Pleuroparenchymal fibroelastosis (PPFE) is a rare variant of interstitial lung disease (ILD). PPFE is characterizsed by peculiar clinical, radiological and histological features. Initially considered an idiopathic entity, this disease is now classified in the rare category of the recent classification of idiopathic interstitial pneumonias (IIPs). However, it largely continues to be underdiagnosed owing to the lack of awareness about this condition. PPFE may be misdiagnosed as the other commonly diagnosed ILDs, especially IIP. The diagnosis is clinched with lung biopsy. We report a case of PPFE diagnosed by transbronchial lung biopsy.
Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126:2007–13.
Amitani R, Niimi A, Kuse F. Idiopathic pulmonary upper lobe fibrosis (IPUF). Kokyu 1992;11:693–9.
American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2002;165:277–304.
Hamada K, Nagai S, Kitaichi M, Jin G, Shigematsu M, Nagao T, et al. Cyclophosphamide-induced late-onset lung disease. Intern Med 2003;42:82–7.
Nath AR, Capel LH. Inspiratory crackles and mechanical events of breathing. Thorax 1974;29:695–8.
Noh HJ, Seo Y, Huo SM, Kim TJ, Kim HL, Song JS. Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax: a case report. Tuberc Respir Dis 2014;77:184–7.
Singh R, Sundaram P, Joshi JM. Upper lobe fibrosis in ulcerative colitis. J Assoc Physicians India 2003;51:515–7.
Watanabe K, Nagata N, Kitasato Y, Wakamatsu K, Nabeshima K, Harada T, et al. Rapid decrease in vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Invest 2012;50:88–97.
Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis, a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377–85.