Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 305
Filtrar
1.
Zhonghua Gan Zang Bing Za Zhi ; 29(1): 9-12, 2021 Jan 20.
Artigo em Chinês | MEDLINE | ID: mdl-33541017

RESUMO

Autoimmune liver disease is not common in children. In addition to autoimmune hepatitis, it also includes autoimmune sclerosing cholangitis, Giant cell hepatitis with autoimmune hemolytic anemia, and de novo autoimmune hepatitis after liver transplantation as well as two acquired autoimmune liver diseases: neonatal lupus and Gestational alloimmune liver disease (alternate name neonatal hemochromatosis). The age-specific systemic developmental characteristics and immune system association determine the type of autoimmune liver disease in children, and its clinical manifestations and prognostic transition may vary from adults. Here, we discuss the rising clinical incidence of autoimmune hepatitis in children.


Assuntos
Colangite Esclerosante , Hepatite Autoimune , Hepatopatias , Transplante de Fígado , Adulto , Criança , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Humanos , Prognóstico
2.
BMJ Case Rep ; 13(12)2020 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-33376090

RESUMO

Autoimmune hepatitis (AIH) is an autoimmune liver disease characterised by the presence of autoantibodies including antinuclear antibodies, anti-smooth muscle antibody and hypergammaglobulinaemia. Systemic lupus erythematosus (SLE) is a systemic disease that can affect multiple organs. Coexistence of AIH and SLE as an overlap syndrome occurs in about 1%-2.6% of the AIH cases. Since both conditions share common autoimmune features, their coexistence can pose a diagnostic dilemma which can result in a delay in treatment. We present here a challenging case of a middle-aged woman with AIH in remission who later developed new-onset fatigue, pleural effusion and splenomegaly.


Assuntos
Autoanticorpos/sangue , Hepatite Autoimune , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico , Derrame Pleural , Esplenomegalia , Biópsia/métodos , Comorbidade , Diagnóstico Diferencial , Progressão da Doença , Feminino , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/imunologia , Hepatite Autoimune/terapia , Humanos , Fígado/patologia , Testes de Função Hepática/métodos , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/terapia , Linfadenopatia/diagnóstico , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Derrame Pleural/diagnóstico , Derrame Pleural/etiologia , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
3.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(5): 886-891, 2020 Oct 18.
Artigo em Chinês | MEDLINE | ID: mdl-33047724

RESUMO

OBJECTIVE: To analyze the clinical features and prognosis in patients with primary Sjögren's syndrome (pSS) and autoimmune liver diseases (ALD). METHODS: A retrospective analysis of clinical manifestation and prognosis was performed in patients with ALD or without ALD during the three years (February 2014 to December 2017). RESULTS: Totally, 203 patients with pSS were included in this study, 68 patients had ALD (31 patients with autoimmune hepatitis, 37 patients with primary biliary cholangitis), while 135 patients did not have ALD. There were no differences between the two groups regarding age, gender, clinical manifestations, such as dry mouth, dry eyes, pain, fatigue, lymphadenopathy, glandular swelling, cutaneous involvement, lung involvement, and renal involvement, and the incidence rate of other autoimmune diseases, such as autoimmune thyroid disease, rheumatoid arthritis, and vasculitis. There were also no differences in the titer of antinuclear antibody (ANA), the positive rates of anti-Sjögren's syndrome A antibody (SSA), SSA52, and anti-Sjögren's syndrome B antibody (SSB), and at the levels of erythrocyte sedimentation rate and C-reactive protein between the two groups. Most importantly, the pSS patients with ALD had a shorter disease course, a higher positive rate of anti-mitochondrial M2 antibody (AMA-M2) and anti-centromere antibody, a higher level of IgG and IgM, a lower level of complement 3, and a decreased number of blood cells. They also had a higher level of liver related serum index, such as alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase and total bilirubin, direct bilirubin, indirect bilirubin, a higher incidence rate of liver cirrhosis, an increased death incident (the mortality was 13.24% in the pSS patients with ALD, while 2.96% in the controls, P=0.013), and a worse prognosis. Binary Logistic regression analysis revealed that liver cirrhosis, the EULAR Sjögren's syndrome disease activity index (ESSDAI) scores and the level of total bilirubin were the prognostic factors of mortality in the pSS patients with ALD. The survival curve was estimated by the Kaplan-Meier method. It demonstrated that the pSS patients with ALD had a lower survival rate when compared with the controls. CONCLUSION: The patients with both pSS and ALD will suffer from a more severe disease and a higher death incident. We should pay more attention to these patients and provide a better symptomatic treatment for them during clinical practice.


Assuntos
Hepatite Autoimune , Cirrose Hepática Biliar , Síndrome de Sjogren , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Humanos , Prognóstico , Estudos Retrospectivos , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia
4.
Expert Rev Gastroenterol Hepatol ; 14(12): 1215-1219, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32909852

RESUMO

OBJECTIVES: We aimed to evaluate the feasibility of telehealth in the management of patients with autoimmune hepatitis (AIH). The COVID-19 outbreak during the study period provided an opportunity to evaluate any pandemic influence on how telehealth was perceived by patients and physicians. METHODS: We included patients with AIH who were followed in the Harran University hospital, Turkey. Patients were managed by either remote telehealth or standard care. RESULTS: A total of 46 (telehealth, n=19 and standard care, n= 27) patients (40 female) with a median age of 32 (range 17-74) years at diagnosis were included in the study. Until the start of the COVID-19 pandemic, the rates of biochemical remission and relapse after remission were similar in the telehealth and standard care groups (89.5% vs. 89.1% and 15.8% vs. 25.9%, p=ns, for both). The telehealth group maintained remission significantly better than the standard care group (100% vs. 77.3%, p=0.035) during the COVID-19 period. All relapses were due to non-adherence to therapy. Psychiatric problems, pregnancy-related issues and drug side-effects could all be managed remotely by telehealth. CONCLUSIONS: In this study, we show for the first time that telehealth is a feasible alternative for managing AIH, both under normal circumstances and during the COVID-19 pandemic. EXPERT OPINION: Autoimmune hepatitis (AIH) requires long-life lifelong immunosuppression and follow-up for most patients. The use of telehealth may be an alternative way to evaluate these patients remotely. We show for the first time that telehealth is effective and useful in the management of AIH in regular time as well during COVID-19. We hope that our study can extend use of telehealth in the evaluation of patients with other causes of chronic liver disease.


Assuntos
Infecções por Coronavirus/epidemiologia , Surtos de Doenças/estatística & dados numéricos , Progressão da Doença , Hepatite Autoimune/terapia , Pandemias/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Telemedicina/estatística & dados numéricos , Adulto , Idoso , Estudos de Coortes , Infecções por Coronavirus/diagnóstico , Estudos de Viabilidade , Feminino , Hepatite Autoimune/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia Viral/diagnóstico , Prognóstico , Recidiva , Estudos Retrospectivos , Medição de Risco , Fatores de Tempo , Turquia , Adulto Jovem
5.
Ann Saudi Med ; 40(4): 273-280, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32564624

RESUMO

In December 2019, a novel coronavirus was identified in patients in Wuhan, China. The virus, subsequently named severe acute respiratory syndrome coronavirus-2, spread worldwide and the disease (coronavirus disease 2019 or COVID-19) was declared a global pandemic by the World Health Organization in March 2020. Older adults and individuals with comorbidities have been reported as being more vulnerable to COVID-19. Patients with chronic liver disease (CLD) have compromised immune function due to cirrhosis and are more susceptible to infection. However, it is unclear if patients with CLD are more vulnerable to COVID-19 and its complications than other populations. The high number of severe cases of COVID-19 has placed an unusual burden on health systems, compromising their capacity to provide the regular care that patients with CLD require. Hence, it is incredibly crucial at this juncture to provide a set of interim recommendations on the management of patients with CLD during the current COVID-19 outbreak.


Assuntos
Infecções por Coronavirus/epidemiologia , Hepatopatias/epidemiologia , Pneumonia Viral/epidemiologia , Monofosfato de Adenosina/efeitos adversos , Monofosfato de Adenosina/análogos & derivados , Corticosteroides/efeitos adversos , Alanina/efeitos adversos , Alanina/análogos & derivados , Amidas/efeitos adversos , Antivirais/uso terapêutico , Azetidinas/efeitos adversos , Betacoronavirus , Biópsia/métodos , Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/terapia , Comorbidade , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/prevenção & controle , Combinação de Medicamentos , Interações Medicamentosas , Inibidores Enzimáticos/efeitos adversos , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/terapia , Hepatite Viral Humana/epidemiologia , Hepatite Viral Humana/terapia , Humanos , Hidroxicloroquina/efeitos adversos , Imunossupressores/uso terapêutico , Inibidores de Janus Quinases/efeitos adversos , Cirrose Hepática/epidemiologia , Cirrose Hepática/terapia , Hepatopatias/terapia , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/terapia , Transplante de Fígado , Lopinavir/efeitos adversos , Hepatopatia Gordurosa não Alcoólica/epidemiologia , Hepatopatia Gordurosa não Alcoólica/terapia , Pandemias/prevenção & controle , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/prevenção & controle , Pirazinas/efeitos adversos , Ritonavir/efeitos adversos , Arábia Saudita/epidemiologia , Sulfonamidas/efeitos adversos , Ultrassonografia/métodos
6.
Artigo em Inglês | MEDLINE | ID: mdl-32376296

RESUMO

Autoimmune hepatitis (AIH) is a rare liver disease of autoimmune aetiology that classically affects women at reproductive age. Diagnosis of AIH is not always straightforward, and other causes of chronic liver disease must be excluded. Pregnancy in patients with AIH is associated with an increased risk of adverse maternal and foetal outcomes. In older studies, the incidence of adverse outcomes was high, with a large number of flare-ups, maternal deaths, and perinatal complications. In the most recent series, improved care based on multidisciplinary surveillance, a larger number of patients treated before and during pregnancy, and reduced incidence of cirrhosis at conception have led to better maternal outcomes and a live-birth rate similar to that in the general population. Nonetheless, AIH is still associated with preterm birth, foetal growth restriction, and unpredictable liver flares, and it represents a group of patients who need close evaluation during pregnancy.


Assuntos
Hepatite Autoimune/diagnóstico , Complicações na Gravidez/epidemiologia , Nascimento Prematuro/epidemiologia , Idoso , Autoanticorpos/sangue , Feminino , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/terapia , Humanos , Recém-Nascido , Testes de Função Hepática , Parto , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Resultado da Gravidez , Nascimento Prematuro/etiologia
7.
Aliment Pharmacol Ther ; 51(12): 1286-1304, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32363674

RESUMO

BACKGROUND: Thiopurines in combination with glucocorticoids are used as first-line, second-line and maintenance therapies in autoimmune hepatitis and opportunities exist to improve and expand their use. AIMS: To describe the metabolic pathways and key factors implicated in the efficacy and toxicity of the thiopurine drugs and to indicate the opportunities to improve outcomes by monitoring and manipulating metabolic pathways, individualising dosage and strengthening the response. METHODS: English abstracts were identified in PubMed by multiple search terms. Full-length articles were selected for review, and secondary and tertiary bibliographies were developed. RESULTS: Thiopurine methyltransferase activity and 6-tioguanine (6-thioguanine) nucleotide levels influence drug efficacy and safety, and they can be manipulated to improve treatment response and prevent myelosuppression. Methylated thiopurine metabolites are associated with hepatotoxicity, drug intolerance and nonresponse and their production can be reduced or bypassed. Universal pre-treatment assessment of thiopurine methyltransferase activity and individualisation of dosage to manipulate metabolite thresholds could improve outcomes. Early detection of thiopurine resistance by metabolite testing, accurate estimations of drug onset and strength by surrogate markers and adjunctive use of allopurinol could improve the management of refractory disease. Dose-restricted tioguanine (thioguanine) could expand treatment options by reducing methylated metabolites, increasing the bioavailability of 6-tioguanine nucleotides and ameliorating thiopurine intolerance or resistance. CONCLUSIONS: The efficacy and safety of thiopurines in autoimmune hepatitis can be improved by investigational efforts that establish monitoring strategies that allow individualisation of dosage and prediction of outcome, increase bioavailability of the active metabolites and demonstrate superiority to alternative agents.


Assuntos
Hepatite Autoimune/tratamento farmacológico , Purinas/uso terapêutico , Alopurinol/uso terapêutico , Azatioprina/uso terapêutico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Nucleotídeos de Guanina/uso terapêutico , Hepatite Autoimune/epidemiologia , Humanos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Mercaptopurina/uso terapêutico , Melhoria de Qualidade , Tioguanina/uso terapêutico , Tionucleotídeos/uso terapêutico , Resultado do Tratamento
8.
Medicine (Baltimore) ; 99(20): e20205, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32443344

RESUMO

BACKGROUND: Autoimmune liver disease (ALD) is a chronic liver disease caused by immune dysfunction in the body. However, no causative or curative medical treatment with proven efficacy exists to cure ALDs, and liver transplantation (LT) remains the only effective treatment available. However, the problem of recurrence of ALDs (rALDs) still remains after LT, which seriously affects the survival rate of the patients. Therefore, clinicians need to be aware of the risk factors affecting rALDs after LT. Therefore, this meta-analysis aims to define the risk factors for rALDs, which include the recurrence of primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune hepatitis. METHODS: A systematic search in Pubmed, Embase, Cochrane library and Web of Science databases was performed from 1980 to 2019. The inclusion criteria were risk factors for developing rALDs after LT. However, case series, case reports, reviews, meta-analysis and studies only including human immunodeficiency virus cases, children, and pregnant patients were excluded. RESULTS: The electronic database search yielded 1728 results. Sixty-three retrospective cohort studies met the inclusion criteria and 13 were included in the meta-analysis. The final cohort included 5077 patients, and among them, 21.96% developed rALDs. Colectomy before LT, HR 0.59 (95% confidence interval [CI]: 0.37-0.96), cholangiocarcinoma, HR 3.42 (95% CI: 1.88-6.21), multiple episodes of acute cellular rejection, HR 2.07 (95% CI: 1.27-3.37), model for end-stage liver disease score, HR 1.05 (95% CI: 1.02-1.08), use of mycophenolate mofetil, HR 1.46 (95% CI: 1.00-2.12) and the use of cyclosporin A, HR 0.69 (95% CI: 0.49-0.97) were associated with the risk of rprimary sclerosing cholangitis. In addition, the use of tacrolimus, HR 1.73 (95% CI: 1.00-2.99) and cyclosporin A, HR 0.59 (95% CI: 0.39-0.88) were associated with the risk of rALD. CONCLUSIONS: Multiple risk factors for rALDs were identified, such as colectomy before LT, cholangiocacinoma, multiple episodes of acute cellular rejection, model for end-stage liver disease score, and especially the use of mycophenolate mofetil, cyclosporin A and tacrolimus.


Assuntos
Colangite Esclerosante/etiologia , Hepatopatias/imunologia , Transplante de Fígado/efeitos adversos , Adulto , Inibidores de Calcineurina/efeitos adversos , Colangiocarcinoma/complicações , Colangite Esclerosante/induzido quimicamente , Colangite Esclerosante/epidemiologia , Colectomia/efeitos adversos , Colectomia/estatística & dados numéricos , Ciclosporina/efeitos adversos , Doença Hepática Terminal/complicações , Inibidores Enzimáticos/efeitos adversos , Feminino , Rejeição de Enxerto/complicações , Hepatite Autoimune/complicações , Hepatite Autoimune/epidemiologia , Humanos , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/epidemiologia , Hepatopatias/etiologia , Hepatopatias/mortalidade , Hepatopatias/patologia , Transplante de Fígado/mortalidade , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Recidiva , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Tacrolimo/efeitos adversos
9.
Z Gastroenterol ; 58(5): 431-438, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32392605

RESUMO

BACKGROUND: Population-based data on the prevalence of and real-life treatment for the autoimmune liver diseases (AILD), primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH), are scarce, and such knowledge may help to improve patient care. METHODS: Data of 8.1 million individuals having health insurance with the "Techniker Krankenkasse," the largest German health insurer, were analyzed with regard to the prevalence of and prescribed medication for AILD in Germany from 2011 until 2014. Patients with viral hepatitis B infection (HBV) and alcoholic liver cirrhosis (ALC) served as control groups. Case definition was based on ICD codes. RESULTS: The prevalences of PBC and AIH were 36.9/100 000 inhabitants (95 % CI: 35.6-38.2) and 23.0/100 000 inhabitants (95 % CI: 22.0-24.0) in 2014, respectively. The prevalences of AILD increased from 2011 to 2014 (for PBC by 31 % and for AIH by 29 %), with the largest increase for male patients with PBC. In contrast, the prevalence of HBV declined while that of ALC remained stable. The analysis of prescribed real-life treatment revealed considerable deviations from standard treatment recommendations. Specifically, in older patients with PBC or AIH, undertreatment was common. CONCLUSION: The prevalence of PBC and AIH based on ICD codes is increasing in Germany. The analysis of real-life treatment in this large and population-based cohort points to the unmet need to improve the implementation of treatment guidelines for autoimmune liver diseases in the broader medical community.


Assuntos
Hepatite Autoimune/epidemiologia , Cirrose Hepática Biliar/epidemiologia , Idoso , Alemanha/epidemiologia , Humanos , Classificação Internacional de Doenças , Masculino , Vigilância da População , Prevalência
10.
Isr Med Assoc J ; 22(2): 100-103, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32043327

RESUMO

BACKGROUND: Autoimmune hepatitis (AIH) may be associated with other autoimmune diseases. Autoantibodies are common in AIH suggesting their potential role in the pathogenesis of the disease. Among these autoantibodies, thyroid autoantibodies have been reported in patients with chronic hepatitis, with greater prevalence in patients with chronic hepatitis C infection. OBJECTIVES: To assess the prevalence of thyroid dysfunction among patients with AIH. METHODS: In this case-control, retrospective study, we examined patients diagnosed with AIH according to both the original and revised international AIH group scoring systems. Patients with other hepatic pathologies were excluded AIH was evaluated as an independent risk factor for thyroid disease by a logistic regression model. Univariate and multivariate regression analyses were conducted using hypothyroidism and hyperthyroidism as the dependent variables. RESULTS: Our cohort comprised 163 patients diagnosed with AIH and 1104 healthy age- and gender-matched controls. Hypothyroidism was more prevalent among those with AIH compared to controls (17.7% vs. 5%, respectively, 95% confidence interval [95%CI] 1.68-2.48, P < 0.001). Hyperthyroidism was more prevalent in AIH patients compared to controls (odds ratio 3.2% and 1.2%, respectively, 95%CI 1.68-2.47, P < 0.001). Using a multivariate logistic analysis, we found an independent association between AIH and hypothyroidism but not with hyperthyroidism. CONCLUSIONS: Thyroid dysfunction is more prevalent in patients with AIH. Whether thyroid dysfunction is the cause or a risk factor for AIH, or vice versa, is still unclear. Screening for thyroid dysfunction is warranted after AIH is diagnosed.


Assuntos
Hepatite Autoimune , Hipotireoidismo , Glândula Tireoide/imunologia , Adulto , Autoanticorpos/análise , Autoimunidade/imunologia , Estudos de Casos e Controles , Feminino , Hepatite C Crônica/diagnóstico , Hepatite C Crônica/epidemiologia , Hepatite C Crônica/imunologia , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/imunologia , Humanos , Hipotireoidismo/diagnóstico , Hipotireoidismo/epidemiologia , Hipotireoidismo/imunologia , Israel/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Medição de Risco , Testes de Função Tireóidea/métodos , Testes de Função Tireóidea/estatística & dados numéricos
11.
J Pediatr ; 218: 121-129.e3, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31955873

RESUMO

OBJECTIVES: To report baseline features and long-term medical/social outcomes of juvenile autoimmune liver disease, including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. STUDY DESIGN: Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. RESULTS: Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. CONCLUSIONS: Seamless management of juvenile autoimmune liver disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile autoimmune liver disease.


Assuntos
Colangite Esclerosante/terapia , Hepatite Autoimune/terapia , Adolescente , Autoanticorpos/imunologia , Criança , Colangite Esclerosante/epidemiologia , Continuidade da Assistência ao Paciente , Emprego , Feminino , Seguimentos , Hepatite Autoimune/epidemiologia , Humanos , Imunossupressão , Imunossupressores/uso terapêutico , Estimativa de Kaplan-Meier , Transplante de Fígado , Masculino , Equipe de Assistência ao Paciente , Período Pós-Operatório , Recidiva , Estudos Retrospectivos , Centros de Atenção Terciária , Fatores de Tempo , Resultado do Tratamento
12.
J Immunol Res ; 2019: 9437043, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31886312

RESUMO

Autoimmune hepatitis (AIH) is a severe liver disease that arises in genetically predisposed male and female individuals worldwide. Diagnosis of AIH is made clinically applying diagnostic scores; however, the heterotopic disease phenotype often makes a rapid determination of disease challenging. AIH responds favorably to steroids and pharmacologic immunosuppression, and liver transplantation is only necessary in cases with acute liver failure or end-stage liver cirrhosis. Recurrence or development of de novo AIH after transplantation is possible, and treatment is similar to standard AIH therapy. Current experimental investigations of T cell-mediated autoimmune pathways and analysis of changes within the intestinal microbiome might advance our knowledge on the pathogenesis of AIH and trigger a spark of hope for novel therapeutic strategies.


Assuntos
Autoimunidade , Suscetibilidade a Doenças/imunologia , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/etiologia , Hepatite Autoimune/terapia , Animais , Biomarcadores , Terapia Combinada , Hepatite Autoimune/epidemiologia , Humanos , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
13.
Aliment Pharmacol Ther ; 50(10): 1120-1126, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31617229

RESUMO

BACKGROUND: Autoimmune hepatitis requires long-term therapy, and systemic corticosteroids are the backbone of therapeutic management. Prolonged use of corticosteroids may lead to adverse events but data from long-term studies are mainly derived from studies in rheumatic diseases. AIM: To assess cataract, diabetes and fractures in relation to corticosteroid doses in the long-term maintenance treatment of patients with autoimmune hepatitis. METHODS: We retrospectively collected data on 476 patients (77% women) with an established diagnosis of autoimmune hepatitis. Binary logistic regression with a generalised estimating equation was used to analyse the association between current corticosteroid use and the incidence of cataract, diabetes and fractures with onset after autoimmune hepatitis diagnosis. We corrected for sex, age, cirrhosis at diagnosis and predniso(lo)ne use in the prior 3 years to account for possible ongoing effects. RESULTS: A total of 6634 years, with a median of 13 (range 1-40) per patient were recorded. The median age at diagnosis was 44 years (range 2-88). Adverse events were documented in 120 (25%) patients. Low-dose predniso(lo)ne (0.1-5.0 mg/d) increased the odds of fractures whereas higher doses (>5.0 mg/d) increased the odds of cataracts and diabetes. Budesonide increased the odds of cataract and fractures; this effect was independent of predniso(lo)ne use in the prior 1, 2 or 3 years. CONCLUSIONS: Even low doses of corticosteroids frequently lead to substantial adverse events refuting the assumption that adverse events are prevented by administering low doses.


Assuntos
Corticosteroides/administração & dosagem , Corticosteroides/efeitos adversos , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Hepatite Autoimune/tratamento farmacológico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Fraturas Ósseas/induzido quimicamente , Fraturas Ósseas/epidemiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Hepatite Autoimune/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
14.
World J Gastroenterol ; 25(35): 5388-5402, 2019 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-31558881

RESUMO

BACKGROUND: Abnormal liver chemistry is a common problem in human immunodeficiency virus (HIV)-infected patients. Common causes of abnormal liver enzymes in this population include viral hepatitis B/C or opportunistic infection, drug toxicity, and neoplasm. Autoimmune hepatitis is a rare cause of hepatitis in HIV-infected individuals; however, this condition has been increasingly reported over the past few years. CASE SUMMARY: We present 13 HIV-infected patients (5 males and 8 females) who developed autoimmune hepatitis (AIH) after their immune status was restored, i.e. all patients had stable viral suppression with undetectable HIV viral loads, and median CD4+ counts of 557 cells/× 106 L. Eleven patients presented with chronic persistent elevation of aminotransferase enzyme levels. One patient presented with acute hepatitis and the other patient presented with jaundice. The median levels of aspartate aminotransferase and alanine aminotransferase enzymes were 178 and 177 U/mL, respectively. Elevation of immunoglobulin G levels was present in 11 (85%) patients. Antinuclear antibody and anti-smooth muscle antibody were positive in 11 (85%) and 5 (38%) patients. Liver biopsy was performed in all patients. They had histopathological findings compatible with AIH. The patients were started on prednisolone for remission induction, with good response. After improvement of the liver chemistry, the dose of prednisolone was tapered, and azathioprine was added as life-long maintenance therapy. At the last follow-up visit, all were doing well, without HIV viral rebound or infectious complications. CONCLUSION: This report underscores the emergence of autoimmune hepatitis in the context of HIV infection.


Assuntos
Carga Global da Doença , Infecções por HIV/complicações , Hepatite Autoimune/epidemiologia , Adulto , Alanina Transaminase/sangue , Fármacos Anti-HIV/uso terapêutico , Anticorpos Antinucleares/sangue , Anticorpos Antinucleares/imunologia , Aspartato Aminotransferases/sangue , Biópsia , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Diagnóstico Diferencial , Feminino , Glucocorticoides/uso terapêutico , Infecções por HIV/sangue , Infecções por HIV/tratamento farmacológico , Infecções por HIV/imunologia , Hepatite Alcoólica/diagnóstico , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/tratamento farmacológico , Hepatite Autoimune/imunologia , Humanos , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Incidência , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Prevalência , Indução de Remissão/métodos , Resultado do Tratamento
15.
Medicine (Baltimore) ; 98(37): e17094, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31517833

RESUMO

BACKGROUND: Liver disease in patients with HIV is common and typically has complex and multifactorial presentations that represent a major cause of morbidity and mortality. Autoimmune hepatitis (AIH) is rarely reported in patient with HIV and the disease course and clinical outcomes for treatment have not been well characterized. We are aiming to determine the patient characteristics, disease prevalence, and treatment outcomes from published articles of patients with HIV and AIH. METHOD: A systematic search of PubMed, Web of Science, and Google Scholar through February 20, 2019 identified 15 studies that reported the outcomes of AIH in patients with HIV. Because of the small sample sizes and skewed distributions, resampling tests of mean differences using permutation distributions (MAXn = 10,000 permutations) were utilized; analyses were performed using R (v. 3.5.1). Categorical differences were calculated using Fisher exact test for odds ratio = 1 (equal odds), and Cramer V was calculated for effect size; analyses were completed in SPSS (v. 25). RESULTS: By reviewing 15 studies reporting a total of 35 patients with AIH and HIV, male patients were found to have significantly higher aspartate transaminase and alanine transaminase levels at time of diagnosis. No other significant findings identified. The CD4 count and viral load did not show significant correlation with AIH diagnosis or its prognosis. All patients but one who presented with severe immune deficiency and responded to highly active anti-retroviral therapy received immunosuppressive treatment without side effects and achieved remission except 2 lost to follow-up and 3 expired. CONCLUSION: Although rare, but AIH can develop in patients with HIV and physicians should consider it in the differential diagnosis for HIV patients presented with abnormal liver function tests, especially after excluding hepatitis C virus and drug-induced liver injury.Patients with immune deficiency disorders who present with AIH can be treated safely with steroid either as monotherapy or in combination with another immune suppressant therapy.


Assuntos
Infecções por HIV/complicações , Hepatite Autoimune/complicações , Adulto , Alanina Transaminase/análise , Alanina Transaminase/sangue , Aspartato Aminotransferases/análise , Aspartato Aminotransferases/sangue , Feminino , Infecções por HIV/sangue , Infecções por HIV/epidemiologia , Hepatite Autoimune/sangue , Hepatite Autoimune/epidemiologia , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
16.
BMC Gastroenterol ; 19(1): 153, 2019 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-31455255

RESUMO

BACKGROUND: Primary sclerosing cholangitis is a chronic cholestatic liver disease. The pathomechanism is still not fully understood, but there is evidence that immune-mediated processes may contribute to disease progression. METHODS: We studied the prognostic relevance of serum immunoglobulin G (IgG) elevated above the upper limit of normal as a marker for immune activation at initial diagnosis and its influence on transplantation-free survival in a well-defined cohort of PSC patients. RESULTS: The final study cohort comprises of 148 PSC patients. Elevated IgG levels were found in 66 patients (44.6%). Apart from their younger age at first diagnosis, there was no significant difference between patients with or without elevated IgG levels. The presence of a concomitant inflammatory bowel disease, an autoimmune hepatitis or immunosuppressive medication was equally distributed between both groups. Patients with elevated IgG levels reached the combined endpoint (34 (59.6%) vs. 23 (40.4%); p = 0.004) significantly more often and had reduced transplantation-free survival (Log-rank: 24.0 (10.2-37.9) vs. 14.0 (8.5-19.5); p < 0.05). Cox regression analysis including age, gender, presence of IBD, presence of dominant stricture (DS), Mayo Risk Score (MRS), immunosuppression, biochemical response to UDCA and elevated IgG-levels confirmed MRS (p = 0.03), DS (p = 0.04), biochemical response (p = 0.04) and elevated IgG level (p = 0.04) as independent risk factors for reduced transplantation-free survival. CONCLUSION: We identified elevated serum IgG levels at first diagnosis as an independent risk factor for reduced transplant free-survival in patients with PSC.


Assuntos
Colangite Esclerosante , Colestase , Hepatite Autoimune , Imunoglobulina G/sangue , Cirrose Hepática Biliar , Transplante de Fígado/estatística & dados numéricos , Adulto , Autoimunidade , Biomarcadores/sangue , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/imunologia , Colangite Esclerosante/mortalidade , Colangite Esclerosante/cirurgia , Colestase/diagnóstico , Colestase/etiologia , Progressão da Doença , Feminino , Alemanha/epidemiologia , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Humanos , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/etiologia , Masculino , Avaliação de Processos e Resultados em Cuidados de Saúde , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Análise de Sobrevida
17.
Liver Int ; 39(12): 2341-2349, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31436903

RESUMO

BACKGROUND & AIMS: Population-based studies on the epidemiology of autoimmune hepatitis (AIH) are scarce. Drug-induced AIH (DIAIH) is increasingly recognized in association with immunomodulatory therapy. We aimed to determine the incidence, prevalence and natural history of AIH in a population-based setting. METHODS: We collected data of new diagnosis of AIH in Iceland from 2006 to 2015. Cases were identified through search of diagnostic codes and text search for AIH within electronical medical records of all hospitals in Iceland and through records of smooth muscle antibodies (SMA) test results by the only laboratory in the country analyzing SMA. Patients were included in the final analysis if they received the clinical diagnosis of AIH or were started on immunosuppressive therapy. RESULTS: The mean annual incidence of AIH in Iceland was 2.2 cases per 100 000 inhabitants. Point prevalence on 31 December 2015 was 27/100 000. The median age at diagnosis was 56 years and 86% of patients were of female gender. DIAIH was suspected in 13 of 71 patients (18%) of which eight cases were related to infliximab. Immunosuppressive treatment was started in all but two patients. At the end of follow-up (median 4.8 years) 66 of 71 (93%) patients were alive. CONCLUSION: The incidence and prevalence rates of AIH in Iceland are the highest reported so far in a population-based setting. Higher incidence can partly be explained by the increasing use of biological drugs. Immunosuppressive therapy was very effective in achieving remission and prognosis was favorable.


Assuntos
Produtos Biológicos/efeitos adversos , Hepatite Autoimune/epidemiologia , Imunossupressores/uso terapêutico , Adolescente , Adulto , Idoso , Criança , Estudos de Coortes , Feminino , Hepatite Autoimune/tratamento farmacológico , Hepatite Autoimune/etiologia , Humanos , Islândia/epidemiologia , Incidência , Masculino , Pessoa de Meia-Idade , Prevalência , Adulto Jovem
18.
Liver Int ; 39(9): 1768-1775, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31152478

RESUMO

BACKGROUND & AIMS: Sclerosing cholangitis (SC) is a severe liver disease leading to destruction of bile ducts. It is believed to run a milder course in children than in adults. To test this assumption, we evaluated time-to-complication curves in two independent paediatric-onset cohorts from the same geographical area. METHODS: Short-term disease outcomes were evaluated with an online clinical registry that was filled with data on children with SC diagnosed between 2000 and 2017 and who were followed bi-annually thereafter. Long-term disease outcomes were evaluated in a paediatric-onset subcohort derived from a previously published population-based study from the Netherlands. Time-to-complication in the first cohort was defined as the time from diagnosis until portal hypertension, biliary obstructions and infections, development of malignancy, or liver transplantation, whichever came first. In the second cohort time-to-complication was defined as the time until liver transplantation or PSC-related death. RESULTS: Median age at diagnosis in the first cohort (n = 86) was 12.3 years. In the first 5 years post-diagnosis 23% of patients developed complications. The patients in the population-based study (n = 683) were stratified into those diagnosed before the age of 18 years ('paediatric-onset' subcohort, n = 43) and those diagnosed after the age of 18 years ('adult-onset' subcohort, n = 640). Median age at diagnosis was 14.6 and 40.2 years, respectively. Median time-to-complication in the paediatric-onset and adult-onset subcohorts was not statistically different. CONCLUSION: Paediatric and adult-onset SC run a similar long-term disease course. Paediatricians who treat children with SC should monitor them closely to recognize early complications and control long-term sequelae.


Assuntos
Colangite Esclerosante/epidemiologia , Hepatite Autoimune/epidemiologia , Hipertensão Portal/epidemiologia , Adolescente , Adulto , Idade de Início , Criança , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/mortalidade , Estudos de Coortes , Progressão da Doença , Feminino , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/mortalidade , Humanos , Fígado/patologia , Transplante de Fígado , Modelos Logísticos , Masculino , Países Baixos/epidemiologia , Prognóstico , Sistema de Registros , Adulto Jovem
19.
Dig Liver Dis ; 51(11): 1604-1609, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31171486

RESUMO

BACKGROUND: Autoimmune Hepatitis is a chronic liver disease while Cardiovascular Disease is seen in inflammatory states. This study sought to determine if Cardiovascular Disease was associated with Autoimmune Hepatitis. METHODS: The National Inpatient Sample selected patients with a primary diagnosis of Autoimmune Hepatitis and secondary diagnosis of Cardiovascular Disease in 2014. The primary outcome was the association of Autoimmune Hepatitis with Cardiovascular Disease. Secondary outcomes evaluated the hospital burden with Cardiovascular Disease. RESULTS: 16,375 patients with Autoimmune Hepatitis were included in the study. There was a decreased association between Autoimmune Hepatitis and Cardiovascular Disease (aOR 0.77, 95% CI 0.69-0.85, p < 0.00), Coronary Artery Disease, (aOR 0.75, 95% CI 0.67-0.85, p < 0.00), and Peripheral Vascular Disease (aOR 0.75, 95% CI 0.60-0.93, p = 0.01). Moreover, Coronary Artery Disease comprises 84% of the overall Cardiovascular Disease cohort and did not demonstrate significantly increased length of stay (aOR -0.53, 95% CI -1.16 to 0.12, p = 0.11) or hospitalization cost (aOR -6711, 95% CI -14336 to 912, p = 0.08). DISCUSSION: The decreased association between Autoimmune Hepatitis and Cardiovascular Disease is likely multifactorial in etiology. Consequently, this observation requires further examination with prospective trials.


Assuntos
Doenças Cardiovasculares/epidemiologia , Hepatite Autoimune/epidemiologia , Idoso , Doenças Cardiovasculares/mortalidade , Estudos Transversais , Bases de Dados Factuais , Feminino , Hepatite Autoimune/mortalidade , Mortalidade Hospitalar , Humanos , Pacientes Internados , Tempo de Internação/economia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco , Estados Unidos
20.
Sci Rep ; 9(1): 7925, 2019 05 28.
Artigo em Inglês | MEDLINE | ID: mdl-31138864

RESUMO

Autoimmune hepatitis (AIH) is an autoimmune liver disease and cirrhosis is sometimes complicated with AIH at diagnosis, influencing its prognosis. TNFAIP3 gene encodes A20, an inhibitor of nuclear factor-κB pathway, and is a susceptibility gene for autoimmune diseases. We investigated deleterious variants in the coding regions of TNFAIP3 gene of Japanese AIH patients or those with cirrhosis. The deleterious variants in the coding regions of TNFAIP3 gene were analyzed by the cycle sequencing method and the frequencies of deleterious TNFAIP3 alleles of AIH or AIH with cirrhosis were compared with those of Japanese controls. The deleterious alleles in TNFAIP3 were not associated with AIH. A significant association was shown for the deleterious alleles in TNFAIP3 (P = 0.0180, odds ratio (OR) 4.28, 95% confidence interval (CI) 1.53-11.95) with AIH with cirrhosis at presentation. The serum IgM levels in AIH patients with deleterious alleles in TNFAIP3 were tended to be lower than those without (P = 0.0152, Q = 0.1216). The frequency of deleterious alleles in TNFAIP3 was higher in the AIH subset without the DRB1 risk alleles than that with (P = 0.0052, OR 5.10, 95%CI 1.55-16.74). The deleterious alleles in TNFAIP3were associated with AIH with cirrhosis.


Assuntos
Hepatite Autoimune/genética , Cirrose Hepática/genética , Polimorfismo de Nucleotídeo Único , Proteína 3 Induzida por Fator de Necrose Tumoral alfa/genética , Adulto , Idoso , Feminino , Frequência do Gene , Predisposição Genética para Doença , Hepatite Autoimune/complicações , Hepatite Autoimune/epidemiologia , Humanos , Japão/epidemiologia , Cirrose Hepática/complicações , Cirrose Hepática/epidemiologia , Masculino , Pessoa de Meia-Idade
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...