”first and finest” lupus enteritis: a pain in the gut lt james prim, do lcdr shauna...

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Lupus Enteritis:A Pain in the Gut

LT James Prim, DO

LCDR Shauna O’Sullivan, DO

Naval Medical Center Portsmouth

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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

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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

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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

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CT Imaging

“Target sign”: circumferential mucosal edema

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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

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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.

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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

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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.

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Questions?