Pneumatosis cystoides intestinalis in systemic sclerosis.

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of multiple gas containing thin walled cysts in the intestinal wall and mesentery. It is sometimes associated with chronic obstructive pulmonary disease, but has been described most often in patients with gastrointestinal disorders including duodenal and gastric ulceration, small bowel obstruction, regional enteritis and gastrointestinal malignancy. Its association in the patient described below with severe systemic sclerosis is of particular interest. There have been occasional previous reports of this association which should be considered in any patient with systemic sclerosis who develops abdominal symptoms suggestive of acute or sub-acute intestinal obstruction, not readily explicable by other causes.