Is unclassifiable idiopathic interstitial pneumonia a result of idiopathic pleuroparenchymal fibroelastosis?

The present article reports the case of a 51-year-old female patient who was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis in the initial phase of the disease. The upper and lower lobes showed a pleuroparenchymal fibroelastosis pattern and reticular shadow, respectively. Both upper and lower shadows gradually progressed. Five years following the first detection of the chest shadow, left single-lung transplantation was performed. In the multidisciplinary discussion held after the lung transplantation, most participants recognized this end-stage lung disease as unclassifiable idiopathic interstitial pneumonia though the major pathological findings showing a pleuro-parenchymal fibroelastosis pattern. (c) 2020 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.