일란성 쌍둥이에서 발견된 미만성 범세기관지염

Diffuse panbronchiolitis (DPB) is a recently described entity characterized by chronic inflammation localized just distal to the terminal bronchiole, known as the transitional zone between the airway and the pulmonary parenchyma. The chief manifestations of the disease are chronic cough, exertional dyspnea, wheezing and hypoxemia. The condition carries a poor prognosis in cases detected in earlier studies, probably because only advanced cases were diagnosed. With the development of better knowledge of the disease, milder forms with lesser symptoms and minimal signs are being recognized. Differential diagnosis from diseases which are ordinarily grouped as COPD is important, and it is possible to diagnose the disease by considering its characteristic clinical findings. However, the precise etiology of this disease remains unclear. In East Asia, this is not a newly occurring one, but in the past it had been diagnosed as COPD. DPB is not rare, and because of its poor prognosis and characteristic features, it is a very important entity in the treatment of pulmonary disease. An interesting fact about DPB is that similar diseases have not been described in English literature. Some cases of familial DPB were reported in Japan, and it is usual for some siblings of the patients to have chronic paranasal sinusitis only. This suggests that the disease may have a genetic background and racial susceptibility. We experienced two cases of DPB in monozygotic twins which is, to the best of our knowledge, the first in the world. We report that with literature review.