title: Macrophage activation syndrome: A severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients.
abstract: We previously reported a case series of acute pancreatitis (AP) and macrophage activation syndrome (MAS) in childhood (cSLE) patients; however, there are no data regarding the comparison of AP and MAS in large populations of cSLE and adult SLE (aSLE).
  
  
  
  A study included 362 cSLE and 1830 aSLE patients. MAS was diagnosed according to preliminary diagnostic guidelines and AP according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Demographic data, clinical features, SLEDAI-2K, SLICC/ACR-DI, and treatment were assessed.
  
  
  
  Age in MAS patients was significantly lower compared with those without this complication [15 (8.8-55) vs. 33.5 (10.2-45.7) years, p = 0.007]. The frequencies of fever (94% vs. 37%, 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 MAS compared in AP patients without MAS. Fever and hyperferritinemia concomitantly were more frequent in the former group (86% vs. 12%, p = 0.0015). Higher and significant frequency of AP in cSLE compared to aSLE patients [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 (85% vs. 30%, p = 0.003) and death by MAS complication (31% vs. 0%, p = 0.017) were significantly higher in children compared with aSLE.
  
  
  
  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.
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil; Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Natali W S
authors.initials: NW
authors.lastname: Gormezano
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Carini I
authors.initials: CI
authors.lastname: Otsuzi
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Diego L
authors.initials: DL
authors.lastname: Barros
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Mariana A
authors.initials: MA
authors.lastname: da Silva
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Rosa M R
authors.initials: RM
authors.lastname: Pereira
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Lucia M A
authors.initials: LM
authors.lastname: Campos
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Eduardo F
authors.initials: EF
authors.lastname: Borba
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, no. 455, 3 andar, sala 3190-Cerqueira César, São Paulo 05403-010, Brazil; Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. Electronic address: clovisaasilva@gmail.com.
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D002648:Child; D005260:Female; D005293:Ferritins / Q000097:blood; D005334:Fever / Q000209:etiology; D006801:Humans; D015228:Hypertriglyceridemia / Q000209:etiology; D007970:Leukopenia / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000503:physiopathology*; D055501:Macrophage Activation Syndrome / Q000097:blood / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000097:blood / Q000150:complications / Q000503:physiopathology*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013921:Thrombocytopenia / Q000209:etiology; D055815:Young Adult
chemical_list: D005293:Ferritins
keywords: Childhood systemic lupus erythematosus; Corticosteroid; Immunosuppressive; Macrophage activation syndrome; Pancreatitis