Fenofibrate normalizes alkaline phosphatase and improves long-term outcomes in patients with advanced primary biliary cholangitis refractory to ursodeoxycholic acid / El fenofibrato normaliza la fosfatasa alcalina y mejora los resultados a largo plazo en pacientes con colangitis biliar primaria avanzada refractaria al ácido ursodesoxicólico

Background: Although patients with advanced liver disease have been included in studies evaluating fibrates for the treatment of primary biliary cholangitis (PBC), the frequency of biochemical responses and adverse effects for this group of patients was not reported separately and comprehensively. Aims: to evaluate the efficacy and safety of additional fenofibrate therapy in patients with advanced and ursodeoxycholic acid (UDCA)-refractory PBC. Methods: Patients were analyzed retrospectively to determine the clinical therapeutic effects of UDCA with additional fenofibrate therapy versus continued UDCA monotherapy. The liver transplantation (LT)-free survival and the alkaline phosphatase (ALP) normalization rates were estimated using Cox regression analyses and Kaplan–Meier plots with inverse probability of treatment weighting (IPTW). Results: A total of 118 patients were included: 54 received UDCA alone and 64 received UDCA in combination with fenofibrate therapy. In the fenofibrate and UDCA groups, 37% and 11% of patients with advanced and UDCA-refractory PBC, respectively, achieved ALP normalization (P=0.001). Additional fenofibrate therapy improved both LT-free survival and ALP normalization rate after IPTW (hazard ratio [HR]: 0.23, 95% confidence interval [CI]: 0.07–0.75, P=0.015; and HR: 11.66, 95% CI: 5.02–27.06, P=0.001, respectively). These effects were supported by parallel changes in the rates of liver decompensation and histologic progression, and the United Kingdom (UK)-PBC and Globe risk scores. (AU)

Antecedentes: Aunque los pacientes con enfermedad hepática avanzada se han incluido en los estudios que evalúan los fibratos para el tratamiento de la colangitis biliar primaria, la frecuencia de las respuestas bioquímicas y los efectos adversos para este grupo de pacientes no se informó por separado y de forma exhaustiva. Objetivos: Evaluar la eficacia y la seguridad del tratamiento adicional con fenofibrato en pacientes con colangitis biliar primaria avanzada y refractaria al ácido ursodesoxicólico. Métodos: Se analizaron los pacientes de forma retrospectiva para determinar los efectos terapéuticos clínicos del ácido ursodesoxicólico con terapia adicional de fenofibrato frente a la monoterapia continuada con ácido ursodesoxicólico. La supervivencia sin trasplante de hígado y las tasas de normalización de la fosfatasa alcalina se estimaron mediante análisis de regresión de Cox y gráficos de Kaplan-Meier con ponderación de la probabilidad inversa del tratamiento. Resultados: Se incluyeron un total de 118 pacientes: 54 recibieron ácido ursodesoxicólico solo y 64 recibieron ácido ursodesoxicólico en combinación con el tratamiento con fenofibrato. En los grupos de fenofibrato y ácido ursodesoxicólico, 37 y 11% de los pacientes con colangitis biliar primaria avanzada y refractaria al ácido ursodesoxicólico, respectivamente, lograron la normalización de la fosfatasa alcalina (p=0,001). El tratamiento adicional con fenofibrato mejoró tanto la supervivencia libre de trasplante de hígado como la tasa de normalización de la fosfatasa alcalina tras la ponderación de la probabilidad inversa del tratamiento (cociente de riesgos: 0,23, intervalo de confianza del 95% [IC 95%]: 0,07-0,75, p=0,015; y cociente de riesgos: 11,66, IC 95%: 5,02–27,06, p=0,001, respectivamente). (AU)

Ulcerative colitis complicated by autoimmune hepatitis-primary biliary cholangitis-primary sclerosing cholangitis overlap syndrome

Autoimmune liver diseases mainly include autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and overlap syndrome. Patients with IBD are usually complicated by autoimmune liver diseases. We described a rare case of UC complicated by AIH-PBC-PSC overlap syndrome. The male patient had a long history of UC. After admitted to hospital, the patient was found to have abnormal liver function, and was diagnosed with AIH-PBC-PSC overlap syndrome by liver puncture biopsy. This case will help us learn more about patients who are confirmed with IBD complicated by autoimmune liver diseases. (AU)

Practical management of primary biliary cholangitis

Primary biliary cholangitis (PBC) is a chronic and cholestatic liver disease of autoimmune pathogenesis that mainly affects middle-aged women. Patients show elevated alkaline phosphatase and bilirubin levels as the disease progresses. The main symptoms of the disease are pruritus and fatigue, which interfere with the quality of life of patients. Progressive damage leading to end stage liver disease could require liver transplantation. Despite the efficacy of ursodeoxycholic acid (UDCA), the current standard of care for PBC, up to 40% of patients have an inadequate response to the treatment, requiring a second-line therapy. Obeticholic acid is the only second-line treatment approved for PBC in combination with UDCA in adults with an inadequate response to UDCA, or as monotherapy in patients intolerant to UDCA. Although different clinical guidelines for the diagnosis and management of PBC have been published, PBC is still challenging for many physicians. In this article we briefly review the main characteristics of the disease and include a practical user-friendly algorithm for the diagnosis and management of PBC developed by Spanish PBC experts and based on the European Association for the Study of the Liver recommendations. (AU)

Clinical aspects and prognosis of patients with inflammatory bowel disease associated with autoimmune liver diseases / Aspectos clínicos y pronósticos de pacientes con enfermedad inflamatoria intestinal asociada a enfermedades hepáticas autoinmunes

Background and aims: Inflammatory bowel diseases (IBD) are chronic conditions that may be accompanied by autoimmune liver disease (AILD), most commonly primary sclerosing cholangitis (PSC). The objective of this study was to evaluate the behaviour of patients with IBD associated with AILD and compare a PSC group with a non-PSC group.Methods: Medical records of patients with IBD associated with PSC, autoimmune cholangitis, primary biliary cholangitis, small-duct PSC, autoimmune hepatitis (AIH) and overlapping syndromes were assessed.Results: Fifty-four patients were included: 48 (88.9%) had ulcerative colitis and six (11.1%) had Crohn's disease; 35 (64.8%) had PSC and 19 (35.2%) did not have PSC. There was no difference in outcomes (surgical treatment for IBD, liver transplantation or death) between the groups. Time since the diagnosis of IBD was associated with surgical treatment of IBD (p=0.041; OR: 1.139, 95% CI: 1.006–1.255). Time since the diagnosis of AILD (p=0.003; OR: 1.259, 95% CI: 1.1–1.396), as well as portal hypertension at diagnosis (p=0.014; OR: 18.22, 95% CI: 1.815–182.96), were associated with liver transplantation. In addition, previous diagnosis of AIH was associated with de novo IBD (p=0.012; OR: 7.1, 95% CI: 1.215–42.43).Conclusion: Both groups had similar disease behaviour. A longer time since the diagnosis of IBD increased the risk for surgical treatment (13.9%/year). A 25.9%/year increase in liver transplantation was observed after the diagnosis of AILD, which was increased 18.22 times by the presence of portal hypertension. In addition, the diagnosis of AIH was associated with an increase in the number of diagnoses of de novo IBD (7.1).

Antecedentes y objetivos: Las enfermedades inflamatorias intestinales (EII) son afecciones crónicas que pueden ir acompañadas de enfermedad hepática autoinmune (EHA) o colangitis esclerosante primaria (CEP). El objetivo del estudio fue evaluar el comportamiento de pacientes con EII asociada a EHA y comparar un grupo con CEP con un grupo sin CEP.Métodos: Se evaluaron las historias clínicas de pacientes con EII asociadas con CEP, colangitis autoinmune, colangitis biliar primaria, CEP de conductos pequeños, hepatitis autoinmune (HAI) y síndromes superpuestos.Resultados: Se incluyeron 54 pacientes. De ellos, 48 (88,9%) tenían colitis ulcerosa y seis (11,1%) tenían enfermedad de Crohn; 35 (64,8%) tenían CEP y 19 (35,2%) no tenían CEP. No hubo diferencias en los resultados (tratamiento quirúrgico para la EII, trasplante de hígado o muerte) entre los grupos. El tiempo transcurrido desde el diagnóstico de EII se asoció con el tratamiento quirúrgico de la EII (p=0,041). El tiempo desde el diagnóstico de EHA (p=0,003), así como la hipertensión portal en el momento del diagnóstico (p=0,014), fueron asociado con el trasplante de hígado. Además, el diagnóstico previo de HAI se asoció con EII de novo (p=0,012).Conclusión: Ambos grupos tuvieron un comportamiento de enfermedad similar. Un mayor tiempo desde el diagnóstico de EII aumentó el riesgo de tratamiento quirúrgico (13,9%/año). Se observó un aumento del 25,9%/año en el trasplante de hígado después de diagnóstico de EIA, que se incrementó 18,22 veces por la presencia de hipertensión portal. Además, el diagnóstico de HAI se asoció con un aumento en el número de diagnósticos de EII de novo (7,1).

Remission of Rheumatoid Arthritis and Primary Biliary Cholangitis After Treatment With Tocilizumab / Remisión de artritis reumatoide y colangitis biliar primaria tras tratamiento con tocilizumab

Rheumatoid arthritis (RA) is characterized by synovitis of multiple joints which if untreated progresses to joint destruction. Primary biliary cholangitis (PBC) is an autoimmune and progressive disease of the liver of unknown origin. About 1.8–5.6% of individuals with PBC have RA and patients with RA are at higher risk of developing PBC compared to the general population. We report a case of a 76-year-old man, with a history of PBC, and a recent RA diagnosis, in which tocilizumab therapy was effective in the control of RA and PBC, and a literature review was performed. This case, along with only one case published in literature in which tocilizumab was used in the treatment of RA and PBC, suggests that tocilizumab may be effective and safe in the treatment of RA in patients with PBC. Inhibition of IL-6 may also be effective in PBC treatment.(AU)

La artritis reumatoide (AR) se caracteriza por sinovitis de múltiples articulaciones que, de no tratarse, deriva en destrucción articular. La colangitis biliar primaria (CBP) es una enfermedad hepática autoinmune y progresiva de origen desconocido. Cerca del 1,8-5,6% de los individuos con CBP padecen AR, y los pacientes con AR tienen mayor riesgo de desarrollar CBP, en comparación con la población general. Reportamos el caso de un varón de 76 años con historia de CBP y diagnóstico reciente de AR, en el que la terapia con tocilizumab fue efectiva para el control de ambas situaciones y realizamos una revisión de la literatura sobre el caso. Este caso junto con otro publicado en la literatura, en el que se utilizó tocilizumab para tratar CBP y AR, sugieren que este fármaco puede ser efectivo y seguro para el tratamiento de AR en pacientes con CBP. La inhibición de IL-6 puede resultar también eficaz en el tratamiento de la CBP.(AU)

Effective treatment of autoimmune hepatitis-primary biliary cholangitis overlap syndrome with obeticholic acid

No disponible

Efficacy of ursodeoxycholic acid combined with prednisolone and immunosuppressant triple therapy in the treatment of refractory primary biliary cholangitis / Eficacia del ácido ursodesoxicólico combinado con prednisolona y triple terapia inmunosupresora en el tratamiento de la colangitis biliar primaria refractaria

Background and AIM: To explore the efficacy treatment regimen in refractory PBC. METHODS: Triple treatment including ursodeoxycholic acid, prednisolone and immunosuppressant was prescribed to 47 refractory patients. Biochemistries, immune parameters, non-invasive liver fibrosis assessments were measured during follow-up. RESULTS: Triple therapy resulted in significant decrease in ALP, GGT, ALT, AST, TBIL, ALB, IgG, IgM, APRI, FIB-4 and S-INDEX. The biochemical cumulative normalization rates of ALP and other biochemical parameters were higher in long-term follow-up. Poor outcome was observed in patients with lower ALB, higher TBIL, PT, sp100 positivity and advanced liver pathology at baseline. Osteoporosis and bone fracture were observed in 15% patients. CONCLUSIONS: Triple therapy is associated with marked decrease and normalization of ALP and other parameters. ALB, TBIL, PT, sp100 and pathology were related with poor outcome. Osteoporosis should be closely monitored

ANTECEDENTES Y OBJETIVO: Explorar el régimen de tratamiento de eficacia en PBC refractario. MÉTODOS: Se prescribió un tratamiento triple que incluyó ácido ursodesoxicólico, prednisolona e inmunosupresor a 47 pacientes refractarios. Las bioquímicas, los parámetros inmunes, las evaluaciones no invasivas de fibrosis hepática se midieron durante el seguimiento. RESULTADOS: La triple terapia resultó en una disminución significativa de ALP, GGT, ALT, AST, TBIL, ALB, IgG, IgM, APRI, FIB-4 y S-INDEX. Las tasas de normalización bioquímica acumulada de ALP y otros parámetros bioquímicos fueron mayores en el seguimiento a largo plazo. Se observó un resultado deficiente en pacientes con ALB más bajo, TBIL más alto, PT, positivo de SP100 y enfermedad hepática avanzada al inicio del estudio. La osteoporosis y la fractura ósea se observaron en el 15% de los pacientes. CONCLUSIONES: La triple terapia se asocia con una marcada disminución y normalización de ALP y otros parámetros. ALB, TBIL, PT, SP100 y la enfermedad se relacionaron con un mal resultado. La osteoporosis debe estar bajo estrecha supervisión

Rituximab therapy in rheumatoid arthritis and primary biliary cholangitis / Terapia con Rituximab en artritis reumatoide y colangitis biliar primaria

No disponible

Características y supervivencia del carcinoma hepatocelular en hígado no cirrótico / Characteristics and survival of hepatocellular carcinoma in non-cirrhotic liver

Introducción: las características, cribado, y supervivencia del carcinoma hepatocelular (CHC) en pacientes sin cirrosis están menos definidas. Pacientes y métodos: se recogieron retrospectivamente (enero 2004-octubre 2018) los pacientes con CHC diagnosticados citohistológicamente sin cirrosis. Analizamos sus características, tratamiento y supervivencia. Resultados: de los 332 pacientes con CHC, 25 cumplían los criterios de inclusión (7,5%). Varones: 76%. Mediana de edad: 69,9 años. El virus de la hepatitis B (VHB) fue el principal agente etiológico de hepatopatía: 32%, seguido de la esteatohepatitis no alcohólica (EHNA): 20%. La fibrosis fue leve (0-1) en el 44%. El nódulo se descubrió por ecografía de seguimiento en el 32%, en el 60% fue casual, y 8% por síntomas. El estadio de Barcelona Clinic Liver Cancer (BCLC) fue 0 en 4%, A 88%, B 4%, y C 4%. El tratamiento inicial mayoritario fue la resección quirúrgica (76%), 8% rechazaron tratamiento, y se realizó etanolización, quimioembolización, sorafenib y tratamiento sintomático en el 4% para cada uno. El 21% de los pacientes operados presentó complicaciones, la mitad severas. La mediana de seguimiento fue 22,2 (2,9-150,6) meses, con remisión en el 56%. Mediana de supervivencia global: 57,4 +/- 29,8 meses. Supervivencia acumulada: 84% al año, 61,6% a los 3 años y 47,9% a los 5 años. Conclusión: el 7,5% de los CHC se desarrollaron sin cirrosis. El grado de fibrosis fue leve en casi la mitad. El VHB fue la causa principal, seguida de EHNA. El estadio de BCLC principal al diagnóstico fue el precoz. La cirugía fue el tratamiento más habitual. La supervivencia a los 5 años fue cercana al 50%

Introduction: the characteristics, screening, and survival of hepatocellular carcinoma (HCC) for patients without cirrhosis have not been fully studied. Methods: A retrospective cohort study was performed in non-cirrhotic patients with histological HCC, between January 2004 and October 2018. Their characteristics, treatment, follow-up and overall survival were described. Results: 25 of the 332 patients with HCC met the inclusion criteria (7.5%), 76% were males and the median age was 69.9 years. The main etiology of liver disease was the hepatitis B virus (HBV) (32%), followed by non-alcoholic steatohepatitis (NASH) (20%). Liver fibrosis was mild (0-1) in 44% of cases. The nodule was diagnosed by ultrasonography in 32% of cases, 60% were found incidentally and 8% due to clinical symptoms. The Barcelona Clinic Liver Cancer (BCLC) staging was 0 in 4% of cases, A in 88%, B in 4% and C in 4%. The main initial treatment was surgical resection (76%) and 8% refused to be treated. Percutaneous ethanol injection, chemoembolization, sorafenib and palliative care were each performed in 4% of cases. There were some complications in 21% of patients treated with surgery, half of them were severe. The median follow-up was 22.2 (2.9-150.6) months and 56% were in remission and the median overall survival was 57.4 +/- 29.8 months. The overall cumulative survival at 1, 3 and 5 years was 84%, 61.6% and 47.9%, respectively. Conclusion: 7.5% of HCC presented without cirrhosis and almost half of patients had mild fibrosis. HBV was the main cause of HCC, followed by NASH. The most frequent BCLC stage at diagnosis was early stage and surgery was the most common treatment. Overall cumulative survival at 5 years was almost 50%

Endoluminal radiofrequency ablation with SpyGlass(TM) in the management of cholangiocarcinoma

Most extrahepatic cholangiocarcinomas are unresectable at the time of diagnosis and even in case of a resectable cancer, surgery is not an option for the elderly or patients with comorbidities (1). Current treatment alternatives in these scenarios are very limited. Biliary stenting with self-expanding metal stents (SEMS) is the mainstay palliative treatment for biliary obstruction (2). However, emerging experience with endoscopic RF ablation (RFA) in this setting has been reported in the literature

No disponible

Cumbersome removal of an endoscopic nasobiliary drainage tube

No disponible

Xantomas y xantelasmas en colangitis biliar primaria / Xanthomas and xanthelasmas in primary biliary cholangitis

No disponible