title: Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients.
abstract: OBJECTIVE
  Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes.
  
  
  METHODS
  A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE.
  
  
  RESULTS
  Pancreatitis was diagnosed in 22 of 852 (2.6%) patients with cSLE. It was classified as acute pancreatitis in 20 (91%), acute recurrent pancreatitis in 2 (9%), and none of them had chronic pancreatitis. None of them had gallstones, traumatic pancreatitis, or reported alcohol/tobacco use. The comparison of patients with pancreatitis (first episode) and without this complication revealed a shorter disease duration (1 [0-10] vs 4 [0-23] years, P < 0.0001) and higher median of Systemic Lupus Erythematosus Disease Activity Index 2000 (21 [0-41] vs 2 [0-45], P < 0.0001). The frequencies of fever (P < 0.0001), weight loss (P < 0.0001), serositis (P < 0.0001), nephritis (P < 0.0001), arterial hypertension (P < 0.0001), acute renal failure (P < 0.0001), macrophage activation syndrome (P < 0.0001), and death (P = 0.001) were also higher in patients with pancreatitis. The frequencies of intravenous methylprednisolone use (P < 0.0001) and the median of prednisone dose (55 [15-60] vs 11 [1-90] mg/day, P < 0.0001) were significantly higher in patients with pancreatitis. Of note, the 2 patients with acute recurrent pancreatitis had 2 episodes, with pain-free interval of 1 and 4 years.
  
  
  CONCLUSIONS
  This was the first study characterizing pancreatitis using the International Study Group of Pediatric Pancreatitis standardized definitions in patients with cSLE showing that the predominant form is acute pancreatitis seen in association with glucocorticoid treatment and active severe disease.
authors.affiliation: *Pediatric Rheumatology Unit †Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo ‡Pediatric Rheumatology Unit, Universidade Federal de São Paulo §São Paulo State University (UNESP)-Faculdade de Medicina de Botucatu ||Irmandade da Santa Casa de Misericórdia de São Paulo ¶State University of Campinas #Ribeirão Preto Medical School, University of São Paulo **Hospital Infantil Darcy Vargas ††Pontifical Catholic University of Sorocaba ‡‡Hospital Municipal Infantil Menino Jesus, São Paulo, Brazil.
authors.forename: Victor L S
authors.initials: VL
authors.lastname: Marques
authors.forename: Natali W S
authors.initials: NW
authors.lastname: Gormezano
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.forename: Nadia E
authors.initials: NE
authors.lastname: Aikawa
authors.forename: Maria T
authors.initials: MT
authors.lastname: Terreri
authors.forename: Rosa M
authors.initials: RM
authors.lastname: Pereira
authors.forename: Claudia S
authors.initials: CS
authors.lastname: Magalhães
authors.forename: Andressa
authors.initials: A
authors.lastname: Guariento
authors.forename: Simone
authors.initials: S
authors.lastname: Appenzeller
authors.forename: Virgínia P
authors.initials: VP
authors.lastname: Ferriani
authors.forename: Cássia M
authors.initials: CM
authors.lastname: Barbosa
authors.forename: Valéria C
authors.initials: VC
authors.lastname: Ramos
authors.forename: Simone
authors.initials: S
authors.lastname: Lotufo
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000208:Acute Disease; D058186:Acute Kidney Injury / Q000209:etiology; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005334:Fever / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D008262:Macrophage Activation; D008297:Male; D009393:Nephritis / Q000209:etiology; D010195:Pancreatitis / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence; D012700:Serositis / Q000209:etiology; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D015431:Weight Loss; D055815:Young Adult