Ileannette Robledo, MD, Jose Carlos Barrientos, MD, Joan Bosco, MD, Christine Chen, MD, Harry Fischer, MD, Wan Ling Lam MD, Mount Sinai Beth Israel, Icahn School of Medicine, New York, NY,
Abstract Text:
Case Presentation: We describe a case of a 28-year-old Asian woman with history of connective tissue disorder presenting with recurrent admissions for localized postprandial abdominal pain, watery diarrhea, and bilious emesis. Labs included: BUN 16mg/dL, Cr 0.65mg/dL, WBC 9.5 K/uL, AST/ALT of 15/15 U/L, lactic acid of 0.7 mmol/L, and hCG negative. Stool culture, ova and parasites, and Clostridium difficile toxin, were negative. Physical exam at each admission revealed a woman with dry mucus membranes, and bilateral lower abdominal tenderness. Prior workup included colonoscopy with biopsy that were negative for inflammatory bowel disease, as well as an abdominal CT scan which was consistent with acute enterocolitis. On a prior admission, the patient was treated with ciprofloxacin and metronidazole with no relief of symptoms until intravenous methylprednisolone, 20 mg every eight hours, was initiated. The patient had complete resolution of symptoms, but returned to the hospital shortly after due to recurrence. Repeat CT scan showed worsening enteritis, ascites and diffuse circumferential bowel wall thickening in the small intestine. Once an infectious etiology was ruled out, rheumatologic workup was initiated. C-reactive protein was 3.42 mg/L, antinuclear antibody (ANA) was 1:320, and complement C3 and C4 were low at 33 and 5 mg/dL, respectively. The patient was diagnosed with lupus enteritis (LE), and was treated with intravenous methylprednisolone. She was discharged on oral prednisone, with no further hospitalizations since.
Discussion: This case demonstrates how LE can be the prominent presentation of systemic lupus erythematosus (SLE). To promptly diagnose and treat LE can be challenging due to its acute presentation in a healthy young patient. However, prompt diagnosis can make a difference in outcome. Literature review reveals that the clinical symptoms most commonly seen in LE include abdominal pain (97%), vomiting, diarrhea and fever. Laboratory features include low complement levels (88%), anemia, leukocytopenia or lymphocytopenia and thrombocytopenia. Median CRP level was 2.0 mg/dL. Imaging findings include bowel wall edema with characteristic target sign, ascites, hydronephrosis, mesenteric abnormalities and bowel dilation. If left untreated, LE can progress into intestinal necrosis and perforation.
Conclusions: Our case highlights a patient with LE without a preexisting diagnosis of SLE. LE should be considered in patients with a prior diagnosis of autoimmune disorder who present with recurrent and intractable abdominal pain, diarrhea and emesis. LE has an excellent response to steroids, and, if promptly treated, symptoms, laboratory and imaging abnormalities are reversible.