LT James Prim DO LCDR Shauna OSullivan DO Naval Medical Center Portsmouth Disclaimer Nothing to disclose The views expressed in this presentation are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy Department of D ID: 812326
Download The PPT/PDF document "Lupus Enteritis: A Pain in the Gut" is the property of its rightful owner. Permission is granted to download and print the materials on this web site for personal, non-commercial use only, and to display it on your personal computer provided you do not modify the materials and that you retain all copyright notices contained in the materials. By downloading content from our website, you accept the terms of this agreement.
Slide1
Lupus Enteritis:A Pain in the Gut
LT James Prim, DO
LCDR Shauna O’Sullivan, DO
Naval Medical Center Portsmouth
Slide2DisclaimerNothing to discloseThe 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
Slide3CaseNineteen 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
Slide4Case (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
Slide5CT Imaging“Target sign”: circumferential mucosal edema
Slide6Lupus EnteritisA rare gastrointestinal manifestation of SLEPathophysiology:
I
mmune
complex deposition, complement
activation
Clinical findings:
Pain
: diffuse ± peritoneal signs
Fever, nausea, diarrhea
Laboratory findings:
Positive ANA,
dsDNA
, other autoantibodiesHypocomplementemia
Produces
focal or diffuse small bowel inflammation
and characteristic findings on abdominal imaging
Slide7Lupus Enteritis: TreatmentMedical 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.
Slide8Outcome of the caseRapid improvement with IV methylprednisolone, eventually transitioned to oral steroidsHas had one recurrent flare of lupus enteritis but has not developed any other clinical manifestations of SLE
Slide9ConclusionsExtremely rare for lupus enteritis to occur as the initial manifestation of SLEAcute abdominal pain in patients with SLE has a wide differential diagnosis and requires a careful evaluation Low threshold for obtaining abdominal CT imaging
Delay in diagnosis or immunosuppressive 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.
Slide10Questions?