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Questions and Answers
What is the main characteristic of interstitial lung disease regarding lung function?
What is the main characteristic of interstitial lung disease regarding lung function?
Which imaging finding is commonly associated with interstitial lung disease?
Which imaging finding is commonly associated with interstitial lung disease?
What does the term 'pulmonary fibrosis' strictly refer to?
What does the term 'pulmonary fibrosis' strictly refer to?
What percentage of interstitial lung disease cases have an unknown aetiology?
What percentage of interstitial lung disease cases have an unknown aetiology?
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What is a common clinical feature of interstitial lung disease?
What is a common clinical feature of interstitial lung disease?
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Why is it important to seek an active diagnosis for interstitial lung disease?
Why is it important to seek an active diagnosis for interstitial lung disease?
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Which characteristic is NOT associated with the restrictive pattern of lung function in interstitial lung disease?
Which characteristic is NOT associated with the restrictive pattern of lung function in interstitial lung disease?
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What percentage of interstitial lung disease cases is known to be caused by identifiable aetiologies?
What percentage of interstitial lung disease cases is known to be caused by identifiable aetiologies?
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What is a common symptom of interstitial lung disease?
What is a common symptom of interstitial lung disease?
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Which pathological pattern is associated with Idiopathic Pulmonary Fibrosis?
Which pathological pattern is associated with Idiopathic Pulmonary Fibrosis?
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Which demographic is most commonly affected by Idiopathic Pulmonary Fibrosis?
Which demographic is most commonly affected by Idiopathic Pulmonary Fibrosis?
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What is a distinguishing feature of Desquamative Interstitial Pneumonia (DIP)?
What is a distinguishing feature of Desquamative Interstitial Pneumonia (DIP)?
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Which sign is commonly observed in patients with interstitial lung disease?
Which sign is commonly observed in patients with interstitial lung disease?
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What type of interstitial pneumonia has a less severe fibrosis and an even distribution?
What type of interstitial pneumonia has a less severe fibrosis and an even distribution?
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What can indicate right heart failure in patients with interstitial lung disease?
What can indicate right heart failure in patients with interstitial lung disease?
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What is a potential symptom of interstitial lung disease in the absence of dyspnoea?
What is a potential symptom of interstitial lung disease in the absence of dyspnoea?
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What is currently recognized as the primary event leading to fibroblast activation in interstitial lung disease?
What is currently recognized as the primary event leading to fibroblast activation in interstitial lung disease?
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Which cytokine is NOT mentioned as a fibrogenic factor involved in the pathogenesis of interstitial lung disease?
Which cytokine is NOT mentioned as a fibrogenic factor involved in the pathogenesis of interstitial lung disease?
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Which of the following classifications is associated with the incorporation of exudates into the walls affecting the bases of the lungs?
Which of the following classifications is associated with the incorporation of exudates into the walls affecting the bases of the lungs?
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Which systemic disease is commonly associated with the exudative form of interstitial lung disease?
Which systemic disease is commonly associated with the exudative form of interstitial lung disease?
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What type of fibrosis is characterized by the proliferation of myofibroblasts producing extracellular matrix?
What type of fibrosis is characterized by the proliferation of myofibroblasts producing extracellular matrix?
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In granulomatous interstitial lung disease, which regions of the lungs are typically spared by the formation of granulomas?
In granulomatous interstitial lung disease, which regions of the lungs are typically spared by the formation of granulomas?
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Which of the following is considered an example of pneumoconiosis associated with interstitial lung disease?
Which of the following is considered an example of pneumoconiosis associated with interstitial lung disease?
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Which condition is categorized under the granulomatous classification of interstitial lung disease?
Which condition is categorized under the granulomatous classification of interstitial lung disease?
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What is a common finding in lung function tests for interstitial lung disease?
What is a common finding in lung function tests for interstitial lung disease?
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Which imaging finding is typical for diagnosing idiopathic pulmonary fibrosis?
Which imaging finding is typical for diagnosing idiopathic pulmonary fibrosis?
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What triggers interstitial lung injury in idiopathic pulmonary fibrosis?
What triggers interstitial lung injury in idiopathic pulmonary fibrosis?
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In chronic extrinsic allergic alveolitis, repeated exposure to antigens leads to which immune response type?
In chronic extrinsic allergic alveolitis, repeated exposure to antigens leads to which immune response type?
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Which of the following conditions is associated with honeycomb lung?
Which of the following conditions is associated with honeycomb lung?
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What findings would you expect in a lung biopsy of an individual with extrinsic allergic alveolitis?
What findings would you expect in a lung biopsy of an individual with extrinsic allergic alveolitis?
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Which complication is most directly associated with interstitial fibrosis in the lungs?
Which complication is most directly associated with interstitial fibrosis in the lungs?
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Asbestos-related diseases typically follow what kind of exposure pattern?
Asbestos-related diseases typically follow what kind of exposure pattern?
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What is a major histological feature associated with asbestosis?
What is a major histological feature associated with asbestosis?
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What percentage of patients with chronic extrinsic allergic alveolitis may eventually develop honeycomb lung?
What percentage of patients with chronic extrinsic allergic alveolitis may eventually develop honeycomb lung?
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Which statement describes a characteristic of pulmonary hypertension due to interstitial lung disease?
Which statement describes a characteristic of pulmonary hypertension due to interstitial lung disease?
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What type of lung biopsy technique is commonly used for diagnosing interstitial lung disease?
What type of lung biopsy technique is commonly used for diagnosing interstitial lung disease?
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What genetic abnormality is associated with some cases of idiopathic pulmonary fibrosis?
What genetic abnormality is associated with some cases of idiopathic pulmonary fibrosis?
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Study Notes
Interstitial Lung Disease (ILD)
- ILD is a heterogeneous group of diseases affecting the lungs, some common, some rare
- It represents ~15% of respiratory clinical cases
- Characterized by diffuse parenchymal lung injury/inflammation, progressing to fibrosis
- Airway structures remain unaffected
ILD Terminology
- Often used synonymously with pulmonary fibrosis
- Strictly, ILD is used only when histology shows fibrosis
- Most diffuse lung diseases cause varying degrees of fibrosis
Common ILD Features
- Clinical: Dry cough and shortness of breath (dyspnea)
- Lung Function: Restrictive pattern (reduced lung expansion, capacity, and compliance)
- Radiology: Reticular & nodular shadows on X-ray and high-resolution CT scans of the chest
Lung Function: Restrictive Pattern
- Restriction of airflow due to reduced lung parenchyma expansion
- Reduced total lung capacity, vital capacity, and residual volume
- Reduced lung compliance
- May show decreased Forced Vital Capacity (FVC)
- May show normal or increased ratio of FEV1/FVC
- Measurement of carbon monoxide transfer (carbon monoxide diffusion capacity) may also be abnormal
ILD Pathogenesis
- For many years, inflammation in the septa (alveolitis) and progressive diffuse septal/interstitial fibrosis were thought to be the cause
- The focus now is on the role of epithelial cell injury, leading to fibroblast activation and eventually fibrosis
ILD Pathogenesis (cellular mechanisms)
- Injury to alveolar epithelial and/or endothelial cells (e.g. from pollutants)
- Activation of inflammatory cells (macrophages, lymphocytes, eosinophils, mast cells)
- Release of fibrogenic cytokines and growth factors (TNF-α, IL-4, TGF-β, endothelin-1)
- Proliferation of fibroblasts, and myofibroblasts, leading to increased extracellular matrix production and fibrosis
ILD Classification
-
Exudative:
- Idiopathic pulmonary fibrosis (IPF, or cryptogenic fibrosing alveolitis)
- Systemic diseases (e.g., connective tissue disorders like rheumatoid arthritis, systemic sclerosis)
- Pneumoconioses (e.g., asbestosis)
-
Granulomatous:
- Sarcoidosis
- Extrinsic allergic alveolitis (hypersensitivity pneumonitis)
- Pneumoconioses (e.g., silicosis)
Simplified ILD Classification
- Describes different ILD categories based on clinical presentation
- Includes information on demographics and common pathology patterns
ILD Presentation
-
Symptoms:
- Shortness of breath (dyspnea) on exertion, fatigue, cough
-
Signs:
- Clubbing of fingers
- Bilateral end-expiratory crackles(Velcro-like)
- Signs of right-sided heart failure
ILD Other Features
- Fatigue may occur even without dyspnea
- Cough may be the only symptom
- Non-specific systemic symptoms such as fever and weight loss
- Incidental abnormalities of lung function tests
- An abnormal X-ray in the absence of symptoms
ILD Diagnosis Criteria
- Abnormal lung function tests showing restriction (reduced vital capacity) and impaired gas exchange
- Evidence of basilar reticular abnormalities and/or ground-glass appearance on high-resolution CT scans
- Lung biopsy (or bronchoalveolar lavage) showing no alternative diagnosis
ILD Biopsy Techniques
- Open lung biopsy
- Video-assisted thoracic surgery (VATS)
Common ILD Conditions
- Idiopathic Pulmonary Fibrosis (IPF)
- Extrinsic Allergic Alveolitis
- Asbestos Related Disease
Idiopathic Pulmonary Fibrosis (IPF)
- Insidious onset of dyspnea and tachycardia
- Most common in the 40s and 50s
- Unknown aetiology, pathogenesis uncertain
- Some genetic abnormalities (e.g., in mucin gene MUC5B, Serum surfactant protein C, and telomerase)
Honeycomb Lung
- A late stage of chronic interstitial fibrosis
- Commonest causes include IPF, extrinsic allergic alveolitis and sarcoidosis
Extrinsic Allergic Alveolitis (Hypersensitivity Pneumonitis)
- Caused by an immune response to inhaled antigens
- Characterized by acute and/or chronic manifestations and a specific/predictable exposure history
- Can present with acute exposure to antigen:
- Dyspnea, fever, cough 4-8 hours after exposure, resolving within 12-24 hours
- Or with chronic exposure to antigen:
- Interstitial fibrosis; small granulomas in 70%; lymphocytes, eosinophils, mast cells and interstitial fibrosis; eventually honeycomb lung in 5% of cases
- Lab findings: specific cell pattern in bronchoalveolar lavage (BAL) samples
Asbestos Related Disease
- Occupational exposure (mining, insulation, dockyards)
- High exposure risk with high duration and/or intensity of exposure to various types of asbestos
- Risk of disease depends on asbestos fibre type and size ( > 8um)
- Symptoms and diseases include pleural plaques, effusion, thickening, asbestosis, mesothelioma, and lung cancer
- Asbestos bodies: Long thin asbestos fibres coated with haemosiderin and protein that form distinctive structures. These structures are often present in sputum.
ILD Outcome & Prognosis
- Complications: Fibrosis greatly reduces the pulmonary capillary network leading to pulmonary hypertension and right heart failure (cor pulmonale)
-
Prognosis:
- Mild focal fibrosis has a 10% recovery rate with minimal residual respiratory dysfunction
- Marked interstitial fibrosis/honeycomb lung has a poor prognosis with likely mortality due to respiratory and/or cardiac failure within 20%, or death from chronic severe respiratory impairment in 70% of cases in acute phase
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Description
This quiz examines key characteristics, clinical features, and diagnostic criteria related to interstitial lung disease (ILD). Test your understanding of its lung function impact, common imaging findings, and etiology recognition. Ideal for medical students and healthcare professionals looking to reinforce their knowledge of ILD.
