Pneumatosis cystoides intestinalis in progressive systemic sclerosis: a case report and literature review

Abstract

Pneumatosis cystoides intestinalis (PCI) is an uncommon disease manifestation characterized by the presence of air in the bowel wall. It is a benign condition that often responds to conservative management; however, it may be a harbinger of end stage disease, particularly in progressive systemic sclerosis. The authors report a case of pneumatosis cystoides intestinalis in a patient with progressive systemic sclerosis in the setting of mixed connective tissue disease that responded to conservative treatment. They review the current literature on pneumatosis cystoides intestinalis, focusing on possible etiologies and potential therapies.