Living-donor lobar lung transplantation for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of a case.

Diffuse interstitial pneumonia (IP) associated with collagen disease is a rare indication for lung transplantation. The manifestations of collagen disease are variable and dermatomyositis (DM) is often considered a contraindication for lung transplantation because of active myositis and a high incidence of malignancy. Furthermore, clinically amyopathic dermatomyositis (C-ADM) is associated with rapidly progressive IP resulting in a poor prognosis. Bilateral living-donor lobar lung was transplanted in a 52-year-old female with rapidly progressive IP associated with C-ADM, and the postoperative course was uneventful. To our knowledge, this case represents the first living-donor lobar lung transplantation for a patient with rapidly progressive IP associated with C-ADM.