Systemic Lupus Erythematosus-Related Pancreatitis in Children: Severe and Lethal Form

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease of unknown cause, characterized by multisystemic involvement. Its occurrence in children is rare, and acute pancreatitis is exceptional in this matter. Its diagnosis is clinical, biological, and radiological. Its treatment is based on corticosteroid therapy, and its progress is generally lethal. We report two cases of acute pancreatitis in the course of SLE, highlighting its life-threatening severity despite well-conducted treatment. Case 1: 14-year-old patient, admitted to the pediatric ICU for altered state of consciousness. This child, an outpatient since 2009 for chronic arthralgia, was hospitalized five days previously in the pediatric ward for suspicion of severe SLE, before presenting abdominal pain and vomiting. Hyperlipasemia was found, and an abdominal CT scan confirmed the diagnosis of acute pancreatitis. The patient was put under immunosuppressive therapy composed of high-dosage of corticosteroid and cyclophosphamide cures. She died 20 days after her hospitalization by severe lupus flare with multiorgan failure. Case 2: 14-year-old child, admitted to the Pediatric ward for prolonged fever associated with polyarthralgia (nondeforming, immovable, and additive) that had been progressing since 6 months with altered general state; his symptoms got worst 15 days before his hospitalization by having behavioral disorders and epigastralgia with vomiting. Pancreatitis was strongly suspected in the absence of improvement on symptomatic treatment and confirmed by hyperlipasemia 6 times the normal value and a swollen pancreas on the abdominal CT scan. The child was treated with Solumedrol and cyclophosphamide without improvement and then died after one month of hospitalization by a septic shock.

Untitled

“…The diagnostic criteria of acute pancreatitis require two of the following three criteria: Typical abdominal pain; amylase and/or lipase levels that are three or more times the upper limit of normal; and typical abdominal ultrasoundor an abdominal CT scan or MRI of the pancreas [15].…”

The application of current classification systems in pediatric cytopathology: Perspectives from the pediatric cytopathology symposium at the 20th International Congress of Cytology 2019

“…22 These benign lesions can be associated with severe pancreatitis, which can prove to be lethal if not treated emergently. 23 In the spectrum of neoplastic lesions, pancreatoblastoma, pancreatic neuroendocrine neoplasm, solid pseudopapillary tumor, germ cell tumor, sarcoma, and lymphoma should be considered before a pancreatic ductal adenocarcinoma in a young patient (Table 1). For PSC-RPBC category IV neoplasms in children, category IV of "neoplastic: benign" would apply to serous cystadenomas and schwannomas, whereas category IV "neoplastic: other" would apply to neuroendocrine tumors and solid pseudopapillary tumors.…”

“…The more likely etiology of a benign pancreatobiliary lesion in a child is a developmental cyst, hamartoma, or inflammatory process (Rosai‐Dorfman disease, autoimmune/IgG4‐related disease, or infection) . These benign lesions can be associated with severe pancreatitis, which can prove to be lethal if not treated emergently …”