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Ann Thorac Med. 2019 Jan-Mar;14(1):94-98. doi: 10.4103/atm.ATM_106_18.

Idiopathic pleuroparenchymal fibroelastosis: The first case to be managed with a successful lung transplant at King Faisal Specialist Hospital and Research Center, Riyadh.

Author information

1
Alfaisal Medical School, Alfaisal University, Riyadh, Saudi Arabia.
2
Section of Thoracic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
3
Department of Histopathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
4
Section of Pulmonary Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

Abstract

Idiopathic Pleuro-Parenchymal Fibroelsatosis (PPFE) is a rare, progressive and recently recognized subtype of idiopathic interstitial lung disease with no recorded successful treatment other than lung transplant. We report a case of idiopathic pleuroparenchymal fibroelastosis from the Middle East, managed successfully by bilateral lung transplant performed on a 26 year old Saudi male.

KEYWORDS:

Interstitial lung disease; lung transplant; pleuroparenchymal fibroelastosis

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