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Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia:A case report

World Journal of Clinical Cases 2020年第18期8卷 4186-4192页

作者： Jae-Ha lee Hang-Jea Jang Jin-Han Park Hyun-Kuk Kim sunggun lee Ji-Yeon Kim Seong-Ho Kim Department of Internal Medicine Haeundae Paik HospitalInje University College of MedicineBusan 48108South Korea Department of Pathology Haeundae Paik HospitalInje University College of MedicineBusan 48108South Korea

BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal ***,there have been increasing reports of PPFE,and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper ***,cases of unilateral PPFE are *** SUMMARY A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional *** patient’s symptoms started 6 mo previously and had been gradually *** the time of presentation,he felt dyspnea when walking at his own *** findings suggested PPFE,but the lesion was localized in the upper lobe of the right *** multidisciplinary discussion,a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis,and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis,which met the histopathologic criteria of definite *** multidisciplinary discussion in an experienced interstitial lung disease center,we confirmed the diagnosis of unilateral ***,we confirmed the progression of PPFE on radiologic findings during the followup *** Clinicians should consider PPFE,even in cases with unilateral,predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.

关键词： Interstitial lung disease Pleuroparenchymal fibroelastosis Progression Unilateral Multidisciplinary discussion Case report

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