S2881 Lupus Enteritis as the Unique Manifestation of Lupus Flare - Report of 2 Cases

Abstract

INTRODUCTION: Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematous (SLE) but real GI involvement is rare. Lupus enteritis refers to either vasculitis or inflammation of the bowel wall. It is present in 0.2–5.8% of patients usually in the context of high-SLE activity. The symptoms are nonspecific and can be life-threatening especially if the treatment is delayed. We present two cases of lupus enteritis as the sole manifestation of SLE flare. CASE DESCRIPTION/METHODS: Case 1. 19-year-old female with no prior SLE diagnosis was admitted with four months of persistent diarrhea, vomiting, and epigastric pain. Workup revealed Mg 0.5 mg/dl, K 2.5 mEq/l, Hb 11.2 g/dl, CRP 1.40 mg/dl, and subnephrotic proteinuria. Infectious workup was negative. CT scan showed diffuse thickening and enhancement of the small and large bowel wall. Upper and lower endoscopy with biopsy revealed nonspecific edema and chronic inflammation. Further evaluation showed low complement levels, positive ANA ( > 1:160), anti-dsDNA ( > 300 IU/ml), SSA, SSB, anti-histone Ab, and lupus anticoagulant. IV methylprednisolone was started with marked improvement, later transitioned to hydroxychloroquine, azathioprine, and prednisone with taper dose. Case 2. 49-year-old female, SLE diagnosed in 2017, no treatment for 2 years was admitted with one month of persistent vomiting and abdominal pain. Evaluated initially with upper and lower endoscopy which revealed Barret’s esophagus. Workup revealed Na 132 mEq/L, Mg 1.6 mg/dl, lymphopenia (200 k/uL), elevated CRP (0.67 mg/dl), and subnephrotic proteinuria. Negative infectious workup. Further evaluation showed ANA ( > 1:160), Anti Smith ( > 8), anti-dsDNA (4 IU/ml), SSA ( > 8), and low complement levels. CT scan with diffuse thickening and enhancement of the small bowel and entire colon and rectum. IV methylprednisolone and a short course of IV erythromycin was started with significant improvement; later transitioned to hydroxychloroquine, azathioprine, and prednisone with taper dose. Both patients did not present recurrence of symptoms during the next 3 months. DISCUSSION: Lupus enteritis is very rare as an unique feature of SLE flare. Current knowledge is scarce. CT scan is the test of choice. It usually responds to steroids. Steroid-sparing agents are required in severe-relapsing cases. Our cases highlight the difficult diagnosis of this disease. Identifying this condition is crucial to avoid complications as bowel necrosis or perforation.Figure 1.: Contrast enhanced abdomen CT scan coronal axial views revealed “target” sign that represent circumferential thickening of the intestine wall. In this case affecting both small and large intestine.Figure 2.: Contrast enhanced abdomen CT scan showing diffuse thickening of the small and large intestine wall. "Target" sign can also be appreciated here.