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Intestinal pseudo-obstruction as the initial presentation of systemic lupus erythematosus in a 13-year-old girl

Korean Journal of Pediatrics 2008;51(6):655-659.
Published online June 15, 2008.
Intestinal pseudo-obstruction as the initial presentation of systemic lupus erythematosus in a 13-year-old girl
Ky Young Cho1, Tae Young Khil1, Hye Mi Ahn1, Sun Wha Lee2, Jeong Wan Seo1
1Department of Pediatrics, School of Medicine, Ewha Womans University, Seoul, Korea
2Department of Radiology, School of Medicine, Ewha Womans University, Seoul, Korea
장 가성 폐쇄로 진단된 전신 홍반 루푸스 1예
조기영1, 길태영1, 안혜미1, 이선화2, 서정완1
1이화여자대학교 의과대학 소아과학교실
2이화여자대학교 의과대학 영상의학과학교실
Correspondence: 
Jeong Wan Seo, Email: jwseo@ewha.ac.kr
Abstract
Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement. Simple abdominal radiographs revealed mild dilatation with partial air-fluid levels in the small intestine. Abdominal CT and methylcellulose small bowel studies showed massive ascites, engorgement of the small mesenteric vessels, pleural effusion, and diffuse bowel wall thickening of the gastric antrum, duodenum. and jejunum. The delayed passage of contrast for 15 days after the methylcellulose small bowel studies was suggestive of decreased bowel motility. Laboratory findings were positive for ANA, anti-double-stranded DNA, anti-Smith and lymphopenia. After 10-day treatment with high-dose corticosteroids, the symptoms improved. IPO associated with SLE should be considered in the differential diagnosis for patients presenting with symptoms of intestinal obstruction. Early recognition of IPO in SLE and appropriate therapy are important for prevention of complications and unnecessary surgery. This case raises awareness among pediatricians that although rare, IPO can be the presenting symptom of SLE in children.
Key Words: Intestinal pseudo-obstruction, Systemic lupus erythematosus


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