Background/Purpose: Pancreatitis is uncommon and a life-threatening SLE manifestation in childhood-onset systemic lupus erythematosus (c-SLE) and it has been restricted to case reports or case series. Recently the International Study Group of Pediatric Pancreatitis (INSPPIRE) proposed definitions to standardize pancreatitis in children. However, studies using these new definitions in a large population of c-SLE patients were not performed. Therefore, the objective of the study was to systematically classify pancreatitis in c-SLE according to INSPPIRE and determine the overall prevalence, clinical features, laboratory and outcome of the first episode.

Methods: A retrospective multicenter study was performed in 852 cSLE patients from 10 Pediatric Rheumatology services of São Paulo state, Brazil. An investigator meeting was held to define the protocol, to harmonize clinical parameters definition, disease activity and damage tools scoring and outcome parameters. Demographic data, clinical, laboratorial, disease activity (SLEDAI-2K), cumulative damage (SLICC/ACR-DI), treatment and outcome were also evaluated.

Results: Pancreatitis was diagnosed in 22/852 (2.6%) cSLE patients. They were classified as: 20 (91%) acute pancreatitis, 2 (9%) acute recurrent pancreatitis and none had chronic pancreatitis. None of them had gallstone, traumatic pancreatitis or reported alcohol and tobacco use. The first pancreatitis episode was identified at disease onset in 6 (27%) cSLE patients. The comparison of patients with pancreatitis (first episode) and without this complication revealed higher median of SLEDAI-2K [21(0-41) vs. 2(0-45), p<0.0001]. The frequencies of fever (70% vs. 6%,p<0.0001), weight loss (50% vs. 3%,p<0.0001), hepatomegaly (36% vs. 2%,p<0.0001), splenomegaly (18% vs. 1%,p<0.0001), serositis (45% vs. 2%,p<0.0001), nephritis (75% vs. 20%,p<0.0001), arterial hypertension (59% vs. 12%,p<0.0001), acute renal failure (38% vs. 3%,p<0.0001), macrophage activation syndrome (36% vs. 0.5%, p<0.0001) and death (32% vs. 7%,p=0.001) were also higher in patients with pancreatitis. Frequencies of current methylprednisolone pulse (p<0.0001) and the median of current prednisone dose [55(15-60) vs. 11(1-90) mg/day, p<0.0001] were significantly higher in patients with pancreatitis, however no differences were observed of prednisone cumulative dose, intravenous methylprednisolone cumulative dose and total glucocorticoid cumulative dose in both groups (p>0.05). Of note, the two patients with acute recurrent pancreatitis had two episodes, with pain-free interval of 1 and 4 years.

Conclusion: This was the first study phenotyping pancreatitis according to INSPPIRE standardized definitions evidencing that this complication in c-SLE is predominantly an acute subtype with rare recurrence or progression to chronic damage. We also identified an association with current glucocorticoid use, disease activity and severity.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pancreatitis-subtypes-survey-in-852-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-cohort/