[Scleroderma with an unusual complication]

A 30 year-old woman who had had progressive systemic sclerosis for seven years was admitted to our hospital with a history of one week of increasing abdominal girth and one month's history of increasing peripheral oedema, especially in the lower limbs, and a weight loss of 10 kilograms in the course of five months. On admission she had oedema of the lower limbs, lower back and abdomen, and had ascites but no signs of peritonitis. An abdominal X-ray examination revealed distended bowels with elevated air fluid levels and pneumoperitoneum. The combination of the clinical and radiological findings, as well as the history, strongly suggested a diagnosis of pneumocystosis cystoides intestinalis as a complication to progressive systemic sclerosis. The symptoms improved on treatment and no signs of recurrence were found at follow-up after four years. We discuss the underlying pathology and management of this condition.