Search

The patient was a 70-year-old man who had been given a diagnosis of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome and had been placed on low-dose steroid therapy in the Department of Orthopedics. During treatment, sudden fever, hypoxemia and chest radiography-confirmed interstitial shadows throughout the lung fields were noted, and the patient was referred to the Department of Internal Medicine. RS3PE complicated by interstitial pneumonia was diagnosed, and steroid pulse therapy and immunosuppressant therapy were initiated. In the present case, soluble interleukin-2 receptor (sIL-2R) proved useful for assessing symptoms. To the best of our knowledge, RS3PE syndrome complicated by pulmonary lesions and accompanied by severe acute respiratory failure requiring noninvasive positive-pressure ventilation has not previously been reported, and this rare case is discussed with reference to the literature.

A 43-year-old woman developed dyspnea on effort in January 1996. She was treated with various antibiotics but developed dyspnea and pretibial edema. She was referred to our hospital and admitted on February 20, 1996. On the basis of the clinical course and radiological findings, she was diagnosed as having idiopathic pulmonary fibrosis with right-side heart failure. After high-dose steroid therapy (methylprednisolone, 1,000 mg/day for 3 days) and the administration of a diuretic, oral prednisolone therapy was initiated. Her condition gradually recovered. To obtain a definite diagnosis, an open lung biopsy was recommended but the patient refused the procedure. She was discharged from the hospital and placed on home oxygen therapy. After her informed consent was obtained, she became a candidate recipient for the nationwide Central Lung Transplant Evaluation Committee on August 7, 1998. Her name was then listed in the Japan Organ Transplant Network. The patient was admitted to our hospital in October 1998 because of respiratory failure. She underwent left lung transplantation at Osaka University Hospital on March 29, 2000. After the lung transplantation, she was discharged and is presently doing well without the need for supplementary oxygen. A differential diagnosis of the removed lung as nonspecific interstitial pneumonia (NSIP) group III or UIP was required. We finally diagnosed NSIP group III because of the temporal uniformity and diffuse distribution of the fibrosis. In this report, we also describe the background of the clinical diagnosis, the indications for lung transplantation and the clinical course before and after transplantation.