A Case of Idiopathic Bronchiolitis Obliterans Organizing Pneumonia

원발성 Bronchiolitis Obliterans Organizing Pneumonia 1예

Bronchiolitis obliterans organizing pneumonia (BOOP) is a type of diffuse interstitial lung disease that primarily affects the small conducting airways and characterized by the presence of granulation tissue plugs within the lumen of small airways often extending into alveolar ducts. It is associated with a number of different causes, including a variety of infections, fume exposures, drugs, collagen diseases and idiopathic. Recently we have experienced one patient with idiopathic BOOP. The patient was a 58 year old man presented with 2 months' history of dry cough and exertional dyspnea. The phyical examination showed inspiratory crackles at both lower lung field. Chest X-ray showed bilateral multiple patchy alveolar density. Pulmonary function studies showed a moderate degree of restrictive lung disease. Open lung biopsy carried out and revealed findings characteristic of BOOP. There was a dramatic response clinically and radiologically to high dose predinisolone therapy. Chest X-ray and pulmonary function test under-taken one year later showed marked improvement. New lesion on chest PA was developed during the period of tapering of prednisolone dose, but it was soon disappeared after increasing of prednisolone dose. One year later, he is well without steroid therapy.

본 증례에서와 같이 BOOP는 임상상 및 조직소견에서의 특징과 좋은 예후를 갖는 미만성 침윤성 폐질환의 한 아형이며 원발성 폐섬유화증과는 달리 부신피질스테로이드에 의해 완치될 수 있다는 점에서 이 질환에 대한 인식은 매우 중요하다. 저자들은 부신피질스테로이드를 사용한후 1개월후부터 임상적으로 뚜렷한 호전을 보였으며, 2개월후부터는 흉부 X선 및 폐기능검사상 현저한 호전을 보였으며, 치료 3개월 후 부터는 prednisolne 요량 감소를 시작하였다. 경과도중 증상의 재발없이 일시적으로 흉부 X선상 새로운 병소가 나타났으나 용량을 증가시켜 곧 소실되었다. 치료 1년뒤 Prednisolone 중단 후에도 재발없이 안정상태를 유지하고 있다.