Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome

Rodrigues, Adriana Teixeira; Liu, Priscila M.F.; Fagundes, Eleonora Druve Tavares; Queiroz, Thaís Costa Nascentes; Barbosa, Pamela de Souza Haueisen; Silva, Soraya L.C.; Silva, Ana Cristina Simões e; Miranda, Débora Marques de; Ferreira, Alexandre Rodrígues; Alberti, Luiz Ronaldo

doi:10.1097/mpg.0000000000001125

Cited by 39 publications

(26 citation statements)

References 28 publications

(70 reference statements)

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“…Also these adult patients had greater indication of orthotopic liver transplantation ( p = 0.009), but no differences in mortality. However, Rodrigues et al (27) reported statistically significant difference of prognosis and response to treatment was observed between the pediatric AIH and ASC groups.…”

Section: Discussionmentioning

confidence: 95%

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

et al. 2019

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Background: The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) is not easily established because of its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is usually used in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few cases of AIH complicated by SLE have been reported in the literature, and the condition is even rarer in childhood. Case presentation: Here we report the case of a 16-year-old girl with SLE who initially presented with autoimmune (cholestatic) hepatitis. According to American Association for the Study of Liver Diseases practice guidelines, the diagnosis was made based on aggregated scores including female (+2); ALP:AST (or ALT) ratio <1.5(+2); elevated serum IgG level(+3); ANA > 1:80 (+3); negative hepatitis viral markers and drug history (+3, +1); average alcohol intake <25 g/day (+2); and histological interface hepatitis features (+3). She then developed a malar rash, ANA positivity, anti-double-stranded DNA (anti-dsDNA) antibodies, and a low complement level. She met 4 of 17 Systemic Lupus International Collaborating Clinics classification criteria ( 1 ) for SLE. Our patient responded very well to corticosteroid at an initial dose of methylprednisolone 40 mg Q12H for 4 days tapering to 1 mg/kg/day according to liver function test results and bilirubin level. No relapse occurred during the 3-year follow-up course. Conclusions: Overlapping of SLE and AIH should be suspected when children with SLE have impaired liver function or AIH patients present with a malar or other skin rash. Liver biopsy plays an important role in establishing the differential diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate further treatment plans can improve disease outcomes.

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Section: Discussionmentioning

confidence: 95%

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

et al. 2019

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“…that, it is important separate PSC-AIH overlap with features of both diseases that is termed autoimmune sclerosing cholangitis (ASC) (2,4,5,15) . Separation between patients with isolated PSC and those with ASC is relevant because their prognosis is different due to response to immunosuppressants in ASC, unlike isolated PSC (8,12) . Differentiation between the two isn't always clear and progression of AIH to ASC has been documented in children in particular (8) .…”

Section: Discussionmentioning

confidence: 99%

Primary Sclerosing Cholangitis in Children and Adolescents

Fagundes

Ferreira

Hosken

et al. 2017

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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Clinical Characteristics and Prognosis in Children and Adolescents With Autoimmune Hepatitis and Overlap Syndrome

Abstract: No statistically significant difference was observed between the groups in relation to prognosis and response to treatment.

Cited by 39 publications

References 28 publications

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review

Primary Sclerosing Cholangitis in Children and Adolescents

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

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