CHEST Guidelines-A-23-Year-Old-Woman-With-a-Rare-Presentation-of-Sy

A-23-Year-Old-Woman-With-a-Rare-Presentation-of-Sy

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This case study explores a 23-year-old woman diagnosed with systemic lupus erythematosus (SLE) presenting with rare gastrointestinal involvement, known as lupus enteritis, and its severe complication, necrotizing enterocolitis (NEC). Initially, she reported experiencing vomiting, diarrhea, significant weight loss, polyarthralgia, oral ulcers, and nonscarring alopecia. Diagnostic imaging revealed diffuse thickening of various intestinal sections, which initially led to treatment with high-dose oral prednisone and mesalamine, but with limited success.<br /><br />Upon her hospital presentation, she exhibited pallor, tachycardia, and abdominal tenderness. Lab tests indicated high titers of anti-nuclear antibodies and anti-dsDNA with low complement levels, suggesting an SLE flare-up. She was then administered intravenous methylprednisolone, but this was withheld after developing nosocomial pneumonia. Her condition deteriorated, presenting an acute abdomen with distension. Subsequent imaging showcased hepatic portal venous gas (HPVG), indicative of NEC.<br /><br />An exploratory laparotomy was performed, revealing patchy serosal hemorrhage and other bowel anomalies, leading to jejunal resection and jejunostomy. Post-operation, she was treated with IV cyclophosphamide.<br /><br />GI manifestations, including lupus enteritis, occur in a small percentage of SLE cases and are not typically part of standard diagnostic criteria. Lupus enteritis symptoms range from abdominal pain to severe complications like ischemia. HPVG is a critical diagnostic indicator of severe NEC. Management of lupus enteritis often involves high-dose steroids, with cyclophosphamide or mycophenolate mofetil as alternatives.<br /><br />This case underscores the importance of early detection and intervention in lupus enteritis to prevent fatal complications, highlighting the diagnostic utility of ultrasound and computed tomography in identifying NEC. Meticulous treatment can lead to favorable outcomes despite the complex presentation and potential for severe complications.

Keywords

systemic lupus erythematosus

lupus enteritis

necrotizing enterocolitis

gastrointestinal involvement

anti-nuclear antibodies

hepatic portal venous gas

exploratory laparotomy

jejunal resection

cyclophosphamide

diagnostic imaging