A new definition: Combined pulmonary fibrosis and emphysema syndrome

Abstract

ÖzetKombine 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 kıs-men 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 Sözcükler: Amfizem, fibrozis, pulmoner hipertansiyon. AbstractCombined 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.

Combined Pulmonary Fibrosis and Emphysema Syndrome Complicated by Lung Cancer

“…Recently, cases of CPFE have been published in the literature. [6,7] CPFE is a newly identified syndrome characterized by specific clinical findings and radiological identification with upper lobe emphysema and lower lobe fibrosis. Since the disease is not well known, diagnosis remains inadequate.…”

“…It is thought that 35% of all idiopathic pulmonary fibrosis (IPF) patients are actually cases of CPFE, and in current studies, high rates of emphysema (8-50%) have been reported in patients whose high-resolution computed tomography (HRCT) revealed the presence of pulmonary fibrosis [2,10,11] It is important to be able to define this disease, as the clinical course of CPFE differs from that of IPF. [7] CPFE syndrome is radiologically characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes. In the upper lobes, paraseptal emphysema is more frequent, but centrilobular emphysema and bullous changes may be also seen.…”

“…Mortality is a particular concern in the presence of pulmonary hypertension. [7,[12][13][14] CPFE is an important risk factor for LC because most CPFE patients are smokers. [4] Although CPFE may increase the risk of LC, the prevalence of CPFE in patients with LC and the incidence of LC in patients with CPFE are unknown.…”