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  A new definition: Combined pulmonary fibrosis and emphysema syndrome [Respir Case Rep]
Respir Case Rep. 2017; 6(2): 110-113 | DOI: 10.5505/respircase.2017.93585  

A new definition: Combined pulmonary fibrosis and emphysema syndrome

Dildar Duman, Hakan Günen
Süreyyapaşa Chest Diseases and Thoracic Surgery Trainig and Research Hospital, Istanbul, Turkey

Combined pulmonary fibrosis and emphysema syndrome (CPFE) is a newly defined syndrome with unique clinical findings, characterized by presence of emphysema and pulmonary fibrosis seen radiologically. Since awareness of the syndrome is insufficient, CPFE is under-recognized. Male patient presented with progressive dyspnea and history of smoking 60 packs per year. Upper lobe emphysema and lower lobe fibrosis, which are specific for CPFE syndrome, were observed in chest computed tomography scans. Forced expiratory volume 1 was relatively well preserved, but diffusing capacity of the lungs for carbon monoxide was very low, and is an important feature of the disease. Pulmonary hypertension has also been determined to be characteristic finding of the syndrome. Patient was diagnosed as CPFE syndrome based on presence of all characteristic features and case is presented with review of the literature to draw attention to this disease.

Keywords: emphysema, fibrosis, pulmonary hypertension


Yeni bir tanım: Kombine pulmoner fibrozis ve amfizem sendromu

Dildar Duman, Hakan Günen
Süreyyapaşa Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim Araştırma Hastanesi, İstanbul

Kombine pulmoner fibrozis ve amfizem sendromu (KPFA), kendine özgü klinik bulguları olan ve radyolojik olarak üst lob amfizemi ve alt lob fibrozisi ile karakterize yeni tanımlanan bir sendromdur. Hastalık iyi bilinmediği için yeterince tanı konulamamaktadır. Progresif nefes darlığı şikayetiyle başvuran, 60 paket/yıl sigara öyküsü olan erkek hastanın çekilen toraks tomografisinde KPFA sendromuna özgü üst loblarda amfizem ve alt loblarda fibrozis izlendi. Hastalığın bir diğer önemli özeliği olarak FEV1 kısmen korunmuş, DLCO ise oldukça düşük bulundu. hastada pulmoner hipertansiyon saptandı. Tüm bulgularıyla KPFA sendromu tanısı konulan olgu, bu sendromuna dikkat çekmek için literatür eşliğinde sunuldu.

Anahtar Kelimeler: amfizem, fibrozis, pulmoner hipertansiyon


Dildar Duman, Hakan Günen. A new definition: Combined pulmonary fibrosis and emphysema syndrome. Respir Case Rep. 2017; 6(2): 110-113

Corresponding Author: Dildar Duman, Türkiye


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