Lupus enteritis: from clinical findings to therapeutic management

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.

Figure 1

Imaging findings of lupus enteritis. Panel A: Edematous small-bowel wall (ileum) with the characteristic target sign (abnormal bowel-wall thickening and enhancement) and ascites. Panel B: Small bowel distention with engorgement of mesenteric vessels and ascites. Panel C. Edematous ileum wall with engorgement of mesenteric vessels and increased number of visible vessels (comb’s sign). Panel D: Bowel-wall thickening and enhancement (target sign). All the above described abnormalities are non-specific and can also be seen in patients with pancreatitis, mechanical bowel obstruction, peritonitis, or inflammatory bowel disease, all of which may mimic intestinal ischemia.

Figure 2

Therapeutic strategy for lupus nephritis. CNS: central nervous system; IV: intravenous; HCQ: hydroxychloroquine; AZA: azathioprine; MMF: mycophenolate mofetil.