Open Access Review

Lupus enteritis: from clinical findings to therapeutic management

Peter Janssens12, Laurent Arnaud136*, Lionel Galicier45, Alexis Mathian13, Miguel Hie16, Damien Sene17, Julien Haroche1, Catherine Veyssier-Belot8, Isabelle Huynh-Charlier9, Philippe A Grenier9, Jean-Charles Piette1 and Zahir Amoura136

Author Affiliations

1 Department of internal medicine, French reference centre for Systemic Lupus Erythematosus, AP-HP, Hôpital Pitié-Salpêtrière, F-75013, Paris, France

2 Department of internal medicine, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium

3 Institut National de la Santé et de la Recherche Médicale (INSERM), UMR-S 945, Paris, France

4 Department of clinical immunology, Hôpital Saint Louis, Paris, France

5 Université Paris Diderot, Sorbonne Paris Cité, Paris, France

6 Université Pierre et Marie Curie, UPMC Univ Paris 06, F-75005, Paris, France

7 Department of internal medicine, Hôpital Lariboisière, Paris, France

8 Department of internal medicine and nephrology, Centre Hospitalier Intercommunal de Poissy Saint-Germain-en-Laye, Saint-Germain-en-Laye, France

9 Department of radiology, Hôpital Pitié-Salpêtrière, Paris, France

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Orphanet Journal of Rare Diseases 2013, 8:67  doi:10.1186/1750-1172-8-67

Published: 3 May 2013

Abstract

Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). In this study, we report a series of 7 new patients with this rare condition who were referred to French tertiary care centers and perform a systematic literature review of SLE cases fulfilling the revised ACR criteria, with evidence for small bowel involvement, excluding those with infectious enteritis. We describe the characteristics of 143 previously published and 7 new cases. Clinical symptoms mostly included abdominal pain (97%), vomiting (42%), diarrhea (32%) and fever (20%). Laboratory features mostly reflected lupus activity: low complement levels (88%), anemia (52%), leukocytopenia or lymphocytopenia (40%) and thrombocytopenia (21%). Median CRP level was 2.0 mg/dL (range 0–8.2 mg/dL). Proteinuria was present in 47% of cases. Imaging studies revealed bowel wall edema (95%), ascites (78%), the characteristic target sign (71%), mesenteric abnormalities (71%) and bowel dilatation (24%). Only 9 patients (6%) had histologically confirmed vasculitis. All patients received corticosteroids as a first-line therapy, with additional immunosuppressants administered either from the initial episode or only in case of relapse (recurrence rate: 25%). Seven percent developed intestinal necrosis or perforation, yielding a mortality rate of 2.7%. Altogether, lupus enteritis is a poorly known cause of abdominal pain in SLE patients, with distinct clinical and therapeutic features. The disease may evolve to intestinal necrosis and perforation if untreated. Adding with this an excellent steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare entity.

Keywords:
Systemic lupus erythematosus; Abdominal pain; Lupus enteritis; Small bowel disease; Vasculitis