Abdominal manifestations in childhood-onset systemic lupus erythematosus (SLE)

Olivier Richer ¹, Tim Ulinski ², Irene Lemelle ³, Bruno Ranchin ⁴, Chantal Loirat ⁵, Jean-Charles Piette ⁶, Pascal Pillet ⁷, Pierre Quartier ⁸, remi salomon ⁸ and Brigitte Bader-Meunier ^9*

¹ Hôpital Pellegrin and Hôpital de Bicêtre, France
² Hôpital Trousseau, France
³ Hôpital Brabois, France
⁴ Hôpital Herriot, France
⁵ Hôpital Debré, France
⁶ Hôpital La Pitié, France
⁷ Hôpital Pellegrin, France
⁸ Hôpital Necker, France
⁹ Hôpital Bicêtre, France

^* To whom correspondence should be addressed. E-mail: brigitte.bader-meunier{at}bct.aphp.fr.

Accepted 17 June 2006

Abstract

Objective: Childhood-onset lupus erythematosus (SLE) is a rare disorder of unknown origin. The objective of this study was to describe the frequency of gastrointestinal manifestations at presentation of SLE and at follow-up, and discuss the specific causes of these manifestations.

Methods: The medical records of 201 patients with childhood-onset SLE followed in French pediatric nephrologic, hematologic and rheumatologic centers were reviewed and abstracted for GI manifestations.

Results: GI involvement was recorded in 39 (19 %) children. The median age at the time of initial GI manifestations was 11.3 years ( range: 4.5-16 years). GI were present at or occurred within 1 month after diagnosis in 32 patients. Abdominal pain was the most frequent symptom, present in 34 patients (87%). It was mostly related to lupus involvement, especially ascites (14 patients) and pancreatitis (12 patients), more rarely to therapy-induced events (1 patient) or infection (1 patient), and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before the diagnosis of SLE was made with final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. CRP values were less than 40 mg/L in all but two patients who had surgical abdomen. Abdominal ultrasonography and CT scan were abnormal in 58% and 83% of the evaluated patients respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of GI involvementin 30 out of 31 treated patients.

Conclusion: GI involvement is common in children with SLE, and is mainly due to primary lupus involvement. CS treatment should be promptly considered in lupus children presenting with abdominal pain after infectious disease, therapy side effects and intestinal perforation have been excluded.

Keywords: ascites, children,, pancreatitis, peritonitis, systemic lupus erythematosus

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