1. ”FIRST AND FINEST” Lupus Enteritis: A Pain in the Gut LT James Prim, DO LCDR Shauna O’Sullivan, DO Naval Medical Center Portsmouth

2. ”FIRST AND FINEST” Disclaimer Nothing to disclose The views expressed in this presentation are those of the author(s) and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the United States Government

3. ”FIRST AND FINEST” Case Nineteen year old female presenting with acute, severe, diffuse abdominal pain and intractable nausea Past medical history: –Hospitalized several months prior for a similar episode of abdominal pain CT abdomen: nonspecific jejunal and ileal bowel wall thickening –Gastroenterology evaluation: EGD and colonoscopy: unremarkable

4. ”FIRST AND FINEST” Case (continued) Laboratory findings: –Positive ANA, anti-dsDNA, anti-Sm, anti-Ro, and anti-RNP autoantibodies –↑ ESR, normal CRP, ↓ C3/C4 Imaging: –CT Abdomen: Diffuse bowel wall edema associated with prominence of vasa recta, mesenteric edema, and ascites

5. ”FIRST AND FINEST” CT Imaging “Target sign”: circumferential mucosal edema

6. ”FIRST AND FINEST” Lupus Enteritis A rare gastrointestinal manifestation of SLE Pathophysiology: –Immune complex deposition, complement activation Clinical findings: –Pain: diffuse ± peritoneal signs –Fever, nausea, diarrhea Laboratory findings: –Positive ANA, dsDNA, other autoantibodies –Hypocomplementemia Produces focal or diffuse small bowel inflammation and characteristic findings on abdominal imaging

7. ”FIRST AND FINEST” Lupus Enteritis: Treatment Medical therapy: –IV or high dose oral steroids → excellent response –Cyclophosphamide in severe or refractory cases Untreated → intestinal necrosis, perforation –Requires surgical intervention, high mortality rate Likelihood of recurrence unknown Lee, C. K., et al. "Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis)." Annals of the rheumatic diseases. 61.6 (2002): 547-550. Janssens, Peter, et al. "Lupus enteritis: from clinical findings to therapeutic management." Orphanet journal of rare diseases. 8.1 (2013): 67.

8. ”FIRST AND FINEST” Outcome of the case Rapid improvement with IV methylprednisolone, eventually transitioned to oral steroids Has had one recurrent flare of lupus enteritis but has not developed any other clinical manifestations of SLE

9. ”FIRST AND FINEST” Conclusions Extremely rare for lupus enteritis to occur as the initial manifestation of SLE Acute abdominal pain in patients with SLE has a wide differential diagnosis –Low threshold for obtaining abdominal CT imaging Delay in diagnosis or therapy is associated with significant morbidity and mortality Kwok, S-K., et al. "Lupus enteritis: clinical characteristics, risk factor for relapse and association with anti-endothelial cell antibody." Lupus 16.10 (2007): 803-809. Tian, Xin-Ping, and Xuan Zhang. "Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment." World journal of gastroenterology: WJG. 16.24 (2010): 2971.

10. ”FIRST AND FINEST” Questions?