Respiratory Failure Due to Intestinal Pseudo-obstruction in Systemic Sclerosis

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Excerpt

A 47-year-old woman with a 12-year history of systemic sclerosis was admitted to our hospital because of dyspnea and clouding of consciousness. Physical examination revealed diminished bowel sounds and abdominal distension. She did not have findings of enteritis and history of abdominal operation. Arterial blood gas under 28% inspired oxygen was as follows: pH, 7.375; PaCO2, 88.7 mm Hg; PaO2, 60.8 mm Hg; base excess, 21.0 mmol/L; and bicarbonate, 50.7 mmol/L. Radiograph of the chest and abdomen revealed marked colon gas and elevation of diaphragm by expanded colon (Fig.).
We diagnosed pseudo-obstruction associated with systemic sclerosis. Interstitial pneumonia was found only slightly. She was treated with intravenous hyperalimentation and prokinetic agents, but she died of respiratory failure on the 12th day after admission.
Upward displacement of the diaphragm compresses the lungs and worsens static and dynamic pulmonary compliance, decreasing total lung capacity. This leads anatomically to basal atelectases of the lungs and, functionally, to a restrictive ventilation disorder.1,2
Fatal respiratory failure due to pseudo-obstruction of the intestine may occur in systemic sclerosis.

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