 vs. 75%, p = 0.002). The addition of bezafibrates appeared to induce the strongest beneficial effect in this cohort (Paris II: 24 vs. 74%, p = 0.004; Barcelona: 50 vs. 84%, p = 0.046; ALP < 1.67x ULN and bilirubin ≤ 1 × ULN: 33 vs. 86%, p = 0.001). Conclusion: Our large retrospective multicenter study confirms high response rates following UDCA first-line standard treatment in patients with PBC and highlights the need for close monitoring and early treatment modification in high-risk patients with an insufficient response to UDCA since early treatment modification significantly increases subsequent response rates of these patients.

Sign in / Sign up

Export Citation Format

Share Document