Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes

Porta, Gilda; Carvalho, Elisa de; Santos, Jorge Luiz dos; Gama, Jorge; Borges, Cristian V.; Seixas, Renata Belém Pessôa de Melo; Ferreira, Alexandre Rodrígues; Miura, Irene Kazue; Silveira, Themis Reverbel da; Silva, Luciana Rodrígues; Fagundes, Eleonora Druve Tavares; Bellomo-Brandão, Maria Ângela; Sawamura, Regina; Vieira, S; Melere, Melina Utz; Pugliese, Renata; Danesi, Vera Lúcia Baggio; Porta, Adriana; Marsillac, M. E. De; Valladares, Marcia A.; Menezes, Daniela G.; Kieling, Carlos Oscar; Paula, Mariana Nascimento de; Vasconcelos, Juliana R.; Ferreira, Cristina Targa; Perin, Nilza Medeiros; Resende, Leonardo R.; Maia, Jussara Melo de Cerqueira; Tommaso, Adriana María Alves De; Hessel, Gabriel

doi:10.1016/j.jpedp.2018.06.011

Cited by 6 publications

(15 citation statements)

References 24 publications

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“…On the other hand, when comparing patients from a Southeastern Brazilian center with a North American population, it was observed that Brazilian patients had earlier disease onset, lower frequency of concurrent autoimmune diseases, higher serum levels of aspartate aminotransferase and gammaglobulin, higher frequency of smooth muscle antibodies and lower frequency of antinuclear antibodies than the patients from the United States cohort (14) . Given the genetic diversity of patients with AIH in Latin America (12)(13)(14)(15)(16)(17) , we believe it was also important to study a population in the South of Brazil, especially considering that population in this region is mainly of European ethnicity (18) .…”

Section: Evaluation Of Patients With Autoimmune Hepatitis In a Speciamentioning

confidence: 99%

Evaluation of Patients With Autoimmune Hepatitis in a Specialized Outpatient Clinic in Southern Brazil

Fedrizzi

Coral

Mattos

et al. 2020

Arq. Gastroenterol.

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BACKGROUND: Autoimmune hepatitis (AIH) is a chronic liver disease, characterized by necroinflammation and autoimmune etiology. Studies evaluating the characteristics of patients with AIH are scarce in Brazil. OBJECTIVE: Our objective was to evaluate the profile of patients with AIH in a specialized center in Southern Brazil and to verify factors related to treatment response. METHODS: this was a retrospective cohort study, which analyzed demographic, epidemiological, clinical, laboratory, and histologic data. Patients with AIH diagnosed according to the criteria of the International Autoimmune Hepatitis Group (IAIHG) were included. In liver biopsies, the degree of fibrosis, histological activity, presence of hepatocyte rosettes, plasma cell infiltrates, and confluent necrosis were evaluated. In the statistical analysis, the significance level was 5%. RESULTS: Forty adults patients diagnosed with AIH were included. The evaluated population predominantly consisted of women (75.0%) and the average age at diagnosis was 44.2 years. The association with extrahepatic autoimmune diseases occurred in 20.0% of cases. Clinically, 35.0% of patients presented with acute onset hepatitis, 37.5% with cirrhosis, and 27.5% with other forms of presentation. The most common clinical manifestation was jaundice (47.5%). Thirty-five patients were treated, and of these, 97.1% used prednisone combined with azathioprine. The average treatment time was 2.7 years. Response to treatment was complete or partial in 30 (85.7%) and absent in 5 (14.3%) patients. There was no statistically significant difference when evaluating response to treatment in relation to forms of presentation, histological findings, and the presence of autoantibodies. Regarding fibrosis, regression was observed in 18.75% of the cases. CONCLUSION: Most patients with AIH were young at presentation and of female sex. The association with extrahepatic autoimmune diseases and cirrhosis at presentation was seen in a considerable proportion of patients. Treatment was effective, but there were no clinical, histological or serological parameters capable of predicting treatment response.

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Section: Evaluation Of Patients With Autoimmune Hepatitis In a Speciamentioning

confidence: 99%

Evaluation of Patients With Autoimmune Hepatitis in a Specialized Outpatient Clinic in Southern Brazil

Fedrizzi

Coral

Mattos

et al. 2020

Arq. Gastroenterol.

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“…The AHRQ score of each study was presented in Table 1 and Supplemental Digital Content (Appendix 3, http:// links.lww.com/MD/D462) (Table ), which illustrates the detailed AHRQ score. Ten studies [23][24][25][26][27][28][29][30][31][32] were of high quality and 15 studies [6,7,[33][34][35][36][37][38][39][40][41][42][43][44][45] were of moderate quality. There were no studies with low quality.…”

Section: Methodological Quality Assessmentmentioning

confidence: 99%

The efficacy and safety of different doses of glucocorticoid for autoimmune hepatitis

Zhang

Dong

et al. 2019

Medicine

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Background: Glucocorticoid as the standard treatment of autoimmune hepatitis has been recommended with different doses. The purpose of this study is to compare the efficacy and safety of high and low doses for clinical practice.Methods: Medline, Embase, and Cochrane Library were searched until January 16th, 2019 for cohort studies or randomized controlled trials in patients with autoimmune hepatitis. Glucocorticoid 60 mg/d or 1 mg/kg/d was defined as high dose and 40 to 50 mg/d or 0.5 mg/d as low dose. Outcome of interests includes the incidence of the biochemical remission, adverse event, and endpoint events. Double arcsine method with a random-effect model was used to combine the incidence. Potential heterogeneity was explored by meta-regression and subgroup analysis.Results: Overall, 25 studies (3305 patients) were included, with 10 studies in the high dose group and 15 in low dose group. The biochemical remission rate in the high and low dose group was 0.79 (95% confidence interval [CI] [0.72, 0.85]) and 0.72 (95% CI [0.65, 0.78]), respectively. The incidence of endpoint events and adverse event in the high were slightly higher (0.03, 95% CI [0.02, 0.04]; 0.42, 95% CI [0.30, 0.53]) than that of the low dose group (0.01, 95% CI [0.00, 0.01]; 0.39, 95% CI [0.15, 0.63]).Conclusions: For autoimmune hepatitis patients, 60 mg/d or 1 mg/kg/d of glucocorticoid gives higher biochemical remission rate and higher incidence of endpoint events and adverse events.Abbreviations: AASLD = American Association for the Study of Liver Diseases, AHRQ = Agency for Healthcare Research and Quality, AIH = autoimmune hepatitis, ALT = alanine aminotransferase, CI = confidence interval, EASL = European Association for the Study of the Liver.

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“…Acute hepatitis is the most common presentation (56-58%), with up to 10% in ALF. 11 About 10% will present with complications of cirrhosis and portal hypertension without any prior history of jaundice or liver disease. 12 Up to 20% may have other autoimmune conditions including thyroiditis, vitiligo, type 1 diabetes, inflammatory bowel disease (IBD), coeliac disease and nephrotic syndrome, and 40% will have a family history of autoimmune disease.…”

Section: Autoimmune Liver Disease (Aih/psc)mentioning

confidence: 99%

“…The spectrum of presentation in AIH varies from incidentally noted abnormal liver functions tests, to acute hepatitis and acute liver failure (ALF), to cirrhosis and portal hypertension. Acute hepatitis is the most common presentation (56–58%), with up to 10% in ALF 11. About 10% will present with complications of cirrhosis and portal hypertension without any prior history of jaundice or liver disease 12.…”

Section: Acute Hepatitismentioning

confidence: 99%

Liver disease in the older child

2020

J Paediatrics Child Health

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Liver disease in children tends to present either as: (i) an acute hepatitis with or without jaundice; (ii) incidental finding of abnormal liver function tests; or (iii) from a complication of portal hypertension with either haematemesis and/or incidental splenomegaly. Acute hepatitis may result from acute infection, prescribed or other drugs, ischaemia or vascular causes, autoimmune hepatitis, or idiopathic liver failure. Non‐alcoholic fatty liver disease is now the most likely reason for abnormal liver function tests but medications, metabolic disease, cholangiopathy and non‐liver causes should be considered. Autoimmune hepatitis and alpha‐1‐antitrypsin deficiency are the most likely causes of insidious liver disease. An international normalised ratio uncorrected by vitamin K reflects the severity of liver synthetic dysfunction, but not propensity to bleed. Creatine kinase helps to differentiate muscle from liver disease in patients with raised transaminases. Doppler ultrasound of hepatic vasculature is useful when assessing splenomegaly to differentiate extra‐hepatic portal hypertension from inherent liver disease.

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Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes

Cited by 6 publications

References 24 publications

Evaluation of Patients With Autoimmune Hepatitis in a Specialized Outpatient Clinic in Southern Brazil

Evaluation of Patients With Autoimmune Hepatitis in a Specialized Outpatient Clinic in Southern Brazil

The efficacy and safety of different doses of glucocorticoid for autoimmune hepatitis

Liver disease in the older child

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