01-27-2011, 05:06 PM
A 55 years old smoker man due to chronic cough without sputum and dyspnea came to ED, in physical examination fine crackle was heard at both sides of lung bases and finger clubbing was found also. in spirometery a restrictive disease was suggested. in CXRAY honey comb pattern in bilateral lung bases was seen. what is the most probable diagnosis?
A. bronchiectasis
B. histocytosis x
C. lung fibrocystic disease
D. idiopathic lung fibrosis
Idiopathic Pulmonary Fibrosis
IPF is the most common form of idiopathic interstitial pneumonia. Separating IPF from other forms of lung fibrosis is an important step in the evaluation of all patients presenting with ILD. IPF has a distinctly poor response to therapy and a bad prognosis.
Clinical Manifestations
Exertional dyspnea, a nonproductive coughsmiling face, and inspiratory crackles smiling face with or without digital clubbing smiling face may be present on physical examination. The HRCT lung scans typically show patchy, predominantly basilar, subpleural reticular opacities, often associated with traction bronchiectasis smiling face and honeycombing smiling face (Fig. 255-3). Atypical findings that should suggest an alternative diagnosis include: extensive ground-glass abnormality, nodular opacities, upper or mid-zone predominance, and prominent hilar or mediastinal lymphadenopathy. Pulmonary function tests often reveal a restrictive pattern, a reduced DLCO, and arterial hypoxemia that is exaggerated or elicited by exercise.
Histologic Findings
Confirmation of the presence of the UIP (usual interstitial pneumonia) pattern on histologic examination is essential to confirm this diagnosis. Transbronchial biopsies are not helpful in making the diagnosis of UIP, and surgical biopsy is usually required. The histologic hallmark and chief diagnostic criterion of UIP is a heterogeneous appearance at low magnification with alternating areas of normal lung, interstitial inflammation, foci of proliferating fibroblasts, dense collagen fibrosis, and honeycomb changes smiling face. These histologic changes affect the peripheral, subpleural parenchyma most severely. The interstitial inflammation is usually patchy and consists of a lymphoplasmacytic infiltrate in the alveolar septa, associated with hyperplasia of type 2 pneumocytes. The fibrotic zones are composed mainly of dense collagen, although scattered foci of proliferating fibroblasts are a consistent finding. The extent of fibroblastic proliferation is predictive of disease progression. Areas of honeycomb change smiling face are composed of cystic fibrotic air spaces that are frequently lined by bronchiolar epithelium and filled with mucin. Smooth-muscle hyperplasia is commonly seen in areas of fibrosis and honeycomb change smiling face. A fibrotic pattern with some features similar to UIP may be found in the chronic stage of several specific disorders such as: pneumoconioses (e.g., asbestosis), radiation injury, certain drug-induced lung diseases (e.g., nitrofurantoin), chronic aspiration, sarcoidosis, chronic hypersensitivity pneumonitis, organized chronic eosinophilic pneumonia, and PLCH. Commonly, other histopathologic features are present in these situations thus allowing separation of these lesions from the UIP-like pattern. Consequently, the term UIP is used for those patients in whom the lesion is idiopathic and not associated with another condition.
Patients with IPF may suffer acute deterioration secondary to infections, pulmonary embolism, pneumothorax, or heart failure. These patients also commonly suffer an accelerated phase of rapid clinical decline that is associated with a poor prognosis (so-called acute exacerbations of IPF). These acute exacerbations are defined by: worsening of dyspnea within a few days to 4 weeks; newly developing diffuse radiographic opacities; worsening hypoxemia; and absence of infectious pneumonia, heart failure, and sepsis. The rate of these acute exacerbations ranges from 10–57%, apparently depending on the length of follow-up. During these episodes, the histopathologic pattern of diffuse alveolar damage is often found on the background of UIP.
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A. bronchiectasis
B. histocytosis x
C. lung fibrocystic disease
D. idiopathic lung fibrosis
Idiopathic Pulmonary Fibrosis
IPF is the most common form of idiopathic interstitial pneumonia. Separating IPF from other forms of lung fibrosis is an important step in the evaluation of all patients presenting with ILD. IPF has a distinctly poor response to therapy and a bad prognosis.
Clinical Manifestations
Exertional dyspnea, a nonproductive coughsmiling face, and inspiratory crackles smiling face with or without digital clubbing smiling face may be present on physical examination. The HRCT lung scans typically show patchy, predominantly basilar, subpleural reticular opacities, often associated with traction bronchiectasis smiling face and honeycombing smiling face (Fig. 255-3). Atypical findings that should suggest an alternative diagnosis include: extensive ground-glass abnormality, nodular opacities, upper or mid-zone predominance, and prominent hilar or mediastinal lymphadenopathy. Pulmonary function tests often reveal a restrictive pattern, a reduced DLCO, and arterial hypoxemia that is exaggerated or elicited by exercise.
Histologic Findings
Confirmation of the presence of the UIP (usual interstitial pneumonia) pattern on histologic examination is essential to confirm this diagnosis. Transbronchial biopsies are not helpful in making the diagnosis of UIP, and surgical biopsy is usually required. The histologic hallmark and chief diagnostic criterion of UIP is a heterogeneous appearance at low magnification with alternating areas of normal lung, interstitial inflammation, foci of proliferating fibroblasts, dense collagen fibrosis, and honeycomb changes smiling face. These histologic changes affect the peripheral, subpleural parenchyma most severely. The interstitial inflammation is usually patchy and consists of a lymphoplasmacytic infiltrate in the alveolar septa, associated with hyperplasia of type 2 pneumocytes. The fibrotic zones are composed mainly of dense collagen, although scattered foci of proliferating fibroblasts are a consistent finding. The extent of fibroblastic proliferation is predictive of disease progression. Areas of honeycomb change smiling face are composed of cystic fibrotic air spaces that are frequently lined by bronchiolar epithelium and filled with mucin. Smooth-muscle hyperplasia is commonly seen in areas of fibrosis and honeycomb change smiling face. A fibrotic pattern with some features similar to UIP may be found in the chronic stage of several specific disorders such as: pneumoconioses (e.g., asbestosis), radiation injury, certain drug-induced lung diseases (e.g., nitrofurantoin), chronic aspiration, sarcoidosis, chronic hypersensitivity pneumonitis, organized chronic eosinophilic pneumonia, and PLCH. Commonly, other histopathologic features are present in these situations thus allowing separation of these lesions from the UIP-like pattern. Consequently, the term UIP is used for those patients in whom the lesion is idiopathic and not associated with another condition.
Patients with IPF may suffer acute deterioration secondary to infections, pulmonary embolism, pneumothorax, or heart failure. These patients also commonly suffer an accelerated phase of rapid clinical decline that is associated with a poor prognosis (so-called acute exacerbations of IPF). These acute exacerbations are defined by: worsening of dyspnea within a few days to 4 weeks; newly developing diffuse radiographic opacities; worsening hypoxemia; and absence of infectious pneumonia, heart failure, and sepsis. The rate of these acute exacerbations ranges from 10–57%, apparently depending on the length of follow-up. During these episodes, the histopathologic pattern of diffuse alveolar damage is often found on the background of UIP.
HAVE FUN.