لخّصلي

Introduction Idiopathic Pulmonary Fibrosis (IPF) is defined as a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of usual interstitial pneumonia (UIP) on surgical (thoracoscopic or open) lung biopsy

The precise incidence and prevalence of IPF are not known.High-resolution CT scanning has changed the diagnostic assessment(2) toxins, environmental exposures, and collagen vascular disorders, (3) donum characteristics on conventional chest or high-resolution computed tomography (HRCT) scans, (4) abnormal lung function studies showing restriction (decreased total lung capacity [TLC], or decreased vital capacity [VC] with normal or increased FEV1/FVC ratio) and/or impaired gas exchange (alveolar arterial pressure difference of 02), decreased Pa02 with rest or exercise.GGO should be considered an active process only when there are no HRCT findings associated with fibrosis Discussion Several acute and chronic lung disorders with varying degrees of pneumonia and fibrosis are collectively referred to as interstitial lung diseases (ILDs).Results

The following high-resolution CT findings were noted: predominance of peripheral (100%) and inferior (80%) lesion distribution, evidence of intralobular septal thickening (100%), lymphangiectasia (90%), bronchiectasis (90%), and visceral tissue distortion (100%).Surgical lung biopsy is essential for a definitive clinical-pathological diagnosis except in cases where a typical radiographic clinical picture of heterogeneous interstitial pneumonia/pulmonary fibrosis is present .All images were obtained in suspended inspiration in supine position using 2mm collimation at 10mm interval from apexto the base of the lung at 120 kVp, 45 mA with 2sec exposure time and reconstructed with a bone algorithm.Patients with idiopathic pulmonary fibrosis (IPF) usually present between the ages of 40 and 70 years, and typically present with progressive dyspnea on exertion, chronic cough and dactylism in approximately two-thirds of IPF patients.The most common radiographic finding in idiopathic pulmonary fibrosis, described in approximately 80% of patients, consists of bilateral irregular linear opacities that form a reticular pattern.Conversely, a normal chest radiograph cannot be used to exclude microscopic evidence of UlP on lung biopsy Materials & Methods

A prospective study of ten patients with IPF was done.In individuals with such asymptomatic abnormalities, investigation by physiologic evaluation or by high-resolution CT in diagnosis and management of idiopathic pulm...The earliest histological abnormality in idiopathic pulmonary fibrosis is alveolitis with increased cellularity of the alveolar walls.Furthermore, it has been repeatedly shown that the severity and extent of disease assessed on chest radiograph do not correlate well with functional and clinical impairment.All patients underwent HRCT (TOMOSCAN-EG, PHILIPS, BEST, NETHERLANDS) examination.

Introduction
Idiopathic Pulmonary Fibrosis (IPF) is defined as a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of usual interstitial pneumonia (UIP) on surgical (thoracoscopic or open) lung biopsy

The precise incidence and prevalence of IPF are not known. Previous prevalence estimates for IPF varied from 3 to 6 cases per 100,000 in the general population. Virtually all patients with IPF have an abnormal chest radiograph at the time of presentation . Indeed, basal reticular opacities are often visible on previous chest radiographs in retrospect for several years before the development of symptoms . In individuals with such asymptomatic abnormalities, investigation by physiologic evaluation or by high-resolution CT in diagnosis and management of idiopathic pulm...

scanning (HRCT) could lead to earlier detection and treatment of IPF. Conversely, a normal chest radiograph cannot be used to exclude microscopic evidence of UlP on lung biopsy
Materials & Methods

A prospective study of ten patients with IPF was done. The selection criteria include the patients with chronic respiratory symptoms, chest radiographs suggestive of reticular pattern or spirometric evidence of lung disease. All patients underwent HRCT (TOMOSCAN-EG, PHILIPS, BEST, NETHERLANDS) examination.
All images were obtained in suspended inspiration in supine position using 2mm collimation at 10mm interval from apexto the base of the lung at 120 kVp, 45 mA with 2sec exposure time and reconstructed with a bone algorithm.

‏The study included seven females and three males with idiopathic pulmonary fibrosis. The age range was 42-75 years. The mean age of idiopathic pulmonary fibrosis patients was 59 years for both males and females. The diagnosis was made based on the criteria proposed by the American Thoracic Society (ATS) and the European Respiratory Society (ERS). Pulmonary fibrosis can be diagnosed in the presence of four major and three minor criteria. Major criteria: (1) Exclusion of known causes of infiltrative lung disease such as drug exposure, radiation, and connective tissue diseases. (2) Abnormal pulmonary function tests with evidence of a restrictive pattern. (3) High-resolution CT findings of bibasilar grid with minimal ground-glass opacity. (4) Transbronchial lung biopsy or bronchoalveolar lavage showed no evidence of other disease. Minor criteria: (1) Age greater than 50 years. (2) Insidious onset of dyspnea on exertion. (3) Disease duration of 3 months or more. (4) Bibasilar inspiratory crackles.
Results

The following high-resolution CT findings were noted: predominance of peripheral (100%) and inferior (80%) lesion distribution, evidence of intralobular septal thickening (100%), lymphangiectasia (90%), bronchiectasis (90%), and visceral tissue distortion (100%). These findings are comparable to previous studies. In all patients, the enlargement was bilateral; however, it was asymmetric in three patients. Interlobular septal thickening was noted in only three patients (30%), a relatively rare finding. Two patients (20%) did not show a regional predominance and involved all areas to an equal degree. Pleural thickening was noted in eight patients (80%). Extensive ground-glass opacities (GGO) were noted in 20% of cases and are a rare finding as such. GGO should be considered an active process only when there are no HRCT findings associated with fibrosis
Discussion
Several acute and chronic lung disorders with varying degrees of pneumonia and fibrosis are collectively referred to as interstitial lung diseases (ILDs). Idiopathic pulmonary fibrosis is the most common form of ILD.
The diagnosis of pulmonary fibrosis requires(1) exclusion of other known causes of ILD including drugs in the diagnosis and management of ILDs...
(2) toxins, environmental exposures, and collagen vascular disorders, (3) donum characteristics on conventional chest or high-resolution computed tomography (HRCT) scans, (4) abnormal lung function studies showing restriction (decreased total lung capacity [TLC], or decreased vital capacity [VC] with normal or increased FEV1/FVC ratio) and/or impaired gas exchange (alveolar arterial pressure difference of 02), decreased Pa02 with rest or exercise.

Patients with idiopathic pulmonary fibrosis (IPF) usually present between the ages of 40 and 70 years, and typically present with progressive dyspnea on exertion, chronic cough and dactylism in approximately two-thirds of IPF patients. Pulmonary function testing usually shows a restrictive defect with decreased lung volumes and diffusion capacity. Surgical lung biopsy is essential for a definitive clinical-pathological diagnosis except in cases where a typical radiographic clinical picture of heterogeneous interstitial pneumonia/pulmonary fibrosis is present
. This means that biopsy is not always necessary. Typical interstitial pneumonia is the primary pathological abnormality for the diagnosis of heterogeneous interstitial pneumonia.
The main histological feature and diagnostic criterion is pain. The earliest histological abnormality in idiopathic pulmonary fibrosis is alveolitis with increased cellularity of the alveolar walls.
This inflammatory process can lead to progressive fibrosis. Alveolitis and intraalveolar macrophages in idiopathic pulmonary fibrosis indicate disease activity and may be reversible. Fibrosis and alveolar cells are irreversible.
The mainstay of treatment for patients with idiopathic pulmonary fibrosis has been corticosteroid therapy, which has not been proven effective. The majority of patients diagnosed with idiopathic pulmonary fibrosis develop disease progression with or without treatment, leading to heart, lung and respiratory failure.
In recent years, there has been increasing interest in antifibrotic therapy using agents such as colchicine and pirfenidone.
A relationship between cigarette smoking and pulmonary fibrosis has been suspected for many years.
Almost all patients with idiopathic pulmonary fibrosis have Abnormal chest radiograph at presentation.
The most common radiographic finding in idiopathic pulmonary fibrosis, described in approximately 80% of patients, consists of bilateral irregular linear opacities that form a reticular pattern. Although these opacities may be diffuse, they primarily involve the lower lung regions. Initially, the reticular pattern is fine and granular, and this is described on radiography as a ground-glass appearance.
As fibrosis progresses, the reticular pattern becomes coarser, and there is a gradual loss of lung volume. The honeycomb pattern represents fibrosis in its final stage. The plain radiographic appearance of IPF is nonspecific and misleading, and is similar to that seen in many other interstitial lung diseases. Furthermore, it has been repeatedly shown that the severity and extent of disease assessed on chest radiograph do not correlate well with functional and clinical impairment.
The clinical utility and optimal timing of follow-up chest radiographs are unclear. Radiographs are indicated if clinical deterioration occurs, to assess disease progression, or to identify superimposed infection or malignancy.
High-resolution CT scanning has changed the diagnostic assessment