The worst of both worlds-combined pulmonary fibrosis and emphysema syndrome

Aqeel, Sheeba Habeeb Ba; Biswas, Abhishek; Pothapregada, Sriram

doi:10.21037/atm.2016.05.26

Cited by 3 publications

(2 citation statements)

References 26 publications

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“…Although there is not yet a consensus definition, the characteristic clinical features are used as a guide in diagnosis of this disease: male gender, heavy smoking, partial preservation observed in a PFT, marked reduction in DLCO, presence of severe symptoms, pulmonary hypertension, and a high incidence of LC. [8] Smoking is considered to be the most important risk factor in the etiology of CPFE. [1,2,9] A history of smoking was found in 98% of 607 patients in a meta-analysis.…”

Section: Discussionmentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema Syndrome Complicated by Lung Cancer

Gürbüz¹

2018

South Clin Ist Euras

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Combined pulmonary fibrosis and emphysema syndrome (CPFE) is a rare disease characterized by emphysema in the upper lobes of the lung and fibrosis in the lower lobes. CPFE is an important risk factor for lung cancer (LC). Although CPFE might increase the risk of LC, the prevalence of CPFE in patients with LC and the incidence of LC in patients with CPFE are unknown. LC in patients with CPFE (LC-CPFE) is associated with poor survival outcomes. The aim of this study was to present a case with CPFE to draw attention to the possibility that it may be accompanied by LC.

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Section: Discussionmentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema Syndrome Complicated by Lung Cancer

Gürbüz¹

2018

South Clin Ist Euras

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“…Fibrosis features of CPFE include lower lobe fibrotic, honeycombing, reticulation and traction bronchiectasis. (31) Based on HRCT, COPD has the highest emphysema score, followed by CPFE, and IPF with the lowest score. Fibrosis scores were generally higher in CPFE and IPF than in COPD.…”

Section: Radiology Imagingmentioning

confidence: 98%

Diagnosis and treatment of Combined Pulmonary Fibrosis and Emphysema (CPFE)

Wesnawa¹

2023

World J. Adv. Res. Rev.

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Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combination pulmonary fibrosis and emphysema which is now widely found. Clinical characteristics of the CPFE are current or former heavy smoker, predominantly male, and symptom of shortness of breath on exertion. The standard diagnosis of CPFE is based on high resolution computed tomography (HRCT) with emphysema in the upper lung zone and fibrosis in the lower lung zone. The lung function profile in CPFE is different from patients with emphysema or pulmonary fibrosis alone. The lung volumes in CPFE is preserved, but there is significant reduction in lung diffusion capacity. CPFE has a poor prognosis. The presence of pulmonary hypertension in CPFE is a poor prognostic factor. Because there is no current standard treatment of CPFE, the focus of management is supportive therapy, management of complications, and management of exacerbation CPFE.

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