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

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.

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

“…The reveal prevalence of overlapping SLE and AIH still remained unknown. Balbi et al (15) reported in multicenter cohort study that AIH in pediatric SLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at SLE diagnosis [5/7 (71%)].…”

Neonatal Lupus presenting with neonatal hemochromatosis-like liver disease that responded to steroids: a case report

“…[19,28,29] SMA are typically of IgG subtype and thus can theoretically cross the placenta just like SSA/SSB, however, only 20-60% of NLE mothers have a history of an autoimmune disorder, mainly lupus, which can rarely be associated with AIH-1. [5,30] Thus the finding from our case deserves to be highlighted. Nevertheless, we believe that SMA presence in our patient was due to a false-positive reaction rather than a true early onset AIH-1, especially given the patient's complete resolution of liver injury while being off-immunosuppression.…”

Diagnostic Dilemma of ANA-negative Pediatric Systemic Lupus Erythematosus in a South Asian Female

“…Some of these children will go on to develop SLE, others will develop other rheumatic diseases, and some will remain asymptomatic. 10 SLE typically presents with a variety of complications, such as vascular, cardiac, nervous system, pulmonary, and renal involvement, as well as positive ANA and or patients with seronegative ANA tests have a higher prevalence of positive Ro factor antibody. However, there were no further systemic symptoms or the presence of these antibodies in our patient.…”

“…Some of these children will go on to develop SLE, others will develop other rheumatic diseases, and some will remain asymptomatic. 10 …”