Nemoto Y, Katayama Y, Sugiyama K, Kudo K, Matsuoka T, Nagata N, Takatani O, Kikuchi K, Aida S
Third Department of Internal Medicine, National Defense Medical College, Saitama, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Sep;29(9):1180-5.
A 45-year-old female patient with rheumatoid arthritis and Sjögren's syndrome was admitted because of progressive exertional dyspnea and palpitation. The chest X-ray film was normal except for slight overinflation. Pulmonary function tests indicated severe mixed-type impairment, and arterial blood gas analysis revealed hypoxemia. 99mTc-MAA lung perfusion scintigraphy and 81mKr lung ventilation scintigraphy showed multiple nonsegmental defects in both lungs. During six months of observation, the perfusion defect increased in size and intensity, although the chest radiography remained unchanged. The diagnosis of bronchobronchiolitis obliterans (BBO) was confirmed by open lung biopsy. Glucocorticoid therapy was ineffective, and she died of respiratory failure a month after discharge. It is noteworthy that perfusion scintigram demonstrated the progression of the disease much better than chest radiography, which showed no apparent change during the observation period.