Abdominal manifestations in childhood-onset systemic lupus erythematosus

Abstract

Background: Childhood-onset lupus erythematosus is a rare disorder of unknown origin. Objectives: To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow-up, and discuss the specific causes of these manifestations. Methods: Medical records of 201 patients with childhood-onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations. Results: Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5-16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment-induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were ,40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients. Conclusion: Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded.

Celiac Disease and Autoimmunity in the Gut and Elsewhere

“…[24] A more recent study from Northern Ireland did not reveal clinical evidence of SLE among a cohort of 60 celiac disease patients. [25] While gastrointestinal manifestations of SLE are common in both adults and children, [26][27][28] CD is relatively rare among this patient population but should be considered in the setting of malabsorption symptoms. False positive ANA tests may be found in patients with untreated celiac disease.…”

Clinical and pathologic characteristics of pancreatic necrosis in critically ill children

“…[13] Pancreatic damage is associated with systemic metabolic diseases, such as diabetic ketoacidosis, systemic lupus erythematosus, Henoch-Schonlein purpura, hemolytic uremic syndrome and others. [9,[14][15][16] L-asparaginase can cause pancreatic dysfunction in children with acute lymphoblastic leukemia (ALL). Pancreatic necrosis is one of the serious adverse effects, and the mortality of this type of pancreatitis is 2.5%.…”

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

“…18,19 SLE-related GI involvement is clinically important because most cases can be lifethreatening and complications are common if not treated promptly. C-reactive protein (CRP) levels are frequently normal or only mildly elevated in cSLE-related abdominal pain 18,[20][21][22] , with the exception of acute pancreatitis in which the CRP is very elevated.…”

“…[23][24][25] Multifocal segments of bowel involvement from multiple vascular territories occur over 90% of the time. 18,19 Inflammatory vasculitis is thought to be due to immune complex deposition, resulting in fibrinoid necrosis of subserosal vessels, leukocytoclasis on the vascular wall, and submucosal edema with diffuse mononuclear cell infiltrate. 26 Several studies have demonstrated that LMV occurs when patients are in the active stage of their disease with elevated SLEDAI or European Consensus Lupus Activity Measure (ECLAM) scores.…”

“…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…”