Objectives To summarize and analyze the clinical manifestations of acute pancreatitis associated with childhood systemic lupus erythematosus (cSLE) and to evaluate the therapeutic efficacy of plasma exchange combined with glucocorticoids.

Methods This retrospective study analyzed clinical data from cSLE patients diagnosed and treated at West China Second University Hospital from January 2018 to December 2020. Patients were divided into a pancreatitis group (diagnosed with cSLE-associated acute pancreatitis) and a non-pancreatitis group (without this diagnosis), and differences between groups were compared.

Results Among 170 cSLE patients enrolled, 9 were in the pancreatitis group and 161 were in the non-pancreatitis group. The median SLEDAI score was 24.0 (P25-P75: 20.5-29.0) in the pancreatitis group and 18.0 (P25-P75: 14.0-20.0) in the non-pancreatitis group, with a statistically significant difference (P < 0.05). The incidence rates of abdominal pain, vomiting, abdominal distension, diarrhea, acute pericarditis, and macrophage activation syndrome were higher in the pancreatitis group than in the non-pancreatitis group, with statistically significant differences (P < 0.05). Differences in C-reactive protein, hemoglobin, blood urea nitrogen, serum albumin, serum amylase, serum lipase, and lipid levels between the two groups were also statistically significant (P < 0.05). Multivariate logistic regression analysis indicated that each 1-point increase in SLEDAI score was associated with a 37.5% higher risk of developing pancreatitis (OR 1.375, 95% CI 1.121-1.686, P =0.002). All children in the pancreatitis group received high-dose glucocorticoids combined with multiple plasma exchanges, followed by immunosuppressive therapy. Nine patients achieved remission from acute pancreatitis within two weeks of treatment. Two patients died within 30 days, but the cause of death was unrelated to acute pancreatitis.

Conclusion Acute pancreatitis is a rare but severe complication of cSLE, with higher SLEDAI scores correlating with increased risk of pancreatitis. Early diagnosis and aggressive treatment with glucocorticoids combined with plasma exchange may be associated with improved short-term outcomes.