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1.
BMJ Case Rep ; 14(3)2021 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-33766959

RESUMO

Double filtration plasmapheresis (DFPP) is an apheretic technique that selectively removes high molecular weight substances using a plasma component filter. DFPP has been used to treat positive-sense RNA virus infections, mainly chronic hepatitis C virus (HCV) infection, because of its ability to directly eliminate viral particles from blood plasma from 2008 to about 2015, before direct-acting antiviral agents was marketed. This effect has been termed virus removal and eradication by DFPP. HCV is a positive-sense RNA virus similar to West Nile virus, dengue virus and the SARS and Middle East respiratory syndrome coronaviruses. SARS-CoV-2 is classified same viral species. These viruses are all classified in Family Flaviviridae which are family of single-stranded plus-stranded RNA viruses. Viral particles are 40-60 nm in diameter, enveloped and spherical in shape. We present a rare case of HCV removal where an RNA virus infection that copresented with virus-associated autoimmune hepatitis was eliminated using DFPP. Our results indicate that DFPP may facilitate prompt viraemia reduction and may have novel treatment applications for SARS-CoV-2, that is, use of therapeutic plasma exchange for fulminant COVID-19.


Assuntos
Coinfecção/terapia , Coinfecção/virologia , Hepatite C Crônica/terapia , Hepatite Autoimune/terapia , Plasmaferese/métodos , Antivirais/uso terapêutico , /terapia , Quimioterapia Combinada , Feminino , Hepatite C Crônica/complicações , Hepatite Autoimune/complicações , Humanos , Interferon alfa-2/uso terapêutico , Pessoa de Meia-Idade , Polietilenoglicóis/uso terapêutico , Ribavirina/uso terapêutico , Resultado do Tratamento , Carga Viral
4.
Medicine (Baltimore) ; 99(42): e22446, 2020 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-33080679

RESUMO

Autoimmune hepatitis (AIH) is a form of liver inflammation in which immune cells target hepatocytes, inducing chronic inflammatory states. Bariatric surgery (BS) was shown to reduce inflammation in severely obese patients. We hypothesize that obese patients with AIH and BS have lower prevalence of liver-related complications and in-patient mortality compared to those without BS.The National Inpatient Sample from 2007 to 2013 was queried for hospitalizations of adults over 18 years of age with a diagnosis of AIH. Of those, hospitalizations with BS were selected as cases and those with morbid obesity as controls. Case-control 1:2 matching was done based on sex, age, race, and comorbidities. Primary outcomes were prevalence of liver-related complications and in-patient mortality. Independent risk factors of in-patient clinical outcomes were identified using multivariate regression analysis.From 137,834 hospitalizations with a diagnosis of AIH, 688 with BS were selected as cases, and 1295 were matched as controls. The prevalence of ascites was higher in the BS group compared to the control (odds ratio 1.73, 95% confidence interval (CI) 1.27-2.36). The prevalence of cirrhosis (36.8% vs 33.2%), portal hypertension (7.4% vs 10.0%), hepatic encephalopathy (10.6% vs 8.7%), and varices and variceal bleeding (3.9% vs 5.5%) was not statistically different from case controls, (P > .05).BS was an independent risk factor for ascites (adjusted odds ratio (aOR) 1.87; 95% CI 1.36-2.56) and hepatic encephalopathy (aOR 1.42; 95% CI 1.03-1.97) but was an independent protective factor against in-patient mortality (aOR 0.21, 95% CI 0.08-0.55) once adjusted for age, sex, race, and comorbidities.


Assuntos
Cirurgia Bariátrica , Hepatite Autoimune/complicações , Obesidade Mórbida/cirurgia , Avaliação de Resultados em Cuidados de Saúde , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Pesquisa sobre Serviços de Saúde , Hepatite Autoimune/mortalidade , Mortalidade Hospitalar , Humanos , Hepatopatias/mortalidade , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Prevalência , Estudos Retrospectivos , Fatores de Risco , Estados Unidos/epidemiologia
5.
Medicine (Baltimore) ; 99(42): e22805, 2020 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-33080756

RESUMO

The prevalence and impact of hepatic steatosis among patients with autoimmune hepatitis (AIH) is not well described.We conducted a cross-sectional study to determine the prevalence of hepatic steatosis in AIH patients and examined its relationship with hepatic fibrosis using vibration controlled transient elastography. Liver stiffness measurement (LSM), controlled attenuation parameter (CAP), gender, current age, and body mass index (BMI) were collected from 277 AIH patients. Hepatic steatosis was defined as CAP >263 db/m.The study participants were mostly female (82%) with an average age of 49 years and BMI 29.7 kg/m. Mean LSM was 12.5 (standard deviation 13.5) kPa and CAP was 244 (standard deviation 63) db/m. The prevalence of coexisting steatosis was 33.2%, and steatosis did not correlate with LSM (r = 0.05, P = .46). In this study, only gender (females with 31% lower LSM on average compared to males, P = .001) and BMI (each unit increase of BMI resulted in a 1.48% increase on average LSM, P = .01) correlated with LSM. Male gender had significant association with increased LSM, after controlling for age, BMI, and CAP (P = .001).This exploratory study using noninvasive vibration controlled transient elastography revealed hepatic steatosis is highly prevalent in patients with AIH but not associated with liver fibrosis.


Assuntos
Técnicas de Imagem por Elasticidade , Fígado Gorduroso/diagnóstico , Hepatite Autoimune/complicações , Índice de Massa Corporal , Estudos Transversais , Fígado Gorduroso/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores Sexuais
6.
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
7.
Medicine (Baltimore) ; 99(34): e21408, 2020 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-32846758

RESUMO

Noninvasive tests for the assessment of liver fibrosis are highly needed for the management of patients with autoimmune hepatitis (AIH). We aimed to investigate the accuracy of red cell distribution width to platelet ratio (RPR) in predicting liver fibrosis in AIH patients. One hundred nineteen AIH patients who underwent liver biopsy were enrolled. Liver fibrosis stage was diagnosed using the Scheuer scoring system. The diagnostic accuracy was evaluated by the area under the receiver operating characteristic curve (AUROC). RPR values in AIH patients with S2-S4 (0.10, interquartile range [IQR] 0.08-0.15), S3-S4 (0.10, IQR 0.09-0.14), and S4 (0.14, IQR 0.09-0.19) were significantly higher than patients with S0-S1 (0.07, IQR 0.06-0.08, P < .001), S0-S2 (0.08, IQR 0.06-0.12, P = .025) and S0-S3 (0.09, IQR 0.07-0.13, P = .014), respectively. The RPR was positively correlated with fibrosis stages (r = 0.412, P < .001), while aspartate transaminase to platelet ratio index (APRI) and fibrosis-4 score (FIB-4) were not significantly associated with fibrosis stages in AIH patients. The AUROCs of RPR in identifying significant fibrosis (S2-S4), advanced fibrosis (S3-S4), and cirrhosis (S4) were 0.780 (95% confidence interval [CI] 0.696-0.865), 0.639 (95% CI 0.530-0.748), and 0.724 (95% CI 0.570-0.878), respectively. The AUROCs of RPR were significantly higher than APRI and FIB-4 in diagnosing significant fibrosis, advanced fibrosis, and cirrhosis. Our study demonstrates that the RPR is a simple predictor of liver fibrosis and is superior to APRI and FIB-4 in identifying liver fibrosis in AIH patients.


Assuntos
Hepatite Autoimune/complicações , Cirrose Hepática/sangue , Índices de Eritrócitos , Feminino , Hepatite Autoimune/sangue , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/imunologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
9.
Am J Med Sci ; 360(3): 293-299, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32563569

RESUMO

Hyposplenism is associated with autoimmune diseases, inflammatory bowel disease, severe celiac disease, autoimmune thyroiditis, untreated HIV infection and chronic graft-versus-host disease. The aim of this study was to review the existing data on hyposplenism associated with celiac disease and Hashimoto's autoimmune thyroiditis. Our research was based on a clinical case concerning a 41-year-old female who presented with asthenia, fatigue, dyspepsia and chronic diarrhea. The medical history revealed autoimmune Hashimoto's thyroiditis, type 2 diabetes, fatty liver disease, chronic gastritis and thrombocytosis. Multiple investigations showed hyposplenism and complex autoimmune dysfunction with positive serum markers for celiac disease and type 1 autoimmune hepatitis along with minor symptomatology. The intestinal symptomatology of celiac disease is often hid by hypothyroidism-associated autoimmune thyroiditis. Asymptomatic or minimally symptomatic celiac disease associated with Hashimoto's autoimmune thyroiditis is diagnosed by biomarkers. Hyposplenism in celiac disease can occur regardless of the disease stage, latent or symptomatic.


Assuntos
Doença Celíaca/complicações , Doença de Hashimoto/complicações , Hepatite Autoimune/complicações , Baço/patologia , Adulto , Biópsia , Doença Celíaca/patologia , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/patologia , Duodeno/patologia , Fígado Gorduroso/complicações , Feminino , Gastrite/complicações , Doença de Hashimoto/patologia , Hepatite Autoimune/patologia , Humanos , Imagem por Ressonância Magnética , Trombocitose/complicações
10.
Gan To Kagaku Ryoho ; 47(6): 963-965, 2020 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-32541175

RESUMO

A 64-year-old female presented to our hospital with a chronic cough. She was diagnosed with cStage ⅢA small cell lung cancer(cT2aN2M0, limited disease). On admission for chemoradiation therapy, laboratory data incidentally revealed liver dysfunction. Further examination resulted in the patient being diagnosed with autoimmune hepatitis. Oral prednisolone therapy was started, and after the improvement of liver function tests, consecutive chemoradiation therapy with cisplatin and etoposide was administered. To the best of our knowledge, this is the first report of a patient with autoimmune hepatitis and small cell lung cancer. Autoimmune hepatitis might arise as a paraneoplastic syndrome.


Assuntos
Hepatite Autoimune , Neoplasias Pulmonares , Carcinoma de Pequenas Células do Pulmão , Feminino , Hepatite Autoimune/complicações , Humanos , Testes de Função Hepática , Neoplasias Pulmonares/complicações , Pessoa de Meia-Idade , Prednisolona , Carcinoma de Pequenas Células do Pulmão/complicações
11.
Eur J Gastroenterol Hepatol ; 32(9): 1251-1255, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32541245

RESUMO

Immunocompromised patients may be at increased risk to develop COVID-19 during the 2019 ß-coronavirus infection. We present the unique opportunity we had to monitor the liver, IL-6 and immune cell course before, during and after COVID-19 in a boy with autoimmune hepatitis (AIH) and type 1 diabetes (T1D). CD4 and CD8 T cells frequencies decreased because of prednisolone, followed by a plateauing increase whereas CD19CD20 B cell increased strongly and was unaffected by COVID-19 infection. Moreover, the percentage of activated CD8 T cells expressing HLA-DR (CD8HLA-DR) increased during COVID-19 and subsided after its clearance. Total regulatory T cells (Tregs: CD4CD25CD127FOXP3) remained stable. Although activated Tregs (CD4CD45RAFOXP3) strongly increased upon prednisolone, it decreased afterwards. Furthermore, regulatory B cells (Bregs: CD19CD20CD24CD38) declined sharply owing to prednisolone. Serum IL-6 remained undetectable at all times. We demonstrated for the first time immune monitoring in a child with AIH and T1D before, during and after COVID-19. We hypothesize that continuing with low level of prednisolone without azathioprine may have abrogated activated Tregs, Bregs and IL-6 production and therefore permitting the activation of CD8 T cells, clearing the virus.


Assuntos
Betacoronavirus , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Diabetes Mellitus Tipo 1/imunologia , Hepatite Autoimune/imunologia , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Linfócitos T CD4-Positivos , Linfócitos T CD8-Positivos , Pré-Escolar , Infecções por Coronavirus/complicações , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/terapia , Hepatite Autoimune/complicações , Hepatite Autoimune/terapia , Humanos , Contagem de Linfócitos , Masculino , Monitorização Imunológica , Pandemias , Pneumonia Viral/complicações
12.
Aliment Pharmacol Ther ; 51(12): 1417-1428, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32383181

RESUMO

BACKGROUND: The pathogenesis of autoimmune hepatitis (AIH) is poorly understood and little is known about enteric microbiota in AIH. AIM: To investigate disease-specific microbiome alterations in AIH. METHODS: The V1-V2 variable regions of the 16S rRNA gene were sequenced in faecal samples from 347 patients with AIH and controls (AIH n = 72, healthy controls (HC) n = 95, primary biliary cholangitis (PBC) n = 99 and ulcerative colitis (UC) n = 81). RESULTS: Biodiversity (Shannon entropy) was decreased in AIH patients compared to HC (P = 0.016), which was partially reversed by azathioprine (P = 0.011). Regarding between-sample diversity, AIH patients separated from HC, PBC and UC individuals (all P = 0.001). Compared to HC, decreased relative abundance of anaerobic genera such as Faecalibacterium and an increase of Veillonella and the facultative anaerobic genera Streptococcus and Lactobacillus were detected. Importantly, a disease-specific decline of relative abundance of Bifidobacterium was observed in AIH patients. Lack of Bifidobacterium was associated with failure to achieve remission of AIH (P < 0.001). Of potential therapeutic implication, Bifidobacterium abundance correlated with average protein intake (P < 0.001). Random forests classification between AIH and PBC on the microbiome signature yielded an area under receiver operating characteristic curve (AUC) of 0.787 in the training cohort, and an AUC of 0.849 in an external validation cohort. CONCLUSION: Disease-specific faecal microbial alterations were identified in patients with AIH. Intestinal dysbiosis in AIH was characterised by a decline of Bifidobacterium, which was associated with increased disease activity. These results point to the contribution of intestinal microbiota to AIH pathogenesis and to novel therapeutic targets.


Assuntos
Bifidobacterium/citologia , Disbiose/microbiologia , Microbioma Gastrointestinal , Hepatite Autoimune/microbiologia , Adulto , Idoso , Carga Bacteriana , Bifidobacterium/isolamento & purificação , Estudos de Casos e Controles , Estudos de Coortes , Disbiose/complicações , Fezes/microbiologia , Feminino , Hepatite Autoimune/complicações , Humanos , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/microbiologia , Masculino , Pessoa de Meia-Idade , RNA Ribossômico 16S/genética
13.
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
14.
Zhonghua Gan Zang Bing Za Zhi ; 28(4): 351-356, 2020 Apr 20.
Artigo em Chinês | MEDLINE | ID: mdl-32403889

RESUMO

Objective: To summarize the clinical diagnosis and therapeutic method in chronic hepatitis B (CHB) combined with autoimmune hepatitis (AIH). Methods: Clinical manifestations, laboratory examination, imaging, histopathological characteristics, treatment and prognosis of 19 cases diagnosed with CHB combined with AIH followed at the outpatient Department of Gastroenterology of Renji Hospital Affiliated to Shanghai Jiao Tong University School of Medicine during December 2013 to June 2018 were retrospectively analyzed. Paired sample t-test was used before and after treatment for the measurement of normal distribution data. Measurement data of non-normal distribution were expressed as a median (quartile spacing) and Wilcoxon matched-pairs signed rank test was used before and after treatment. Results: Among the 19 cases, 5 were male and 14 were female. The age of onset was 35 to 63 years, and the average age was 47.10 ± 8.76 years. There were 12 cases diagnosed with CHB before AIH, 5 cases diagnosed with AIH before CHB, and 2 cases diagnosed with AIH and CHB at the same time. After the definite diagnosis of CHB combined with AIH, nucleoside (acid) analogues (antiviral against hepatitis B virus) combined glucocorticoid therapy were given, and azathioprine or mycophenolate mofetil (immunosuppressant) was added according to the intrahepatic inflammation (inflammation graded at G3 and above) and leukocyte conditions. The duration of treatment varied between 2 weeks to 16 (median treatment duration of 6 weeks), except for one case who was just diagnosed and followed up. Biochemical indicators and immunoglobulin of the remaining 18 cases before and after treatment was significantly decreased, and the differences were statistically significant (P < 0.05), with HBV DNA < 20 copies/ml. Conclusion: CHB combined with AIH diagnosis can be easily missed. Therefore, it requires comprehensive diagnosis combined with clinical characteristics, autoantibodies, and immunoglobulin levels with special emphasis on pathological characteristics of liver tissue. Anti-HBc-positive patients using immunosuppressant should be carefully monitored for HBV DNA and anti-HBV treatment should be given if necessary.


Assuntos
Hepatite B Crônica/diagnóstico , Hepatite B Crônica/terapia , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/terapia , Adulto , China , Feminino , Vírus da Hepatite B , Hepatite B Crônica/complicações , Hepatite Autoimune/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
15.
BMJ Case Rep ; 13(2)2020 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-32029513

RESUMO

Statin-induced necrotising autoimmune myopathy (SINAM) is a rare disease characterised by proximal muscle weakness and elevated creatine kinase levels that is usually in the thousands. Anti-3-hydroxy-3-methyl glutaryl co-enzyme A reductase (HMGCR) antibodies are associated with SINAM. Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is usually of unknown aetiology but can also be associated with concurrent extrahepatic autoimmune disorders. We are reporting a case of biopsy proven AIH associated with SINAM in a patient presenting with oropharyngeal dysphagia. The patient had elevated anti-HMGCR antibodies and anti-smooth muscle antibodies. SINAM and AIH were confirmed by muscle biopsy and liver biopsy, respectively. The patient had complete resolution of his symptoms and complete normalisation of his liver function tests after 6 months of the treatment.


Assuntos
Doenças Autoimunes/diagnóstico , Transtornos de Deglutição/etiologia , Hepatite Autoimune/diagnóstico , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Miosite/diagnóstico , Doenças Autoimunes/induzido quimicamente , Hepatite Autoimune/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/induzido quimicamente
16.
Gut ; 69(3): 569-577, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31201284

RESUMO

OBJECTIVE: The significance of the liver-microbiome axis has been increasingly recognised as a major modulator of autoimmunity. The aim of this study was to take advantage of a large well-defined corticosteroids treatment-naïve group of patients with autoimmune hepatitis (AIH) to rigorously characterise gut dysbiosis compared with healthy controls. DESIGN: We performed a cross-sectional study of individuals with AIH (n=91) and matched healthy controls (n=98) by 16S rRNA gene sequencing. An independent cohort of 28 patients and 34 controls was analysed to validate the results. All the patients were collected before corticosteroids therapy. RESULTS: The gut microbiome of steroid treatment-naïve AIH was characterised with lower alpha-diversity (Shannon and observed operational taxonomic units, both p<0.01) and distinct overall microbial composition compared with healthy controls (p=0.002). Depletion of obligate anaerobes and expansion of potential pathobionts including Veillonella were associated with disease status. Of note, Veillonella dispar, the most strongly disease-associated taxa (p=8.85E-8), positively correlated with serum level of aspartate aminotransferase and liver inflammation. Furthermore, the combination of four patients with AIH-associated genera distinguished AIH from controls with an area under curves of approximately 0.8 in both exploration and validation cohorts. In addition, multiple predicted functional modules were altered in the AIH gut microbiome, including lipopolysaccharide biosynthesis as well as metabolism of amino acids that can be processed by bacteria to produce immunomodulatory metabolites. CONCLUSION: Our study establishes compositional and functional alterations of gut microbiome in AIH and suggests the potential for using gut microbiota as non-invasive biomarkers to assess disease activity.


Assuntos
Disbiose/complicações , Disbiose/microbiologia , Microbioma Gastrointestinal , Hepatite Autoimune/complicações , Hepatite Autoimune/microbiologia , Adolescente , Adulto , Idoso , Aspartato Aminotransferases/sangue , Estudos de Casos e Controles , Clostridiales , Estudos Transversais , Feminino , Hepatite Autoimune/sangue , Humanos , Lactobacillus , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Veillonella , Adulto Jovem
17.
Dig Dis Sci ; 65(1): 322-328, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31363952

RESUMO

BACKGROUND AND AIMS: Utilization and safety of cannabidiol (CBD) in patients with autoimmune hepatitis (AIH) are currently unknown. We aimed to identify the frequency of CBD use, impact on symptoms, and safety profile. METHODS: An invitation to complete a CBD-specific questionnaire was posted every other day to well-established autoimmune hepatitis Facebook communities (combined membership of 2600 individuals) during a 10-day study period. Age ≥ 18 years and an AIH diagnosis by a physician were the eligibility criteria for participation in the survey. RESULTS: In total, 371 AIH patients (median age 49 years, 32% reported advanced fibrosis) completed the questionnaire. Respondents were 91% women, 89% Caucasian, and 89% from North America. Ninety-three (25%) respondents were ever CBD users, with 55 of them (15% of the survey responders) identified as current users. Among ever users, 45.7% reported their treating doctors were aware of their CBD use. The most common reason cited for CBD use was pain (68%), poor sleep (62%), and fatigue (38%). Most respondents using CBD for these symptoms reported a significant improvement in pain (82%), sleep (87%), and fatigue (61%). In ever CBD users, 17.3% were able to stop a prescription medication because of CBD use: pain medication (47%), immunosuppression (24%), and sleep aids (12%). Side effects attributed to CBD use were reported in 3% of CBD users, yet there were no reported emergency department visits or hospitalizations. CONCLUSION: CBD use was not uncommon in patients with AIH, and its use was associated with reports of improvement in extrahepatic symptoms.


Assuntos
Canabidiol/administração & dosagem , Hepatite Autoimune/tratamento farmacológico , Automedicação , Adulto , Canabidiol/efeitos adversos , Estudos Transversais , Feminino , Pesquisas sobre Serviços de Saúde , Hepatite Autoimune/complicações , Hepatite Autoimune/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Segurança do Paciente , Medição de Risco , Automedicação/efeitos adversos , Mídias Sociais , Fatores de Tempo , Resultado do Tratamento
18.
J Coll Physicians Surg Pak ; 29(12): S106-S108, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31779758

RESUMO

Celiac disease (CD) is an autoimmune disorder with high incidence of multi organ involvement; especially, gastrointestinal manifestations and an increased risk of malignancies. Here we report a case of CD with celiac hepatitis, autoimmune hemolytic anemia (AIHA) and Grave's disease (GD) with their complications. Polyautoimmunity requires comprehensive analysis. While CD and GD were previously diagnosed, AIHA and cirrhosis were diagnosed during admission upon extensive work-up. Similarly, other autoimmune etiologies, such as autoimmune hepatitis (AIH), and/or primary biliary cholangitis were ruled out. All three diseases were treated afresh with strict adherence to a gluten-free diet (GFD) and carbimazole along with addition of medications for cirrhosis complicated by ascites. This was a rare case where non-adherence to a GFD led to such severe adverse events. A case of celiac hepatitis presenting with such a wide array of signs and symptoms has rarely been reported in the literature and the management of this patient was unique and challenging.


Assuntos
Anemia Hemolítica Autoimune/complicações , Autoimunidade , Carbimazol/uso terapêutico , Doença Celíaca/complicações , Dieta Livre de Glúten/métodos , Doença de Graves/complicações , Hepatite Autoimune/complicações , Anemia Hemolítica Autoimune/imunologia , Anemia Hemolítica Autoimune/terapia , Antitireóideos/uso terapêutico , Biópsia , Doença Celíaca/imunologia , Doença Celíaca/terapia , Feminino , Doença de Graves/imunologia , Doença de Graves/terapia , Hepatite Autoimune/imunologia , Hepatite Autoimune/terapia , Humanos , Adulto Jovem
19.
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
20.
Pediatr Neonatol ; 60(4): 396-404, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31409456

RESUMO

BACKGROUND: Current knowledge on the clinical features and natural history of childhood primary sclerosing cholangitis - inflammatory bowel disease in Asia is limited. We described the presenting features and natural history of primary sclerosing cholangitis-inflammatory bowel disease seen in a cohort of Southeast Asian children. METHODS: We conducted a retrospective review of childhood primary sclerosing cholangitis-inflammatory bowel disease from three tertiary centers in Singapore and Malaysia. RESULTS: Of 24 patients (boys, 58%; median age at diagnosis: 6.3 years) with primary sclerosing cholangitis-inflammatory bowel disease (ulcerative colitis, n = 21; Crohn's disease, n = 1; undifferentiated, n = 2), 63% (n = 15) were diagnosed during follow-up for colitis, and 21% (n = 5) presented with acute or chronic hepatitis, 17% (n = 4) presented simultaneously. Disease phenotype of liver involvement showed 79% had sclerosing cholangitis-autoimmune hepatitis overlap, 54% large duct disease, and 46% small duct disease. All patients received immunosuppression therapy. At final review after a median [±S.D.] duration follow-up of 4.7 [±3.8] years, 12.5% patients had normal liver enzymes, 75% persistent disease, and 12.5% liver failure. The proportion of patients with liver cirrhosis increased from 13% at diagnosis to 29%; 21% had portal hypertension, and 17% had liver dysfunction. One patient required liver transplant. Transplant-free survival was 95%. For colitis, 95% had pancolitis, 27% rectal sparing, and 11% backwash ileitis at initial presentation. At final review, 67% patients had quiescent bowel disease with immunosuppression. One patient who had UC with pancolitis which was diagnosed at 3 years old developed colorectal cancer at 22 years of age. All patients survived. CONCLUSIONS: Liver disease in primary sclerosing cholangitis-inflammatory bowel disease in Asian children has variable severity. With immunosuppression, two-thirds of patients have quiescent bowel disease but the majority have persistent cholangitis and progressive liver disease.


Assuntos
Colagogos e Coleréticos/uso terapêutico , Colangite Esclerosante/tratamento farmacológico , Hepatite Autoimune/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças Inflamatórias Intestinais/tratamento farmacológico , Cirrose Hepática Biliar/etiologia , Adolescente , Grupo com Ancestrais do Continente Asiático , Criança , Pré-Escolar , Colangite Esclerosante/complicações , Colangite Esclerosante/fisiopatologia , Estudos de Coortes , Colite Ulcerativa/complicações , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/fisiopatologia , Doença de Crohn/complicações , Doença de Crohn/tratamento farmacológico , Doença de Crohn/fisiopatologia , Progressão da Doença , Feminino , Hepatite Autoimune/complicações , Hepatite Autoimune/fisiopatologia , Humanos , Hipertensão Portal/etiologia , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/fisiopatologia , Hepatopatias/etiologia , Transplante de Fígado , Malásia , Masculino , Estudos Retrospectivos , Singapura , Adulto Jovem
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