Primary Biliary Cholangitis with Associated Raynaud Syndrome Treatment Outcome: a Case Report
Ainur Doszhan 1 * ,
Nina Tostanovskaya 1,
Galiya Shaimardanova 1,
Larissa Kozina 1,
Gulsara Imambayeva 2 More Detail
1 Therapy department, National Scientific Medical Center, Astana, Kazakhstan
2 Astana Medical University, Astana, Kazakhstan
* Corresponding Author
J CLIN MED KAZ, Volume 22, Issue 5, pp. 119-122.
https://doi.org/10.23950/jcmk/16882
OPEN ACCESS
279 Views
52 Downloads
Author Contributions: Conceptualization, A. D., N. T., G. S., L. K., patient data collection and analysis wrote the manuscript. G. S. performed morphology analysis. G. I. reviewed the manuscript.
Data availability statement: The data that support the findings of this study are not publicly available due to privacy reasons, but are available from the Corresponding Author upon reasonable request.
ABSTRACT
Primary biliary cholangitis is an autoimmune liver disease, which is less common than viral hepatitis and fatty liver disease, but in recent decades there has been a steady increase in the PBC occurrence. The development of severe complications, many concomitant pathologies, and frequent lack of response to basic ursodeoxycholic acid (UDCA) therapy requires timely dynamic monitoring of patients with PBC for effective treatment correction.
Here we describe a case presentation of liver cirrhosis development in patient with PBC combined with Raynaud syndrome. Upon disease development, the patient refused to undergo liver biopsy. She was treated with non-conventional treatment regime. Our clinical case showed that the use of budesonide in PBC treatment did not show any promising result and led to rapid liver cirrhosis development.
CITATION
Doszhan A, Tostanovskaya N, Shaimardanova G, Kozina L, Imambayeva G. Primary Biliary Cholangitis with Associated Raynaud Syndrome Treatment Outcome: a Case Report. J CLIN MED KAZ. 2025;22(5):119-22.
https://doi.org/10.23950/jcmk/16882
REFERENCES
- Reuben A. The serology of the Addison-Gull syndrome. Hepatology. 2003; 37(1): 225–228.
- Trivedi PJ, Hirschfield GM. Recent advances in clinical practice: epidemiology of autoimmune liver diseases. Gut. 2021; 70(10): 1989-2003.
- Lv T, Chen S, Li M, Zhang D, Kong Y, Jia J. Regional variation and temporal trend of primary biliary cholangitis epidemiology: A systematic review and meta-analysis. Journal of gastroenterology and hepatology. 2021; 36(6): 1423–1434.
- Lee YM, Kaplan MM. The natural history of PBC: has it changed? Semin Liver Dis. 2005; 25(3): 321–326.
- Gershwin ME, Mackay IR. The causes of primary biliary cirrhosis: Convenient and inconvenient truths. Hepatology. 2008;47(2):737–745.
- Hirschfield GM, Dyson JK, Alexander GJM, Chapman MH., Collier J., Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DE J. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018;67(9): 1568–1594.
- Gulamhusein AF, Hirschfield GM. Primary biliary cholangitis: pathogenesis and therapeutic opportunities. Nature reviews. Gastroenterology & hepatology. 2020; 17(2): 93–110.
- Shimoda S, Tanaka A. It is time to change primary biliary cirrhosis (PBC): New nomenclature from "cirrhosis" to "cholangitis", and upcoming treatment based on unveiling pathology. Hepatol Res. 2016; 46(5): 407–415
- Tanaka A, Takikawa H, Mochida S, Koike K, Miwa H, Shimosegawa T. Changing Nomenclature for PBC from "Primary Biliary Cirrhosis" to "Primary Biliary Cholangitis". J Gastroenterol. 2016; 51(7): 748–749.
- Zeng N, Duan W, Chen S, Wu S, Ma H, Ou X, You H, Kong Y, Jia J. Epidemiology and clinical course of primary biliary cholangitis in the Asia-Pacific region: a systematic review and meta-analysis. Hepatology international. 2019; 13(6): 788–799.
- Prince MI, James OF. The epidemiology of primary biliary cirrhosis. Clinics in liver disease. 2003; 7(4): 795–819.
- Hirschfield GM, Gershwin ME. The immunobiology and pathophysiology of primary biliary cirrhosis. Annual review of pathology. 2013; 8: 303–330.
- Hirschfield GM., Dyson JK, Alexander GJM, Chapman MH, Collier J, Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones D. EJ. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018; 67(9): 1568–1594.
- Abbas G, Lindor KD. Pharmacological treatment of biliary cirrhosis with ursodeoxycholic acid. Expert opinion on pharmacotherapy. 2010; 11(3): 387–392.
- Trivella J, John BV, Levy C. Primary biliary cholangitis: Epidemiology, prognosis, and treatment. Hepatology communications. 2023; 7(6): e0179.
- Gershwin ME, Ansari AA, Mackay IR, Nakanuma Y, Nishio A, Rowley MJ, Coppel RL. Primary biliary cirrhosis: an orchestrated immune response against epithelial cells. Immunological reviews. 2000; 174: 210–225.
- Patel N, Amarapurkar D, Amarapurkar A. Primary biliary cirrhosis and autoimmune hepatitis switch over: report of 2 cases. Tropical gastroenterology: official journal of the Digestive Diseases Foundation. 2005; 26(3): 136–138.
- Floreani A, Cazzagon N. PBC and related extrahepatic diseases. Best Practice & Research Clinical Gastroenterology. 2018; 34–35: 49–54.
- Murray Lyon IM, Thompson RP, Ansell ID, Williams R. Sclerodermaandprimarybiliarycirrhosis. Br. Med. J. 2005; 3: 258–259(1970).
- Granito A, Muratori P, Muratori L, Pappas G, Cassani F, Worthington J, Ferri S, Quarneti C, Cipriano V, de Molo C, Lenzi M, Chapman RW, Bianchi FB. Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis. Alimentary pharmacology & therapeutics. 2007; 26(6): 831–838.
- Abe N, Takahata Y, Fujita M, Takahashi A, Migita K, Ohira H. Long-term outcomes in patients with primary biliary cholangitis complicated with CREST syndrome. Sci Rep. 2024; 14, 14124.
- Huang C, Fan X, Yang L. Concomitant Sjögren's Syndrome Was Not Associated with a Poorer Response or Outcomes in Ursodeoxycholic Acid-Treated Patients with Primary Biliary Cholangitis. Canadian journal of gastroenterology & hepatology. 2019; 7396870.
- Gonzalez RS, Washington K. Primary Biliary Cholangitis and Autoimmune Hepatitis. Surgical pathology clinics. 2018; 11(2): 329–349.
- Onofrio, Fernanda Q et al. A Practical Review of Primary Biliary Cholangitis for the Gastroenterologist. Gastroenterology & hepatologyvol. 2019; 15 (3): 145–154.
- Ali AH, Lindor KD. Obeticholic acid for the treatment of primary biliary cholangitis. Expert opinion on pharmacotherapy. 2016; 17(13): 1809–1815.
- Zhang H, Yang J, Zhu R, Zheng Y, Zhou Y, Dai W, Wang F, Chen, K, Li J, Wang C, Li S, Liu T, Abudumijiti H, Zhou Z, Wang J, Lu W, Wang J, Xia Y, Zhou Y, Lu J, … Guo C. Combination therapy of ursodeoxycholic acid and budesonide for PBC-AIH overlap syndrome: a meta-analysis. Drug design, development and therapy. 2015; 9: 567–574.
- O'Donnell S, O'Morain CA. Therapeutic benefits of budesonide in gastroenterology. Therapeutic advances in chronic disease. 2010; 1(4): 177–186.
- Corpechot C, Carrat F, Bahr A, Chrétien Y, Poupon RE, Poupon R. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology. 2005; 128(2): 297–303.
- Bergquist A, Färkkilä M, Manns MP, Parés A, Spengler U, Stiess M, Greinwald R, Pröls M, Wendum D, Drebber U, Poupon R. A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA. Journal of hepatology. 2021; 74(2): 321–329. https://doi.org/10.1016/j.jhep.2020.09.011
- U.S. Food and Drug Administration (FDA). Ocaliva (obeticholic acid): Drug Safety Communication – FDA restricts use in primary biliary cholangitis (PBC) patients with advanced cirrhosis. 2021. Available from: https://www.fda.gov/media/151344/download
- European Medicines Agency (EMA). Ocaliva: EPAR – Product Information. Updated 2022. Available from: https://www.ema.europa.eu/en/medicines/human/EPAR/ocaliva
- Lindor KD, Bowlus CL, Levy C, Mayo MJ, Vincent C, O'Neil M. The safety of obeticholic acid in primary biliary cholangitis: review of clinical data and postmarketing experience. Hepatology. 2021; 74(3): 1220–1230. https://doi.org/10.1002/hep.31806
- Nevens F, Andreone P, Mazzella G, Strasser SI, Bowlus C, Invernizzi P, et al. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis. N Engl J Med. 2016; 375(7): 631–643. https://doi.org/10.1056/NEJMoa1509840
- Hirschfield GM, Mason A, Luketic V, Lindor K, Gordon SC, Mayo M, et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Lancet. 2015; 386(10003): 1103–1111. https://doi.org/10.1016/S0140-6736(15)00150-9