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.

Enteritis lúpica como manifestación inicial de lupus eritematoso sistémico. Caso clínico

“…Estos sínto-mas se pueden acompañar de hallazgos de laboratorio inespecíficos como anemia, leucopenia, linfopenia, trombocitopenia y en algunos casos proteinuria 6 .…”

“…Dentro de los hallazgos clási-cos se incluyen engrosamiento difuso de la pared intestinal, dilatación de asas intestinales, ensanchamiento e hipervascularización esplácnica, atenuación incrementada de la grasa mesentérica y ascitis [6][7][8] .…”

Lupus Enteritis: An Important Cause to Be Considered in Systemic Lupus Erythematosus Patients with Acute Abdomen – A Case Report

“…Lupus enteritis has been found in several reports to be generally reversible and steroid responsive, thus considered as the first line therapy (1,5). High dose prednisolone, Methylprednisolone pulse have been used according to individual preferences with favourable responses along with supportive measures which include bowel rest, IV fluids, proton pump inhibitors and Heparin might be added in presence of ApL (Antiphospholipid antibody) antibodies and suspicion of Antiphoshpholipid syndrome (1,3,5) . Cyclophosphamide or Mycophenolate may be added in case of resistance to corticosteroids or when warranted by other organ involvement (1).…”

“…Most gastrointestinal manifestations are caused by adverse reaction of medicines or infections, while the symptoms related to the disease are not as common as other organ involvement. However, abdominal pain is a frequent symptom in patients with SLE and a challenging diagnostic and therapeutic dilemma as patients may be on steroids and immunosuppression, which may mask the manifestations of ischemia or perforation (2,3). developed excessive hair loss and hyperpigmented non-pruritic skin lesions over the face, back and ear lobes, without any other specific systemic complaints.Two months before; she reported an acute episode of a spreading abdominal pain with recurrent bouts of vomiting without significant alterations in bowel movements or frequency.…”

Systemic lupus erythematosus with intestinal perforation: A case report

“…Digestive disorders are less common, but potentially much more serious, and perforation of the intestinal tract is one of the most severe complications of SLE. SLE is caused by gastrointestinal vasculitis and thrombosis, and is considered to be life-threatening (2,3). The clinical symptoms of lupus enteritis are nonspecific, but generally include abdominal pain, vomiting, diarrhea and fever.…”