A case of broncho-bronchiolitis obliterans associated with rheumatoid arthritis and Sjögren's syndrome
Nemoto, Y.; Katayama, Y.; Sugiyama, K.; Kudo, K.; Matsuoka, T.; Nagata, N.; Takatani, O.; Kikuchi, K.; Aida, S.
Nihon Kyobu Shikkan Gakkai Zasshi 29(9): 1180-1185
1991
ISSN/ISBN: 0301-1542 PMID: 1753545 Document Number: 382367
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.