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Here we report a case of systemic sclerosis (SSc) complicated with pneumoperitoneum and pneumatosis cystoides intestinalis (PCI). A 58-year-old woman who had been treated with oxygen therapy for chronic respiratory failure caused by interstitial pneumonia was admitted to Tokyo Metropolitan Ohtsuka Hospital because of abdominal fullness. On admission, laboratory data showed accelerated erythrocyte sedimentation rate, mild anemia, hypoalbuminemia, and elevated level of lactose dehydrogenase (LDH). Anti-nuclear antibodies, anti-RNP antibodies, anti-Topoisomerase I antibodies, anti-Ku antibodies, and rheumatoid factor were positive. Chest and abdominal roentgenograms showed free air under the diaphragm, dilatation of small intestine and colon, and multiple intestinal cysts. The diagnosis of pneumoperitoneum and PCI was made with laboratory findings and radiographic findings. Her symptoms did not respond to medications altering colonic motility and oxygen therapy. Finally, abdominal symptoms and radiographic findings caused by pneumoperitoneum and PCI disappeared three weeks after treatment with 1,500 mg/day of kanamycin sulfate. PCI is rarely associated with SSc. Possible pathogenic relationship between these diseases was discussed.

A case of systemic sclerosis complicated with pneumoperitoneum and pneumatosis cystoides intestinalis.