The Syndrome of Combined Pulmonary Fibrosis and Emphysema

نویسندگان

  • Ines Zendah
  • Houda Gharsalli
  • Amel Khattab
  • Habib Ghedira
چکیده

A syndrome associating an upper-lobe emphysema and pulmonary fibrosis of the lower lung was recently characterized. Few cases were identified in the literature. We report a case of a 68 year- old smoker man presented for exacerbation of a severe dyspnea. Physical examination revealed basal crackles and finger clubbing. Blood gas analysis showed hypoxemia. Chest radiography showed features compatible with emphysema of the upper lobes and fibrosis of the basis. Chest computed tomography confirmed chest radiography's findings and revealed fibrosis. The diagnosis of idiopathic pulmonary fibrosis was made. Pulmonary function tests showed obstructive pattern. Systolic pulmonary arterial pressure was elevated up to 87 mm Hg on heart ultrasonography. The authors emphasize the importance of the diagnosis of this entity made through chest computed tomography and the fact that it is characterized by severe impairment of gas exchange, the high prevalence of pulmonary hypertension and poor survival.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Efficacy of aerobic physical retraining in a case of combined pulmonary fibrosis and emphysema syndrome: a case report

INTRODUCTION Combined pulmonary fibrosis and emphysema has recently been recognized as a syndrome but remains under-diagnosed. Neither clinical management nor therapeutic approaches have been clearly defined. Pulmonary rehabilitation has not been considered within the therapeutic options for combined pulmonary fibrosis and emphysema. In this case we explored the potential benefits of a specific...

متن کامل

Clinical characteristics in Combined Pulmonary Fibrosis and Emphysema Syndrome vs Idiopathic Pulmonary Fibrosis patients

Abstract: Aim: To identify differences between combined pulmonary fibrosis and emphysema syndrome (CPFE) and idiopathic pulmonary fibrosis (IPF) by a retrospective comparison of clinical data including clinical characteristics and baseline changes in pulmonary function. Methods: We studied demographic, clinical and physiologic features for two groups of patients, (total N=26) those with CPFE (7...

متن کامل

Combined Pulmonary Fibrosis and Emphysema (CPFE)

Emphysema is characterized by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. However, coincidental idiopathic pulmonary fibrosis (IPF) and emphysema was firstly reported in 1990 by Wiggins et al (Wiggins J,...

متن کامل

Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome.

This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68+/-9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart cathe...

متن کامل

Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome

A 30-year-old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleural region along with emphysema predominantly in the upper lobes. Lung biopsy specimens showed a ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 10  شماره 

صفحات  -

تاریخ انتشار 2011