P0967 - A Rare Diagnosis of Lupus Enteritis

Michael Coles, MD, Muaaz Masood, MD, Adria Madera Acosta, MD
Medical College of Georgia, Augusta, GA

Introduction: Lupus enteritis (LE) is an uncommon yet potentially fatal complication of systemic lupus erythematosus (SLE).

Case Description/Methods: Patient is a 41-year-old African American female with past medical history significant for Systemic Lupus Erythematosus with nephritic manifestations who presented to the ED with diffuse generalized abdominal pain, and constipation. Of note patient had been off Hydroxychloroquine therapy for several months. CT Abdomen Pelvis without contrast on admission demonstrated short segment intussusception within a loop of ileum within the right lower quadrant (Figure 1A) and submucosal edema involving multiple loops of small bowel along with inflammation within the mesentery (Figure 1B). Laboratory analysis demonstrated pancytopenia, negative stool culture, Clostridium difficile polymerase chain reaction, elevated C-reactive protein at 14.754 mg/dL, nonreactive antinuclear antigen, normal C3 and C4; normal Antineutrophil cytoplasmic antibodies proteinase 3(ANCA PR3) and ANCA myeloperoxidase, and negative acute hepatitis panel. Further work-up showed elevated C1 esterase function. Patient had not been on ACE/ARB therapy. Additionally urinalysis revealed proteinura at 100mg/dL and worsening SCr from 2.1 3 months prior to 3.14 g/dL Patient was initiated on methylprednisolone at 1mg/kg with significant symptomatic improvement. Patient’s condition improved to where she fell well enough to go home and left against medical advice.

Discussion: Lupus enteritis is a rare, potentially life-threatening sequela of SLE. LE typically presents with nonspecific symptoms of abdominal pain, diarrhea or vomiting. Laboratory studies may be significant for hypocomplementemia and pancytopenia . Anti-C1-INH level is higher in SLE patients than in healthy controls and furthermore, the anti-C1-INH level correlates with the duration and activity of the disease which was noted to be elevated in our patient. Her worsening SCr and proteinuria were also concerning for exacerbation of her nephritic manifestations. CT scan is the gold standard for the diagnosis and LE is seldom confirmed on histology. There are three characteristic CT findings of LE: 1) bowel wall thickening >3 mm (“target sign”) (Figure 1C), 2) engorgement of the mesenteric vessels (“Combs sign”) and 3) increased attenuation of mesenteric fat. LE may be life-threatening if untreated. Complications of LE include peritonitis, intestinal perforation and necrosis.


Figure: Figure 1A: CT Abdomen Pelvis with contrast in the axial view demonstrating small bowel intussusception in the right lower quadrant demarcated by a red circle.
Figure 1B: CT Abdomen Pelvis with contrast in the coronal view demonstrating increased prominence of mesenteric vessels or "Combs" sign.
Figure 1C: CT Abdomen Pelvis with contrast in the sagittal view demonstrating small bowel wall thickening or "Target sign" demarcated by a red circle.


Disclosures:
Michael Coles indicated no relevant financial relationships.
Muaaz Masood indicated no relevant financial relationships.
Adria Madera Acosta indicated no relevant financial relationships.


Michael Coles, MD, Muaaz Masood, MD, Adria Madera Acosta, MD. P0967 - A Rare Diagnosis of Lupus Enteritis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.