LE KREMLIN BICÊTRE, France–Children with systemic lupus erythematosus (SLE) who present with abdominal symptoms likely are suffering from exacerbations of the underlying lupus and should be treated with high doses of corticosteroids, not rushed off for exploratory laparotomy, according to researchers from the French Pediatric-Onset SLE Study Group. In data reported in Annals of the Rheumatic Diseases, Olivier Richer and colleagues write that gastrointestinal (GI) involvement was observed in 19% of children with SLE, and that the most common problem was abdominal pain, which was never due to events unrelated to SLE.1
Chart Review Finds Almost No Nonlupus Abdominal Problems in Children With SLE
Dr. Richer analyzed the medical records of 201 patients with childhood-onset SLE who were being followed in French pediatric nephrology, hematology, or rheumatology centers. Thirty-nine (19%) of these children had recorded GI involvement, with median age of onset of GI problems at 11.3 years (range 4.5–16 y). GI problems were present at the time of lupus diagnosis or appeared within 1 month after diagnosis in 32 (16%) of the 201 patients.
The most frequent abdominal symptom was pain, which was present in 34 (87%) of the 39 patients with GI involvement. Dr. Richer said that abdominal pain was related to lupus itself in 31 patients (ascites in 14 patients, pancreatitis in 12), to steroid toxicity in two patients, and to cytomegalovirus infection in one patient. Pancreatitis occurred at onset of SLE in eight children and during the course of disease in four children. These problems occurred in the context of generalized severe SLE flares, were accompanied by serum amylase levels from two to 10 times normal values, and required intensive care unit hospitalization in three cases. "Increasing the previous dosage of corticosteroids (CS) or initiating CS therapy resulted in a resolution of symptoms and decrease of pancreatic enzymes in 11 children. Recurrent episodes of pancreatitis occurred after increase of CS dosage in one child; they were thought to be related to therapy," Dr. Richer and colleagues write.
Three of the five children with surgical abdomen underwent laparotomies before the lupus diagnosis had been made, with final diagnoses of lupus peritonitis and lupus acalculous cholecystitis.
In most patients C-reactive protein values were less than 40 mg/L. Fifty-eight percent of patients had abnormal abdominal ultrasonography, and 83% had abnormal abdominal computed tomography scans.
"Corticosteroids, associated with intravenous cyclophosphamide in eight patients, led to complete remission of GI involvement in 30 out of 31 treated patients," the authors write. They conclude that "GI involvement is common in children with SLE and is mainly due to primary lupus involvement."
"Abdominal pain often is present at the onset of the disease and must be explored, as well as renal or central nervous system involvement. It should not be underestimated. In most of our cases, abdominal manifestations were associated with severe extraintestinal manifestations of SLE," Dr. Richer said. "If you have made the diagnosis of lupus flare-up with abdominal involvement, especially pancreatitis or lupus peritonitis, the use of CS is not a problem and has less pathogenic effect than the disease."
Reference
1. Richer O, Ulinski T, Lemelle I, et al. Abdominal manifestations in childhood-onset systemic lupus erythematosus (SLE). Ann Rheum Dis. 2006;3 July 2006; [Epub ahead of print]