Abstract
We describe here a 51-year-old man with polymyositis and interstitial pneumonia (IP) who developed pneumatosis cystoides intestinalis (PCI). The patient was diagnosed as PM associated with interstitial pneumonia when he was 43 years old. Methylprednisolone and cyclosporine treatment were started. His clinical symptoms including the serum data improved progressively. Cyclosporine was stopped and methylprednisolone was tapered to 2 mg/day. However, IP had become worse when he was 50-year-old. The dosage of methylprednisolone was increased. However, he was admitted to our hospital due to intramural gas in the intenstinal wall and free air in the bottom of diaphragm, which was detectable by follow up CT scan. His vital signs were almost normal, and physical examination showed only mild abdominal distention. Physical, laboratory and radiographic examination showed no evidence of infection or necrotizing enterocolitis. Based on these findings, a diagnosis of PCI was established. We selected conservative treatment, the patient was treated with high dosage oxygen (5 L/min via a face mask for 5h per day), and diet was discontinued for 14 days. High dose oxygen was effective, but the gas did not disappear completely, and recurred upon discontinuation of oxygen. When high dose oxygen was used in addition to oxygen at hyperbaric pressure for1hr per day, PCI disappeared by abdominal CT scan. This progress was monitored by colonoscopy.
We reported this rare case of PCI with polymyositis compared with PCI associated with other rheumatic disorders.
