Pancreatitis Subtypes Survey in 852 Childhood‐Onset Systemic Lupus Erythematosus Patients

Abstract

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.

Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

“…Pancreatitis was diagnosed in 22 of 852 (2.6%) of cSLE patients, classified as acute pancreatitis in 91% of them and associated with active severe disease. The first pancreatitis episode was observed at disease onset in 6 of 22 (27%) cSLE patients [19]. Moreover, macrophage activation syndrome was found in 36% of patients with acute pancreatitis [19].…”

“…The first pancreatitis episode was observed at disease onset in 6 of 22 (27%) cSLE patients [19]. Moreover, macrophage activation syndrome was found in 36% of patients with acute pancreatitis [19].…”

Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

“…Astonishingly, reported mortality rates range from 25-54%. 27,28 When comparing cSLE with aSLE, acute pancreatitis occurs more frequently; is more severe; and is associated with higher mortality in cSLE patients. 27 The pathophysiology of pancreatitis in cSLE is not well understood, but immune complex deposition, microthrombi, vasculitis, intimal thickening, and ischemia are all believed to play a role.…”

“…Very high erythrocyte sedimentation rate (ESR) and CRP values are frequent, as are leucopenia, lymphopenia, thrombocytopenia and anti-dsDNA antibody positivity. 28 Severe pancreatitis, defined as multi-organ dysfunction and pancreatic necrosis, occurs in 50% of cSLE-related pancreatitis. 28,29 Pancreatitis usually occurs in the setting of a generalized SLE flare, or as a presenting manifestation, and requires emergency management in an intensive care setting.…”

“…21,24,25 Pancreatitis Acute pancreatitis in cSLE is rare with a prevalence of 2.6-6%. 18,20,24,27,28 Acute pancreatitis is…”