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

Advances in Rheumatology

Eloisa Bonfá¹  Rosa M. R. Pereira¹  Nadia E. Aikawa¹  Clovis A. Silva²  Juliana C. O. Ferreira²  Kátia Kozu²  Lucia M. Campos²  Adriana M. Sallum²  Verena A. Balbi²  Sylvia C. Farhat²  Laila P. Coelho²  Ana R. Schmidt²  Bárbara Montenegro²  Ana C. Pitta²  Virginia P. Ferriani³  Claudia Saad-Magalhães⁴  Daniela P. Piotto⁵  Ana P. Sakamoto⁵  Maria T. Terreri⁵ 

[1] Division of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo;Pediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo;Pediatric Rheumatology Unit, Ribeirão Preto Medical School, University of São Paulo;Pediatric Rheumatology Unit, São Paulo State University (UNESP) – Faculdade de Medicina de Botucatu;Pediatric Rheumatology Unit, Universidade Federal de São Paulo;

关键词: Autoimmune hepatitis;    Childhood systemic lupus erythematosus;    Hepatomegaly;    Multicenter study;   

DOI  :  10.1186/s42358-018-0043-7

来源: DOAJ

【 摘 要 】

Abstract Objective To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods This 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). Results AIH 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). Conclusions Overlap 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.

【 授权许可】

Unknown