Marques Victor L S, Gormezano Natali W S, Bonfá Eloisa, Aikawa Nadia E, Terreri Maria T, Pereira Rosa M, Magalhães Claudia S, Guariento Andressa, Appenzeller Simone, Ferriani Virgínia P, Barbosa Cássia M, Ramos Valéria C, Lotufo Simone, Silva Clovis A
*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.
J Pediatr Gastroenterol Nutr. 2016 Feb;62(2):328-34. doi: 10.1097/MPG.0000000000000990.
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.
A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE.
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.
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.
胰腺炎是儿童期系统性红斑狼疮（cSLE）中一种罕见且危及生命的表现。本研究的目的是根据国际儿童胰腺炎研究组的标准对cSLE患者的胰腺炎进行系统分类，并确定其总体患病率、临床特征、实验室检查结果及首次发作的转归。
在10个儿科风湿病中心进行的一项多中心队列研究，纳入852例cSLE患者。
852例cSLE患者中有22例（2.6%）被诊断为胰腺炎。其中20例（91%）为急性胰腺炎，2例（9%）为急性复发性胰腺炎，均无慢性胰腺炎。所有患者均无胆结石、创伤性胰腺炎，也未报告有酒精/烟草使用史。胰腺炎患者（首次发作）与无此并发症患者的比较显示，疾病病程较短（1 [0 - 10]年 vs 4 [0 - 23]年，P < 0.0001），系统性红斑狼疮疾病活动指数2000的中位数较高（21 [0 - 41] vs 2 [0 - 45]，P < 0.0001）。胰腺炎患者发热（P < 0.0001）、体重减轻（P < 0.0001）、浆膜炎（P < 0.0001）、肾炎（P < 0.0001）、动脉高血压（P < 0.0001）、急性肾衰竭（P < 0.0001）、巨噬细胞活化综合征（P < 0.0001）及死亡（P = 0.001）的发生率也更高。胰腺炎患者静脉使用甲泼尼龙的频率（P < 0.0001）及泼尼松剂量的中位数（55 [15 - 60] vs 11 [1 - 90] mg/天，P < 0.0001）显著更高。值得注意的是，2例急性复发性胰腺炎患者各发作2次，无痛间期分别为1年和4年。
这是第一项使用国际儿童胰腺炎研究组的标准化定义对cSLE患者的胰腺炎进行特征描述的研究，结果显示主要形式为与糖皮质激素治疗及活动性重症疾病相关的急性胰腺炎。
