Background/Purpose: Acute pancreatitis (AP) is a rare and severe manifestation of childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) patients. Macrophage activation syndrome (MAS) is reported in lupus patients, however the comparisons of specific clinical and laboratorial features of MAS in cSLE and aSLE populations with AP were not performed. Therefore, the aim of this study was to analyze in a large population of cSLE and aSLE patients the rare group of AP and MAS. 

Methods: A retrospective study included 362 cSLE and 1,830 aSLE patients followed in the same University Hospital. AP was defined according to the presence of abdominal pain or vomiting associated with an increase of pancreatic enzymes (3X) and/or pancreatic radiological abnormalities. MAS was diagnosed according to preliminary diagnostic guidelines, requiring the presence of at least one clinical and two laboratorial criteria. Bone marrow aspirate to assess macrophage hemophagocytosis was evaluated when available. Demographic data, clinical features, SLEDAI-2K, SLICC/ACR-DI and treatment were also assessed.

Results: A higher frequency of AP was observed in cSLE compared to aSLE [12/362 (3.3%) vs. 20/1830 (1.1%), p=0.003], with similar AP duration [22(6-60) vs. 15(4-90) days, p=0.534]. MAS was significantly higher in the former group (85% vs. 30%, p=0.003) and four of them had macrophage hemophagocytosis. cSLE patients had higher SLEDAI-2K at AP diagnosis [22(8-41) vs. 10(0-40), p=0.007], fever (p=0.002), anti-dsDNA antibodies (p=0.002) and death by MAS complication (31% vs. 0%, p=0.017) than aSLE. No differences were evidenced in glucocorticoid use in both groups (p=0.394). Further analysis of MAS patients showed that the median of ferritin [1804(28-24,511) vs. 409(25-4,282) ng/ml, p=0.041], aspartate aminotransferase (AST) [121(23-1,156) vs. 30(13-1,446) U/L, p=0.018] and triglyceride [285 (163-526) vs. 172(61-357) mg/dL, p=0.005] were significantly higher in AP patients with MAS compared those without this complication. Fever (94% vs. 38%, p=0.001), leucopenia (82% vs. 19%, p=0.0001), thrombocytopenia (65% vs. 19%, p=0.013), hypertriglyceridemia (87% vs. 42%, p=0.037) and hyperferritinemia (93% vs. 37%, p=0.011) were also more frequently observed in AP patients with versus without MAS. Of note, acute infections were alike in both groups (p=0.438).

Conclusion: This study provides novel data demonstrating that MAS occur in the majority of cSLE with AP with a higher mortality compared to aSLE. In addition, we identified in AP patients, a cluster of MAS clinical and laboratorial parameters more associated with this complication.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/macrophage-activation-macrophage-activation-syndrome-a-severe-and-frequent-manifestation-of-acute-pancreatitis-in-childhood-onset-compared-to-adult-systemic-lupus-erythematosus-patients/