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

Advances in Rheumatology

Eloisa Bonfá¹  Clovis A. Silva²  Ká²  Claudia Saad-Magalhã³  Verena A. Balbi³  Ana R. Schmidt³  Nadia E. Aikawa³  Laila P. Coelho³  rbara Montenegro³  Rosa M. R. Pereira³  Virginia P. Ferriani⁵  tia Kozu⁶  Daniela P. Piotto^{7  8}  Adriana M. Sallum⁸  Lucia M. Campos⁹  Ana P. Sakamoto^1,11  Juliana C. O. Ferreira^1,11  es^1,11  Sylvia C. Farhat^1,11  Maria T. Terreri^1,11  Ana C. Pitta^1,11  Bá^1,11 

[1] Faculdade de Medicina de Botucatu, SãDivision of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil;Pediatric Rheumatology Unit, Children’Pediatric Rheumatology Unit, RibeirãPediatric Rheumatology Unit, SãPediatric Rheumatology Unit, Universidade Federal de São Paulo State University (UNESP) –o Paulo, Brazil;o Paulo, São Preto Medical School, University of Sãs Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil

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

DOI  :  10.1186/s42358-018-0043-7

学科分类：过敏症与临床免疫学

来源: Springer

【 摘 要 】

To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. 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). 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). 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】

CC BY