RESUMO
INTRODUCTION AND OBJECTIVES: Autoimmune liver diseases (AILDs): autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) have different survival outcomes after liver transplant (LT). Outcomes are influenced by factors including disease burden, medical comorbidities, and socioeconomic variables. MATERIALS AND METHODS: Using the United Network for Organ Sharing database (UNOS), we identified 13,702 patients with AILDs listed for LT between 2002 and 2021. Outcomes of interest were waitlist removal, post-LT patient survival, and post- LT graft survival. A stepwise multivariate analysis was performed adjusting for transplant recipient gender, race, diabetes mellitus, model for end-stage liver disease (MELD) score, and additional social determinants including the presence of education, reliance on public insurance, working for income, and U.S. citizenship status. RESULTS: Lack of college education and having public insurance increased the risk of waitlist removal (HR, 1.13; 95 % CI, 1.05-1.23, and HR, 1.09; 95 % CI, 1.00-1.18; respectively), and negatively influenced post-LT patient survival (HR, 1.16; 95 % CI, 1.06-1.26, and HR, 1.15; 95 % CI, 1.06-1.25; respectively) and graft survival (HR, 1.13; 95 % CI, 1.05-1.23, and HR, 1.15; 95 % CI, 1.06-1.25; respectively). Not working for income proved to have the greatest detrimental impact on both patient survival (HR, 1.41; 95 % CI, 1.24-1.6) and graft survival (HR, 1.21; 95 % CI, 1.09-1.35). CONCLUSIONS: Our study highlights that lack of college education and public insurance have a detrimental impact on waitlist mortality, patient survival, and graft survival. Not working for income negatively affects post-LT survival outcomes. Not having U.S. citizenship does not affect survival outcomes in AILDs patients.
Assuntos
Sobrevivência de Enxerto , Hepatite Autoimune , Transplante de Fígado , Fatores Socioeconômicos , Humanos , Masculino , Feminino , Estados Unidos/epidemiologia , Pessoa de Meia-Idade , Hepatite Autoimune/mortalidade , Hepatite Autoimune/cirurgia , Adulto , Colangite Esclerosante/cirurgia , Colangite Esclerosante/mortalidade , Listas de Espera/mortalidade , Cirrose Hepática Biliar/cirurgia , Cirrose Hepática Biliar/mortalidade , Fatores de Risco , Bases de Dados Factuais , Idoso , Escolaridade , Fatores de TempoRESUMO
Ursodeoxycholic acid is the first-line therapy for primary biliary cholangitis. However, a subset of patients fail to show biochemical response. For these patients, adjuvant therapies are warranted. Obeticholic acid was conditionally approved as a second-line drug. Evidence is building up in favor of fibrates, which are available for off-label use.
Assuntos
Ácido Quenodesoxicólico/análogos & derivados , Colagogos e Coleréticos/uso terapêutico , Colangite/tratamento farmacológico , Ácidos Fíbricos/uso terapêutico , Cirrose Hepática Biliar/tratamento farmacológico , Ácido Ursodesoxicólico/uso terapêutico , Ácido Quenodesoxicólico/efeitos adversos , Ácido Quenodesoxicólico/uso terapêutico , Colagogos e Coleréticos/efeitos adversos , Colangite/diagnóstico , Colangite/mortalidade , Quimioterapia Combinada , Ácidos Fíbricos/efeitos adversos , Humanos , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/mortalidade , Uso Off-Label , Resultado do Tratamento , Ácido Ursodesoxicólico/efeitos adversosRESUMO
INTRODUCTION: There is little information on survival rates of patients with primary biliary cholangtis (PBC) in developing countries. This is particularly true in Latin America, where the number of liver transplants performed remains extremely low for patients with advanced liver disease who fulfill criteria for liver transplantation. The goal of this study was to compare survival rate of patients with PBC in developing countries who were treated with ursodeoxycholic acid (UDCA) versus survival of patients who received other treatments (OT) without UDCA, prescribed before the UDCA era. MATERIAL AND METHODS: A retrospective study was performed, including records of 78 patients with PBC in the liver unit in a third level referral hospital in Mexico City. Patients were followed for five years from initial diagnosis until death related to liver disease or to the end of the study. Patients received UDCA (15 mg/kg/per day) (n = 41) or OT (n = 37) before introduction of UDCA in Mexico. RESULTS: Response to treatment was higher in the group that received UDCA. In the five years of follow-up, survival rates were significantly higher in the UDCA group than in the OT group. The hazard ratio of death was higher in the OT group vs. UDCA group, HR 8.78 (95% CI, 2.52-30.61); Mayo Risk Score and gender were independently associated with the risk of death. CONCLUSIONS: The study confirms that the use of UDCA in countries with a limited liver transplant program increases survival in comparison to other treatments used before the introduction of UDCA.
Assuntos
Colagogos e Coleréticos/uso terapêutico , Colangite/tratamento farmacológico , Acessibilidade aos Serviços de Saúde , Cirrose Hepática Biliar/tratamento farmacológico , Transplante de Fígado , Doadores de Tecidos/provisão & distribuição , Ácido Ursodesoxicólico/uso terapêutico , Adulto , Colangite/diagnóstico , Colangite/mortalidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/mortalidade , Masculino , México , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic, autoimmune, liver disease produced by inflammation and destruction of the interlobular bile ducts. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. OBJECTIVE: Our objective was to describe the clinical and epidemiological characteristics of patients with PBC in Uruguay. MATERIAL AND METHODS: This descriptive study included patients from 3 medical centers diagnosed with PBC in the period January 2002 to September 2011. The diagnosis was based on the presence of at least two of the following requirements: cholestasis, antimitochondrial antibodies (AMA) (or AMA subtype 2) or positive antinuclear antibodies (ANA) (anticentromere pattern) and compatible biopsy. Data recorded were sex, age, symptoms, related illness, laboratory results, images and histology at the moment of the diagnosis. RESULTS: We included 81 patients, 94% were women and the mean age was 56 years old (range: 31 to 79 years old). Symptoms were present in 59 patients (73%) and pruritus, found in 51 of them (86%), was the most frequent symptom. Positive AMA was found in 84% of cases. Histological study was available in 35 patients (43%) and 13 of them (37%) had cirrhosis. The mean survival according to the presence or absence of cirrhosis was 9.17 years (95% confidence interval: 6.79-11.56) and 10.7 years (95% confidence interval: 9.27-12.14), respectively (P = 0.03). CONCLUSIONS: Female predominance and frequent association with other autoimmune diseases were confirmed in this group. Although there was a high percentage of symptomatic and cirrhotic patients at diagnosis, only the presence of cirrhosis was associated with a lower survival.
Assuntos
Cirrose Hepática Biliar , Adulto , Idoso , Anticorpos Antinucleares/sangue , Autoanticorpos/sangue , Biópsia , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Cirrose Hepática Biliar/sangue , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/mortalidade , Masculino , Pessoa de Meia-Idade , Mitocôndrias/imunologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Uruguai/epidemiologiaRESUMO
INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic, autoimmune, liver disease produced by inflammation and destruction of the interlobular bile ducts. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. OBJECTIVE: Our objective was to describe the clinical and epidemiological characteristics of patients with PBC in Uruguay. MATERIAL AND METHODS: This descriptive study included patients from 3 medical centers diagnosed with PBC in the period January 2002 to September 2011. The diagnosis was based on the presence of at least two of the following requirements: cholestasis, antimitochondrial antibodies (AMA) (or AMA subtype 2) or positive antinuclear antibodies (ANA) (anticentromere pattern) and compatible biopsy. Data recorded were sex, age, symptoms, related illness, laboratory results, images and histology at the moment of the diagnosis. RESULTS: We included 81 patients, 94
were women and the mean age was 56 years old (range: 31 to 79 years old). Symptoms were present in 59 patients (73
) and pruritus, found in 51 of them (86
), was the most frequent symptom. Positive AMA was found in 84
of cases. Histological study was available in 35 patients (43
) and 13 of them (37
) had cirrhosis. The mean survival according to the presence or absence of cirrhosis was 9.17 years (95
confidence interval: 6.79-11.56) and 10.7 years (95
confidence interval: 9.27-12.14), respectively (P = 0.03). CONCLUSIONS: Female predominance and frequent association with other autoimmune diseases were confirmed in this group. Although there was a high percentage of symptomatic and cirrhotic patients at diagnosis, only the presence of cirrhosis was associated with a lower survival.
Assuntos
Cirrose Hepática Biliar , Adulto , Anticorpos Antinucleares/sangue , Autoanticorpos/sangue , Biópsia , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/mortalidade , Cirrose Hepática Biliar/sangue , Estimativa de Kaplan-Meier , Estudos Retrospectivos , Estudos de Coortes , Feminino , Humanos , Idoso , Masculino , Mitocôndrias/imunologia , Pessoa de Meia-Idade , Uruguai/epidemiologia , Índice de Gravidade de DoençaRESUMO
INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic, autoimmune, liver disease produced by inflammation and destruction of the interlobular bile ducts. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. OBJECTIVE: Our objective was to describe the clinical and epidemiological characteristics of patients with PBC in Uruguay. MATERIAL AND METHODS: This descriptive study included patients from 3 medical centers diagnosed with PBC in the period January 2002 to September 2011. The diagnosis was based on the presence of at least two of the following requirements: cholestasis, antimitochondrial antibodies (AMA) (or AMA subtype 2) or positive antinuclear antibodies (ANA) (anticentromere pattern) and compatible biopsy. Data recorded were sex, age, symptoms, related illness, laboratory results, images and histology at the moment of the diagnosis. RESULTS: We included 81 patients, 94
were women and the mean age was 56 years old (range: 31 to 79 years old). Symptoms were present in 59 patients (73
) and pruritus, found in 51 of them (86
), was the most frequent symptom. Positive AMA was found in 84
of cases. Histological study was available in 35 patients (43
) and 13 of them (37
) had cirrhosis. The mean survival according to the presence or absence of cirrhosis was 9.17 years (95
confidence interval: 6.79-11.56) and 10.7 years (95
confidence interval: 9.27-12.14), respectively (P = 0.03). CONCLUSIONS: Female predominance and frequent association with other autoimmune diseases were confirmed in this group. Although there was a high percentage of symptomatic and cirrhotic patients at diagnosis, only the presence of cirrhosis was associated with a lower survival.
Assuntos
Cirrose Hepática Biliar , Adulto , Idoso , Anticorpos Antinucleares/sangue , Autoanticorpos/sangue , Biópsia , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Cirrose Hepática Biliar/sangue , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/mortalidade , Masculino , Pessoa de Meia-Idade , Mitocôndrias/imunologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Uruguai/epidemiologiaRESUMO
INTRODUCTION: Liver transplantation (OLT) for primary biliary cirrhosis (PBC) is characterized by disease recurrence of up to one third of patients. The diagnosis of recurrence requires a cholestatic profile and a typical histology representing a challenge for transplant hepatologists. Antimitochondrial antibodies (AMA) establish the initial diagnosis, persist after OLT, and are thus of limited value for the diagnosis of recurrence. Aim of this analysis was to identify serological parameters associated with recurrent PBC. PATIENTS AND METHODS: OLT performed between 1992 and 2006 at Hannover Medical School were evaluated retrospectively including histology before and after OLT, autoimmune serological parameters and clinical characteristics. RESULTS: Between 1992 and 2006 72 patients underwent OLT with histologically confirmed PBC. Median follow up was 123 months. AMA persisted in 55 (76%) patients. Anti-parietal cell antibodies (PCA) were detectable in 41% of the patients before and 47% after OLT. Liver biopsies were obtained in 34 patients post OLT upon clinical suspicion, and recurrent PBC diagnosed in 28% after a mean of 71 months (range 13-161). Anti-PCA were detected in 100% of patients with recurrence before and following transplantation, 54% of patients with anti-PCA before OLT developed recurrence during follow-up. There were no differences in immunosuppressive regimen. DISCUSSION: Although unspecific for the diagnosis of PBC, anti-PCA prevalence increased after OLT, and was 100% in patients with recurrent PBC. Recurrent PBC developed in 54% of patients with anti-PCA before OLT suggesting a diagnostic role of anti-PCA as a simple and cost effective marker of recurrence.
Assuntos
Autoanticorpos/sangue , Cirrose Hepática Biliar/imunologia , Cirrose Hepática Biliar/cirurgia , Transplante de Fígado/efeitos adversos , Células Parietais Gástricas/imunologia , Adulto , Idoso , Biomarcadores/sangue , Biópsia , Feminino , Alemanha , Sobrevivência de Enxerto , Humanos , Imunossupressores/uso terapêutico , Cirrose Hepática Biliar/mortalidade , Transplante de Fígado/mortalidade , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Recidiva , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: PBC progresses to cirrhosis and results in death due to liver failure or bleeding portal hypertension. Data of the clinical characteristics and survival of PBC patients allows the assessment of therapeutical alternatives as well as the establishment of inclusion criteria for liver transplantation. AIMS: One hundred and twenty patients with histological diagnosis of PBC, admitted from 1972 to 1992, were selected with the purpose of studying the clinical and biochemical characteristics and survival. METHODS: Patients who underwent liver transplant or those who had an incomplete follow-up were excluded. RESULTS: Therefore only 80 patients were included: these were seventy five women and five men, with mean age 46 +/- 11 years (X +/- SD) to whom demographic data, biochemical analysis, liver function (Child-Pugh) and liver damage (Ludwig) were recorded at the time of histological diagnosis, which was considered zero for calculating the survival (Kaplan Meier). The most common symptoms at diagnosis were pruritus in 63 patients, jaundice in 48, asthenia and adynamia in 55 patients. Eight cases were asymptomatic. According to Child-Pugh's classification, patients were grouped as follows: forty in stage A, 29 in B, and three in C; and according to liver damage (Ludwig), 8 in grade I, 28 in grade II, 22 in grade III and 14 in grade IV. The most frequent clinical associations were Sjögren's syndrome, in 30% of patients, although one case was associated to progressive muscular dystrophy and another one to multiple myeloma and hypothyroidism; in 58.7% of the cases, antimitochondrial antibodies were negative. One year survival was 75%, five years 44%, and seven years 13%. CONCLUSIONS: The most important characteristics of the studied patients were elevated percentage of negative antimitochondrial antibodies and short survival. it is important to impel the development of liver transplantation as the only mean to improve survival.