Interstitial Lung Disease Flashcards by Sarah Moore

What are idiopathic interstitial lung diseases?

Sarcoidosis

Idiopathic pulmonary fibrosis

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What are drug induced interstitial lung diseases?

Methotrexate
Amiodarone
Bleomycin

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What are pneumoconiosis interstitial lung diseases?

Asbestosis
Silicosis
Coal Worker’s Lung

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What are Connective tissue disease

interstitial lung diseases?

Polymyositis
Dermatomyositis
SLE
Rheumatoid arthritis

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Define idiopathic pulmonary fibrosis. When does it occur and what is the survival rate?

Chronic inflammation of alveolar walls with progressive fibrosis
Most commonly occurs between 50-70 yr
Survival is 3-5 years after diagnosis

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What are other names for idiopathic pulmonary fibrosis?

Interstitial lung disease (ILD)
Cryptogenic fibrosing alveolitis
Diffuse interstitial fibrosis
Idiopathic interstitial pneumonitis

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What is the pathophys of idiopathic pulmonary fibrosis?

Accumulation of excessive fibrous or connective tissue in lung parenchyma
Result of inflammatory, immune and fibrotic processes
Inflammation is responsible for injury and fibrosis, distorts & impairs the structure and function of alveolar-capillary gas exchange surface
Lungs become stiff and difficult to ventilate
Diffusion capacity of alveoli-capillary membrane decreases  hypoxemia

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What causes need to be excluded to diagnose idiopathic pulmonary fibrosis?

Environmental or occupational exposure
A. Coal dust, asbestos, silica, iron
Connective tissue diseases
A. Rheumatoid arthritis, SLE
Radiation-induced
Drug-induced
A. Amiodarone, methotrexate, cocaine, heroin, chemo drugs

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What are the sxs of interstitial lung disease?

Dyspnea with rapid shallow breathing
Dry hacking cough
Fatigue
Inspiratory rales on auscultation
Clubbing
Cyanosis
Pulmonary HTN

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What are the PFT results for interstitial lung disease>

↓ TLC

Normal FEV1/FVC

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What are the CXR results for idiopathic pulmonary fibrosis?

Diffuse interstitial lung disease with fibrosis and honeycombing (cystic opacities)
Reticular/nodular opacities are hallmark
usually bilateral

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What are the CT results for iinterstitial lung disease?

Patchy distribution of ground glass in lung parenchyma

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What are the surgical lung biopsy results in interstitial lung disease?

Mixed areas of normal tissue, interstitial inflammation, fibrosis and honeycombing

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What are the treatment options for idiopathic pulmonary fibrosis? What is definitive treatment?

O2 to improve hypoxemia
Corticosteroids to reduce inflammatory response
Pulmonary rehab
Lung transplantation is definitive treatment
A. 5 yr survival rate estimated at 50%

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What si the prognosis for idiopathic pulmonary fibrosis? Who has a better prognosis?

Usually poor response to treatment

Best prognostic factors include:
Young female with less than average dyspnea and hypoxemia
No smoking history
Positive response to corticosteroids

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What are the complications for idiopathic pulmonary fibrosis?

Respiratory failure
Heart failure
Pulmonary embolism
Pneumonia
Lung cancer

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Define Pneumoconiosis

Chronic restrictive lung disease caused by the inhalation of dust or various inert, inorganic or silicate dusts

Clinically important diseases include coal worker’s lung, silicosis, asbestosis, siderosis

When does silicosis occur?

Follows long term inhalation of small particles of silica
Associated with:
Metal mining (Pb, Cu, Ag, Au),
Pottery making
Sandstone & granite cutting
Sandblasting

What is the pathophys of silicosis?

Alveolar macrophages engulf inhaled particles  enter lymphatics and interstitial tissue

Macrophages cause cytotoxic enzymes to be released  fibrosis of lung parenchyma

When macrophage dies, particles are released and engulfed by other macrophages  process keeps repeating

What is the clinical presntation of simple cases of silicosis?

Asymptomatic

20-30 years exposure necessary before disease becomes apparent

What is the clinical presntation of complicated cases of silicosis?

Dyspnea, rales, clubbing, cyanosis

Related to amount of exposure

What are the cxr results of silicosis?

Small nodular opacities at upper lung fields
Calcified hilar lymph nodes
“eggshell” calcification strongly suggests silicosis

What is the treatment for silicosis?

No effective treatment 
Supportive therapy
Oxygen
Immunizations 
Pulmonary rehab

What is the prophylaxis for silicosis?

Effective dust control
External hoods & venting of dust
Surveillance of exposed workers with CXR
Frequency depends on type & amt of exposure

What are the complications of silicosis?

Increased risk of TB Function of alveolar macrophages can be permanently compromised Progressive fibrosis

Define coal workers lung

Diffuse nodular deposition of dust in lungs | Results from long term exposure to coal dust in coal mining

What is the patho phys of coal miners disease?

Coal dust is widely distributed throughout lung  development of coal macules around bronchioles

Differentiate between simple and complicated coal miners disease

Simple disease Mild dilation of bronchioles Not associated with airflow obstruction PFT’s not impressive Complicated disease Progressive massive fibrosis develops as amorphous black mass that compromises and destroys airways and vascular beds

Differentiate between the sxs of simple and complicated coal miners disease

Simple Disease Asymptomatic Underground exposure of 20 years before sx’s start to develop Complicated Disease Dyspnea on exertion “Black lung”

What is the treatment for coal miners disease?

``` No effective treatment Supportive therapy Oxygen Immunizations Pulmonary rehab ```

What is the prophylaxis for coal miners disease?

Suppress coal dust for prevention

What are the complications of coal miners disease?

Progressive massive fibrosis

Define asbestosis

``` Diffuse interstitial lung disease that results from long term inhalation of asbestos dust Mining Milling Manufacturing Removal of asbestos products ``` Asbestosis is a promoter of carcinogenesis

What is the pathophys of asbestosis?

1. Asbestos fibers are inhaled deep into lung parenchyma 2. Deposits retained in lung tissue → alveolar and interstitial fibrosis Slowly progressive, diffuse pulmonary fibrosis

What are the effects of asbestosis?

Asbestosis causes reduction in Lung volume Compliance Gas transfer

Differentiate between the early and late presentation of asbestosis

``` 1. Insidious onset A. Early: DOE ↓ exercise tolerance Cough ``` B. Late: CP Clubbing

When do most ppl seek treatment for asbestosis?

Most seek medical attention 15 or > years after exposure

What are the CXR results for asbestosis?

Diffusely distributed linear opacities/streaking at lung bases Calcified plaques on diaphragm or lateral chest wall (pleural plaques) Thickened pleura Pleural involvement is Hallmark

What is the prophylaxis for asbestosis?

Dust suppression in work environment / Abatement teams | Smoking cessation

What is the supportive treatment for asbestosis?

Oxygen Immunizations Prompt tx of resp infections

What increases the risk of complications from asbestosis?

Smoking/tobacco use

What are the complications of asbestosis?

1. Latency period may be 15-40 yrs 2. Lung cancer 3. Mesothelioma A. Cancer within lining of chest B. Pleural effusion-thoracentesis, PET scan