Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study

Verena A Balbi,Kátia Kozu,Eloisa Bonfá,Maria T Terreri,Juliana C O Ferreira,Bárbara Montenegro,Nadia E Aikawa,Clovis A Silva,Ana R Schmidt,Laila P Coelho,Adriana M Sallum,Sylvia C Farhat,Virginia P Ferriani,Rosa M R Pereira,Daniela P Piotto,Ana C Pitta,Claudia Saad-Magalhães,Lucia M Campos,Ana P Sakamoto

doi:10.1186/s42358-018-0043-7

Abstract

ObjectiveTo evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients.MethodsThis retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni’s correction (p < 0.0033).ResultsAIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0–8.5) vs. 0.12 (0–8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0–26) vs. 7 (0–41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033).ConclusionsOverlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

Highlights

Childhood-onset systemic lupus erythematosus is an autoimmune and inflammatory disease that affects multiple organs and systems, including liver [1,2,3].Of note, autoimmune hepatitis (AIH) is characterized by elevated hepatic enzymes, hypergammaglobulinemia, presence of autoantibodies and liver histology abnormalities, interface hepatitis and lymphocytic infiltrates [4, 5]
To our knowledge the prevalence of overlap AIH and Childhood-onset systemic lupus erythematosus (cSLE) in a large population was not studied and analysis of this very rare association is restricted to few case reports or case series [1, 3, 6, 7]
This study was conducted in 10 Pediatric Rheumatology services in the state of São Paulo, Brazil including a population of 847 cSLE patients [8]

Summary

Introduction

Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune and inflammatory disease that affects multiple organs and systems, including liver [1,2,3]. Autoimmune hepatitis (AIH) is characterized by elevated hepatic enzymes, hypergammaglobulinemia, presence of autoantibodies and liver histology abnormalities, interface hepatitis and lymphocytic infiltrates [4, 5]. To our knowledge the prevalence of overlap AIH and cSLE in a large population was not studied and analysis of this very rare association is restricted to few case reports or case series [1, 3, 6, 7]. The objective of this multicenter cohort study was to evaluate cSLE and AIH and the possible association with demographic data, cumulative clinical manifestations, treatments and outcomes in a large cSLE population in Latin America

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Results

Conclusion