Autoimmune hepatitis and juvenile systemic lupus erythematosus

Area of Science:

• Pediatric Rheumatology
• Pediatric Hepatology
• Autoimmune Diseases

Background:

• Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are rare pediatric autoimmune disorders.
• Co-occurrence of JSLE and AIH is infrequently reported, necessitating further investigation into their association.

Observation:

• This study evaluated the simultaneous prevalence of JSLE and AIH in a large pediatric cohort over 24 years.
• Four pediatric patients diagnosed with both JSLE and AIH were analyzed for demographic, clinical, and laboratory features.
• AIH diagnosis preceded JSLE in three of the four cases.

Findings:

• The prevalence of AIH-JSLE association was found to be 1.8% in the JSLE population and 1.6% in the AIH population.
• All affected patients presented with elevated liver enzymes, characteristic AIH liver biopsy findings, and responded to combined immunosuppressive therapy.
• The median age of affected patients was 15.5 years, with a female predominance.

Implications:

• The co-occurrence of AIH and JSLE is rare but warrants consideration in pediatric autoimmune care.
• Liver biopsy may be indicated for JSLE patients with persistent elevation of liver enzymes to rule out concurrent AIH.
• Early diagnosis and combined treatment strategies are crucial for managing patients with both AIH and JSLE.