초록
특발성 세기관지중심성 간질성 폐렴은 염증 및 섬유화가 주로 소기도 및 주변을 침범하고, 부분적으로 폐포로 퍼져나가는 특징을 보이는 질환으로 아직까지 국내에서는 발표된 증례가 없다. 저자들은 한 달간의 호흡곤란으로 내원한 남자에서 방사선학적으로 망상형 병변과 중심소엽성 결절을 보이면서 조직 검사상 세기관지에 집중된 병리 소견을 보여 세기관지중심성 간질성 폐렴을 진단한 예를 경험하였기에 보고하는 바이다.
Idiopathic bronchiolocentric interstitial pneumonia is one of idiopathic interstitial pneumonia, which has a relatively aggressive course and poor prognosis. It is characterized by diffuse centrilobular nodules radiologically with mainly bronchiolocentric inflammation and fibrosis associated with patchy alveolitis lacking interstitial granuloma histologically. This disorder is a recently classified disease category, and to our knowledge, there is no case report in Korea. We present a case of idiopathic bronchiolocentric interstitial pneumonia. A 62-year-old man presented with exertional dyspnea with a 1 month duration. The radiological findings showed extensive centrilobular lesions at both lungs. The surgical lung biopsy specimen demonstrated a centrilobular inflammatory process with small airway fibrosis and inflammation partially radiating into the interstitium. Therefore, the patient was diagnosed with idiopathic bronchiolocentric interstitial pneumonia. He was treated with immunosuppressants including steroids and azathioprine. However, his symptoms did not improve and he expired 7 months later due to an acute exacerbation of the interstitial pneumonia and probable infectious pneumonia.
