Colangitis biliar primaria: caracterización de una cohorte retrospectiva

Autores/as

  • Edgar Fernando Ayala-Sierra Universidad de Antioquia
  • Octavio G. Muñoz-Maya Hospital Pablo Tobón Uribe, Universidad de Antioquia https://orcid.org/0000-0002-5712-2864
  • Felipe Guerrero-Pérez Universidad de Antioquia
  • Manuela Cardona-Jaramillo Universidad de Antioquia

DOI:

https://doi.org/10.52784/27112330.159

Palabras clave:

colangitis biliar primaria, ácido ursodesoxicólico, trasplante hepático.

Resumen

Introducción. La colangitis biliar primaria (CBP) es una enfermedad hepática crónica de origen autoinmune, caracterizada por inflamación y destrucción progresiva de las células epiteliales de los conductos biliares intralobulillares, que causa de manera secundaria colestasis, fibrosis, cirrosis e insuficiencia hepática. La historia natural de la enfermedad ha cambiado en los últimos años debido a la mejoría en los métodos diagnósticos y terapéuticos. Metodología. Estudio observacional descriptivo de cohorte retrospectivo, en el cual se efectuó la revisión y análisis de las historias clínicas de los pacientes mayores de 16 años con diagnóstico de CBP, atendidos en la Unidad de Hepatología y Trasplante Hepático del Hospital Pablo Tobón Uribe, entre los años 2013 a 2021, con el fin de obtener información sobre las características de esta patología a nivel local. Resultados. Se evaluó un total de 239 pacientes, con un promedio de edad de 61,6±12,31 años, el 97,07% fue del sexo femenino, con criterios serológicos como anticuerpos antimitocondriales (AMA) positivos en un 76,89%, el 66,95% de los pacientes presentaban alguna enfermedad autoinmune concomitante y el 31,60% tuvieron sobreposición con hepatitis autoinmune. La manifestación clínica más frecuente fue el prurito en un 61,92% de los pacientes, seguido por la astenia en un 51,88%. La presencia de hipertensión portal al diagnóstico fue del 29,29%. La colangitis no supurativa y la ductopenia en la biopsia de hígado se documentó en un 43,79% de los casos. El ácido ursodesoxicólico (UDCA) fue la terapia de primera línea en el 100% de los pacientes, se identificó refractariedad del 16,36% según criterios de París II y del 31,79% con los criterios de Toronto. La no respuesta al UDCA, se asoció de manera significativa con mayor mortalidad (p=0,039) y presencia de hepatocarcinoma (p=0,042). Conclusión. Se caracterizó la CBP en nuestra población. El diagnóstico serológico por AMA fue bajo, con altos requerimientos de biopsia hepática en el contexto de síndromes de sobreposición. Los signos de hipertensión portal al momento del diagnóstico fueron prevalentes. La refractariedad bioquímica a la terapia fue descrita en relación con mayor progresión de fibrosis, aumento de mortalidad y presencia de hepatocarcinoma.

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Biografía del autor/a

Edgar Fernando Ayala-Sierra, Universidad de Antioquia

Médico, Especialista en Medicina Interna, Residente de Hepatología Clínica y Trasplante Hepático. Facultad de Medicina, Universidad de Antioquia. Medellín, Colombia.

Octavio G. Muñoz-Maya, Hospital Pablo Tobón Uribe, Universidad de Antioquia

Médico, Especialista en Medicina Interna, Especialista en Hepatología Clínica y Trasplante Hepático. Hospital Pablo Tobón Uribe. Universidad de Antioquia. Medellín, Colombia.

Felipe Guerrero-Pérez, Universidad de Antioquia

Estudiante de Medicina. Facultad de Medicina, Universidad de Antioquia. Medellín, Colombia.

Manuela Cardona-Jaramillo, Universidad de Antioquia

Estudiante de Medicina. Facultad de Medicina, Universidad de Antioquia. Medellín, Colombia.

Referencias bibliográficas

Rodríguez Lugo DA, Coronado Tovar JJ, Solano Villamarin GA, Otero Regino W. Colangitis biliar primaria. Parte 1. Actualización: generalidades, epidemiología, factores involucrados, fisiopatología y manifestaciones clínicas. Revista de Gastroenterología del Perú 2017;37:357-364.

Lleo A, Wang GQ, Gershwin ME, Hirschfield GM. Primary biliary cholangitis. Lancet 2020;396:1915-1926. https://doi.org/10.1016/s0140-6736(20)31607-x.

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. J Gastroenterol Hepatol 2020. https://doi.org/10.1111/jgh.15329.

Lleo A, Marzorati S, Anaya J-M, Gershwin M. Primary biliary cholangitis: a comprehensive overview. Hepatology International 2017;11. https://doi.org/10.1007/s12072-017-9830-1.

Llorente-Ramón A, Moreira-Alcívar JH, Barraza-Ortiz DA, et al. Colangitis biliar primaria. Rev Hosp Jua Mex. 2019;86 (4):188-195.

Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006;130:715-720. https://doi.org/10.1053/j.gastro.2005.12.029.

Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology 2019;69:394-419. https://doi.org/10.1002/hep.30145.

Mahl TC, Shockcor W, Boyer JL. Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. J Hepatol 1994;20:707-713. https://doi.org/10.1016/s0168-8278(05)80139-4.

Springer J, Cauch-Dudek K, O'Rourke K, Wanless IR, Heathcote EJ. Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis. Am J Gastroenterol 1999;94:47-53. https://doi.org/10.1111/j.1572-0241.1999.00770.x.

Lammers WJ, van Buuren HR, Hirschfield GM, Janssen HL, Invernizzi P, Mason AL, et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterology 2014;147:1338-1349.e1335; quiz e1315. https://doi.org/10.1053/j.gastro.2014.08.029.

Lee J, Belanger A, Doucette JT, Stanca C, Friedman S, Bach N. Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007;5:1313-1315. https://doi.org/10.1016/j.cgh.2007.07.015.

Kuiper EM, Hansen BE, Metselaar HJ, de Man RA, Haagsma EB, van Hoek B, et al. Trends in liver transplantation for primary biliary cirrhosis in the Netherlands 1988-2008. BMC Gastroenterol 2010;10:144. https://doi.org/10.1186/1471-230x-10-144.

Montano-Loza AJ, Hansen BE, Corpechot C, Roccarina D, Thorburn D, Trivedi P, et al. Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival. Gastroenterology 2019;156:96-107.e101. https://doi.org/10.1053/j.gastro.2018.10.001.

Carbone M, Bufton S, Monaco A, Griffiths L, Jones DE, Neuberger JM. The effect of liver transplantation on fatigue in patients with primary biliary cirrhosis: a prospective study. J Hepatol 2013;59:490-494. https://doi.org/10.1016/j.jhep.2013.04.017.

Ronca V, Gerussi A, Cristoferi L, Carbone M, Invernizzi P. Precision medicine in primary biliary cholangitis. J Dig Dis 2019;20:338-345. https://doi.org/10.1111/1751-2980.12787.

Lleo A, Leung PSC, Hirschfield GM, Gershwin EM. The Pathogenesis of Primary Biliary Cholangitis: A Comprehensive Review. Semin Liver Dis 2020;40:34-48. https://doi.org/10.1055/s-0039-1697617.

Floreani A, Tanaka A, Bowlus C, Gershwin ME. Geoepidemiology and changing mortality in primary biliary cholangitis. J Gastroenterol 2017;52:655-662. https://doi.org/10.1007/s00535-017-1333-2.

Marzioni M, Bassanelli C, Ripellino C, Urbinati D, Alvaro D. Epidemiology of primary biliary cholangitis in Italy: Evidence from a real-world database. Dig Liver Dis 2019;51:724-729. https://doi.org/10.1016/j.dld.2018.11.008.

EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017;67:145-172. https://doi.org/10.1016/j.jhep.2017.03.022.

Sakauchi F, Mori M, Zeniya M, Toda G. Antimitochondrial antibody negative primary biliary cirrhosis in Japan: utilization of clinical data when patients applied to receive public financial aid. J Epidemiol 2006;16:30-34. https://doi.org/10.2188/jea.16.30.

Morgan MA, Sundaram KM. Primary biliary cholangitis: review for radiologists. Abdom Radiol (NY) 2021. https://doi.org/10.1007/s00261-021-03335-x.

Idilman IS, Venkatesh SH, Eaton JE, Bolan CW, Osman KT, Maselli DB, et al. Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol 2020;30:5139-5148. https://doi.org/10.1007/s00330-020-06855-0.

Tanaka A, Ma X, Yokosuka O, Weltman M, You H, Amarapurkar DN, et al. Autoimmune liver diseases in the Asia-Pacific region: Proceedings of APASL symposium on AIH and PBC 2016. Hepatol Int 2016;10:909-915. https://doi.org/10.1007/s12072-016-9767-9.

Takamura M, Matsuda Y, Kimura N, Takatsuna M, Setsu T, Tsuchiya A, et al. Changes in disease characteristics of primary biliary cholangitis: An observational retrospective study from 1982 to 2016. Hepatol Res 2021;51:166-175. https://doi.org/10.1111/hepr.13586.

Nakanuma Y, Zen Y, Harada K, Sasaki M, Nonomura A, Uehara T, et al. Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: Interobserver agreement. Pathol Int 2010;60:167-174. https://doi.org/10.1111/j.1440-1827.2009.02500.x.

Tan D, Goodman ZD. Liver biopsy in primary biliary cholangitis: Indications and interpretation. Clin Liver Dis 2018;22:579-588. https://doi.org/10.1016/j.cld.2018.03.008.

Assis DN. Chronic Complications of Cholestasis: Evaluation and Management. Clin Liver Dis 2018;22:533-544. https://doi.org/10.1016/j.cld.2018.03.014.

Kaps L, Grambihler A, Yemane B, Nagel M, Labenz C, Ploch P, et al. Symptom burden and treatment response in patients with primary biliary cholangitis (PBC). Dig Dis Sci 2020;65:3006-3013. https://doi.org/10.1007/s10620-019-06009-3.

Zhang Y, Hu X, Chang J, Chen J, Han X, Zhang T, et al. The liver steatosis severity and lipid characteristics in primary biliary cholangitis. BMC Gastroenterol 2021;21:395. https://doi.org/10.1186/s12876-021-01974-4.

Guañabens N, Parés A, Ros I, Caballería L, Pons F, Vidal S, et al. Severity of cholestasis and advanced histological stage but not menopausal status are the major risk factors for osteoporosis in primary biliary cirrhosis. J Hepatol 2005;42:573-577. https://doi.org/10.1016/j.jhep.2004.11.035.

Mazzetti M, Marconi G, Mancinelli M, Benedetti A, Marzioni M, Maroni L. The Management of Cholestatic Liver Diseases: Current Therapies and Emerging New Possibilities. J Clin Med 2021;10. https://doi.org/10.3390/jcm10081763.

Rudic JS, Poropat G, Krstic MN, Bjelakovic G, Gluud C. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database Syst Rev 2012;12:Cd000551. https://doi.org/10.1002/14651858.CD000551.pub3.

Nardo AD, Schneeweiss-Gleixner M, Bakail M, Dixon ED, Lax SF, Trauner M. Pathophysiological mechanisms of liver injury in COVID-19. Liver Int 2021;41:20-32. https://doi.org/10.1111/liv.14730.

Sayiner M, Golabi P, Stepanova M, Younossi I, Nader F, Racila A, et al. Primary Biliary Cholangitis in Medicare Population: The Impact on Mortality and Resource Use. Hepatology 2019;69:237-244. https://doi.org/10.1002/hep.30174.

John BV, Khakoo NS, Schwartz KB, Aitchenson G, Levy C, Dahman B, et al. Ursodeoxycholic Acid Response is associated with reduced mortality in primary biliary cholangitis with compensated cirrhosis. Am J Gastroenterol 2021;116:1913-1923. https://doi.org/10.14309/ajg.0000000000001280.

Lindor KD, Jorgensen RA, Therneau TM, Malinchoc M, Dickson ER. Ursodeoxycholic acid delays the onset of esophageal varices in primary biliary cirrhosis. Mayo Clin Proc 1997;72:1137-1140. https://doi.org/10.4065/72.12.1137.

Imam MH, Talwalkar JA, Lindor KD. Clinical management of autoimmune biliary diseases. J Autoimmun 2013;46:88-96. https://doi.org/10.1016/j.jaut.2013.06.014.

Floreani A, Mangini C. Primary biliary cholangitis: Old and novel therapy. Eur J Intern Med 2018;47:1-5. https://doi.org/10.1016/j.ejim.2017.06.020.

Carbone M, Mells GF, Pells G, Dawwas MF, Newton JL, Heneghan MA, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 2013;144:560-569.e567. https://doi.org/10.1053/j.gastro.2012.12.005.

Cortez-Pinto H, Liberal R, Lopes S, Machado MV, Carvalho J, Dias T, et al. Predictors for incomplete response to ursodeoxycholic acid in primary biliary cholangitis. Data from a national registry of liver disease. United European Gastroenterol J 2021;9:699-706. https://doi.org/10.1002/ueg2.12095.

Carey EJ. Progress in Primary Biliary Cholangitis. N Engl J Med 2018;378:2234-2235. https://doi.org/10.1056/NEJMe1804945.

Murillo Perez CF, Harms MH, Lindor KD, van Buuren HR, Hirschfield GM, Corpechot C, et al. Goals of Treatment for Improved Survival in Primary Biliary Cholangitis: Treatment Target Should Be Bilirubin Within the Normal Range and Normalization of Alkaline Phosphatase. Am J Gastroenterol 2020;115:1066-1074. https://doi.org/10.14309/ajg.0000000000000557.

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;375:631-643. https://doi.org/10.1056/NEJMoa1509840.

Corpechot C, Chazouillères O, Rousseau A, Le Gruyer A, Habersetzer F, Mathurin P, et al. A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis. N Engl J Med 2018;378:2171-2181. https://doi.org/10.1056/NEJMoa1714519.

Reig A, Álvarez-Navascués C, Vergara M, Gómez-Domínguez E, Gallego-Moya A, Pérez-Medrano IM, et al. Obeticholic Acid and Fibrates in Primary Biliary Cholangitis: Comparative Effects in a Multicentric Observational Study. Am J Gastroenterol 2021;116:2250-2257. https://doi.org/10.14309/ajg.0000000000001343.

Schattenberg JM, Pares A, Kowdley KV, Heneghan MA, Caldwell S, Pratt D, et al. A randomized placebo-controlled trial of elafibranor in patients with primary biliary cholangitis and incomplete response to UDCA. J Hepatol 2021;74:1344-1354. https://doi.org/10.1016/j.jhep.2021.01.013.

Goel A, Kim WR. Natural History of Primary Biliary Cholangitis in the Ursodeoxycholic Acid Era: Role of Scoring Systems. Clin Liver Dis 2018;22:563-578. https://doi.org/10.1016/j.cld.2018.03.007.

Shah RA, Kowdley KV. Current and potential treatments for primary biliary cholangitis. Lancet Gastroenterol Hepatol 2020;5:306-315. https://doi.org/10.1016/s2468-1253(19)30343-7.

Shu Y, Song Y, Bai T, Pan X, Shang H, Yang L, et al. Predictive Model of Ursodeoxycholic Acid Treatment Response in Primary Biliary Cholangitis. J Clin Transl Hepatol 2021;9:187-193. https://doi.org/10.14218/jcth.2020.00127.

Cheung AC, Lapointe-Shaw L, Kowgier M, Meza-Cardona J, Hirschfield GM, Janssen HL, et al. Combined ursodeoxycholic acid (UDCA) and fenofibrate in primary biliary cholangitis patients with incomplete UDCA response may improve outcomes. Aliment Pharmacol Ther 2016;43:283-293. https://doi.org/10.1111/apt.13465.

Duan W, Ou X, Wang X, Wang Y, Zhao X, Wang Q, et al. Efficacy and safety of fenofibrate add-on therapy for patients with primary biliary cholangitis and a suboptimal response to UDCA. Rev Esp Enferm Dig 2018;110:557-563. https://doi.org/10.17235/reed.2018.5533/2018.

Hirschfield GM, Beuers U, Kupcinskas L, Ott P, Bergquist A, Färkkilä M, et al. A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA. J Hepatol 2021;74:321-329. https://doi.org/10.1016/j.jhep.2020.09.011.

Yao TT, Qian JD, Wang GQ. Efficacy of ursodeoxycholic acid combined with prednisolone and immunosuppressant triple therapy in the treatment of refractory primary biliary cholangitis. Med Clin (Barc) 2020;155:165-170. https://doi.org/10.1016/j.medcli.2020.03.013.

Zhang H, Zhang W, Jiang L, Chen Y. Recent advances in systemic therapy for hepatocellular carcinoma. Biomark Res 2022;10:3. https://doi.org/10.1186/s40364-021-00350-4.

Banales JM, Marin JJG, Lamarca A, Rodrigues PM, Khan SA, Roberts LR, et al. Cholangiocarcinoma 2020: the next horizon in mechanisms and management. Nat Rev Gastroenterol Hepatol 2020;17:557-588. https://doi.org/10.1038/s41575-020-0310-z.

Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA. Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis. J Hepatol 2020;72:95-103. https://doi.org/10.1016/j.jhep.2019.09.007.

Pol JG, Paillet J, Plantureux C, Kroemer G. Beneficial autoimmunity links primary biliary cholangitis to the avoidance of cholangiocarcinoma. Oncoimmunology 2021;10:1968595. https://doi.org/10.1080/2162402x.2021.1968595.

Aguilar MT, Carey EJ. Current Status of Liver Transplantation for Primary Biliary Cholangitis. Clin Liver Dis 2018;22:613-624. https://doi.org/10.1016/j.cld.2018.03.011.

Horwich BH, Han H. Diagnosis and Treatment of Primary Biliary Cholangitis: A Patient-Friendly Summary of the 2018 AASLD Practice Guidance. Clin Liver Dis (Hoboken) 2021;18:255-259. https://doi.org/10.1002/cld.1158.

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2022-07-05

Cómo citar

Ayala-Sierra, E. F., Muñoz-Maya, O. G., Guerrero-Pérez, F., & Cardona-Jaramillo, M. (2022). Colangitis biliar primaria: caracterización de una cohorte retrospectiva. Hepatología, 3(2), 203–217. https://doi.org/10.52784/27112330.159

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