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Abstract

40-year-old woman with a history of systemic lupus erythematosus (SLE) for a year and a half - involving joints, mucocutaneous, hematological, and renal domains - presented to the emergency department with visceral abdominal pain of two weeks duration. A contrast-enhanced abdominal computed tomography (CT) scan was performed, revealing gas accumulation in the greater omentum and subserosal layer of the small intestine and colon, along with secondary pneumoperitoneum in the coronal plane (Figure A). Due to worsening clinical condition, diagnostic and therapeutic laparoscopy was performed, documenting multiple thin-walled air-filled cysts (cystic pneumatosis) in the colon and small intestine, without visceral perforation (Figure B). Ultimately, conservative medical management was chosen with parenteral steroid pulses, leading to an acceptable medical response over time, without additional surgical interventions.

Lupus enteritis (LE) is a serious and rare manifestation of SLE, with a prevalence ranging from 0.2% to 6.4%. It is defined as imaging evidence, vasculitis biopsy, or inflammation of the small or large intestine (1), encompassing mesenteric arteritis, mesenteric vasculitis, enteric vasculitis (which includes intestinal cystic pneumatosis), gastrointestinal vasculitis, and acute gastrointestinal syndrome, among others. In our setting, Muñoz-Urbano et al. (2) found a prevalence of lupus enteritis of 0.65% in a 10-year retrospective registry of over 2000 hospitalized patients with SLE. The most common tomographic findings were intestinal wall thickening (87.5%), followed by intestinal dilation (68.8%), predominantly in the ileum; no cases of intestinal cystic pneumatosis were documented, as in our case.