Autoimmune hepatitis — Primary biliary cholangitis overlap syndrome. Long-term outcomes of a retrospective cohort in a university hospital

Casas, Omar Yesid Martínez; Ramírez, Gabriel Sebastián Díaz; Zuluaga, Juan Ignacio Marín; Santos, Óscar; Maya, Octavio Muñoz; Gómez, Jorge Hernando Donado; Gutiérrez, Juan Carlos Restrepo

doi:10.1016/j.gastre.2018.11.006

Cited by 8 publications

(6 citation statements)

References 16 publications

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“…There were notable studies of each overlap syndrome that did not meet inclusion criteria for our systematic review. The absence of ≥2 distinct treatment groups [2,3,5,18,21,32,34,[36][37][38]40,42,76] or the failure to stratify clinical outcomes by treatment group [9,19,20,46,58] were the most common reasons for exclusion. Three studies examining the impact of MMF as second-line therapy for AIH-PBC [16], AIH-PSC [15], AIC [15], and ASC [15,45] were also excluded.…”

Section: Resultsmentioning

confidence: 99%

“…UDCA has been augmented with tacrolimus [10], mycophenolate mofetil (MMF) [15,16], or cyclosporine [8,10,17] as second line therapies, with some success, in patients intolerant of or unresponsive to corticosteroids and/or AZA. At 10 years, progression to cirrhosis among patients with AIH-PBC is approximately 44-48% [18,19], and transplant-free survival is 52-92% [19][20][21].…”

Section: Introductionmentioning

confidence: 99%

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Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis

Freedman

Danford

Patwardhan

et al. 2020

JCM

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The treatment of overlap syndromes is guided by small observational studies whose data have never been synthesized in a rigorous, quantitative manner. We conducted a systematic review and meta-analysis to evaluate the efficacy of available treatments for these rare and morbid conditions. We searched the literature for studies comparing ≥2 therapies for autoimmune hepatitis (AIH)-primary biliary cholangitis (PBC), AIH-primary sclerosing cholangitis (PSC), PBC-PSC, AIH-PBC-PSC, autoimmune cholangitis (AIC), or autoimmune sclerosing cholangitis (ASC) with respect to various clinical outcomes, including biochemical improvement and transplant-free survival. A total of 28 studies met the inclusion criteria for AIH-PBC, AIH-PSC, AIC, and ASC. AIH-PBC patients tended to experience more biochemical improvement with ursodeoxycholic acid (UDCA) + [corticosteroids and/or antimetabolites], i.e., “combination therapy”, than with corticosteroids ± azathioprine (RR = 4.00, 95% CI 0.93–17.18). AIH-PBC patients had higher transplant-free survival with combination therapy than with UDCA, but only when studies with follow-up periods ≤90 months were excluded (RR = 6.50, 95% CI 1.47–28.83). Combination therapy may therefore be superior to both UDCA and corticosteroids ± azathioprine for the treatment of AIH-PBC, but additional studies are needed to show this definitively and to elucidate optimal treatments for other overlap syndromes.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis

Freedman

Danford

Patwardhan

et al. 2020

JCM

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“…Martínez Casas et al (26) reported that adult patients with AIH-PBC had greater progression to cirrhosis (22.2 vs. 13.1%, p = 0.038), even in those who achieved partial or complete biochemical remission without relapse. Also these adult patients had greater indication of orthotopic liver transplantation ( p = 0.009), but no differences in mortality.…”

Section: Discussionmentioning

confidence: 99%

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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“…Overlap syndrome refers to cases that meet the diagnostic criteria for AIH and diagnostic criteria of other autoimmune liver diseases, mainly PBC or PSC. AIH-PBC overlap syndrome has a higher risk of cirrhosis complications than AIH alone or PBC alone [ 324 - 326 ]. In a Korean study, AIH-PBC overlap syndrome showed poorer clinical outcomes compared to AIH alone or PBC alone [ 50 ].…”

Section: Treatmentmentioning

confidence: 99%

KASL clinical practice guidelines for management of autoimmune hepatitis 2022

2023

Clin Mol Hepatol

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Autoimmune hepatitis — Primary biliary cholangitis overlap syndrome. Long-term outcomes of a retrospective cohort in a university hospital

Cited by 8 publications

References 16 publications

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis

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

KASL clinical practice guidelines for management of autoimmune hepatitis 2022

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