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Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients
Victor L S Marques1, Natali W S Gormezano, Eloisa Bonfá
1*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.
Insights
Pancreatitis is a rare complication in childhood-onset systemic lupus erythematosus (cSLE), predominantly presenting as acute pancreatitis. This condition is associated with active disease and glucocorticoid treatment in pediatric patients.
Area of Science:
- Pediatric Rheumatology
- Gastroenterology
- Systemic Lupus Erythematosus Research
Background:
- Pancreatitis is a severe, life-threatening manifestation of childhood-onset systemic lupus erythematosus (cSLE).
- Understanding pancreatitis prevalence and characteristics in cSLE is crucial for early diagnosis and management.
Purpose of the Study:
- To systematically classify pancreatitis in cSLE patients using standardized definitions.
- To determine the prevalence, clinical features, laboratory findings, and outcomes of pancreatitis in cSLE.
- To investigate the association between pancreatitis and disease activity, treatment, and patient outcomes.
Main Methods:
- A multicenter cohort study involving 852 patients with cSLE across 10 pediatric rheumatology centers.
- Classification of pancreatitis followed the International Study Group of Pediatric Pancreatitis criteria.
- Comparison of clinical and laboratory features between cSLE patients with and without pancreatitis.
Main Results:
- Pancreatitis occurred in 2.6% (22/852) of cSLE patients, primarily as acute pancreatitis (91%).
- Patients with pancreatitis exhibited shorter disease duration, higher SLE disease activity, and increased incidence of fever, weight loss, serositis, nephritis, and macrophage activation syndrome.
- Higher rates of intravenous methylprednisolone and prednisone use were observed in cSLE patients with pancreatitis.
Conclusions:
- This study is the first to characterize pancreatitis in cSLE using standardized pediatric pancreatitis definitions.
- Acute pancreatitis is the predominant form in cSLE, strongly associated with active disease and glucocorticoid therapy.
- Findings highlight the importance of recognizing pancreatitis as a significant complication in pediatric lupus patients.
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.
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