초록
전신경화증에서 위장관 증상은 흔하게 나타나지만 소화관 장벽의 점막하층 또는 장막하층에 다발성 낭포를 보이는 창자벽 공기낭증은 매우 드문 합병증이다. 본 증례는 복부팽만으로 내원한 전신경화증 환자에서 진찰 및 영상 검사를 통해 기복증이 동반된 창자벽 공기낭증을 진단하였으며 특별한 치료 없이 호전된 경우이다. 창자벽 공기낭증의 경과는 양성부터 생명을 위협하는 경우까지 다양하므로 병력, 진찰소견, 영상 및 검사 결과를 고려하여 치료를 결정해야 한다.
The gastrointestinal tract is commonly involved in patients with systemic sclerosis. The manifestations include motility disorder, pseudo-obstruction, malabsorption, bacterial overgrowth, diverticuli, and, less commonly, pneumatosis cystoides intestinalis (PCI). PCI is characterized by the presence of air in the submucosal or subserosal layer of the bowel wall and is often accompanied by pneumoperitoneum. Although PCI is a benign condition that often responds to conservative management, it is a poor prognostic factor of systemic sclerosis. We report a case of PCI in a patient with systemic sclerosis. The chest and abdominal radiographic findings comprised pneumoperitoneum, marked dilation of the bowels, and intramural air, compatible with PCI. The patient's symptoms improved spontaneously. It is important to recognize PCI as a gastrointestinal manifestation of systemic sclerosis, and physicians should differentiate it from serious complications-such as intestinal perforation-based on clinical manifestations.