Interstitial Lung Disease Flashcards Preview

Gen Med Unit 6 > Interstitial Lung Disease > Flashcards

Flashcards in Interstitial Lung Disease Deck (42)
Loading flashcards...
1
Q

What are idiopathic interstitial lung diseases?

A

Sarcoidosis

Idiopathic pulmonary fibrosis

2
Q

What are drug induced interstitial lung diseases?

A

Methotrexate
Amiodarone
Bleomycin

3
Q

What are pneumoconiosis interstitial lung diseases?

A

Asbestosis
Silicosis
Coal Worker’s Lung

4
Q

What are Connective tissue disease

interstitial lung diseases?

A

Polymyositis
Dermatomyositis
SLE
Rheumatoid arthritis

5
Q

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

A
  1. Chronic inflammation of alveolar walls with progressive fibrosis
  2. Most commonly occurs between 50-70 yr
  3. Survival is 3-5 years after diagnosis
6
Q

What are other names for idiopathic pulmonary fibrosis?

A

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

7
Q

What is the pathophys of idiopathic pulmonary fibrosis?

A
  1. Accumulation of excessive fibrous or connective tissue in lung parenchyma
  2. Result of inflammatory, immune and fibrotic processes
  3. Inflammation is responsible for injury and fibrosis, distorts & impairs the structure and function of alveolar-capillary gas exchange surface
  4. Lungs become stiff and difficult to ventilate
  5. Diffusion capacity of alveoli-capillary membrane decreases  hypoxemia
8
Q

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

A
  1. Environmental or occupational exposure
    A. Coal dust, asbestos, silica, iron
  2. Connective tissue diseases
    A. Rheumatoid arthritis, SLE
  3. Radiation-induced
  4. Drug-induced
    A. Amiodarone, methotrexate, cocaine, heroin, chemo drugs
9
Q

What are the sxs of interstitial lung disease?

A
  1. Dyspnea with rapid shallow breathing
  2. Dry hacking cough
  3. Fatigue
  4. Inspiratory rales on auscultation
  5. Clubbing
  6. Cyanosis
  7. Pulmonary HTN
10
Q

What are the PFT results for interstitial lung disease>

A

↓ TLC

Normal FEV1/FVC

11
Q

What are the CXR results for idiopathic pulmonary fibrosis?

A
  1. Diffuse interstitial lung disease with fibrosis and honeycombing (cystic opacities)
  2. Reticular/nodular opacities are hallmark
  3. usually bilateral
12
Q

What are the CT results for iinterstitial lung disease?

A

Patchy distribution of ground glass in lung parenchyma

13
Q

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

A

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

14
Q

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

A
  1. O2 to improve hypoxemia
  2. Corticosteroids to reduce inflammatory response
  3. Pulmonary rehab
  4. Lung transplantation is definitive treatment
    A. 5 yr survival rate estimated at 50%
15
Q

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

A

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

16
Q

What are the complications for idiopathic pulmonary fibrosis?

A
Respiratory failure
Heart failure
Pulmonary embolism
Pneumonia
Lung cancer
17
Q

Define Pneumoconiosis

A

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

18
Q

When does silicosis occur?

A
  1. Follows long term inhalation of small particles of silica
  2. Associated with:
    Metal mining (Pb, Cu, Ag, Au),
    Pottery making
    Sandstone & granite cutting
    Sandblasting
19
Q

What is the pathophys of silicosis?

A

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

20
Q

What is the clinical presntation of simple cases of silicosis?

A

Asymptomatic

20-30 years exposure necessary before disease becomes apparent

21
Q

What is the clinical presntation of complicated cases of silicosis?

A

Dyspnea, rales, clubbing, cyanosis

Related to amount of exposure

22
Q

What are the cxr results of silicosis?

A

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

23
Q

What is the treatment for silicosis?

A
No effective treatment 
Supportive therapy
Oxygen
Immunizations 
Pulmonary rehab
24
Q

What is the prophylaxis for silicosis?

A

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

25
Q

What are the complications of silicosis?

A

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

Progressive fibrosis

26
Q

Define coal workers lung

A

Diffuse nodular deposition of dust in lungs

Results from long term exposure to coal dust in coal mining

27
Q

What is the patho phys of coal miners disease?

A

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

28
Q

Differentiate between simple and complicated coal miners disease

A

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

29
Q

Differentiate between the sxs of simple and complicated coal miners disease

A

Simple Disease
Asymptomatic
Underground exposure of 20 years before sx’s start to develop

Complicated Disease
Dyspnea on exertion
“Black lung”

30
Q

What is the treatment for coal miners disease?

A
No effective treatment 
Supportive therapy
Oxygen
Immunizations
Pulmonary rehab
31
Q

What is the prophylaxis for coal miners disease?

A

Suppress coal dust for prevention

32
Q

What are the complications of coal miners disease?

A

Progressive massive fibrosis

33
Q

Define asbestosis

A
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

34
Q

What is the pathophys of asbestosis?

A
  1. Asbestos fibers are inhaled deep into lung parenchyma
  2. Deposits retained in lung tissue → alveolar and interstitial fibrosis
    Slowly progressive, diffuse pulmonary fibrosis
35
Q

What are the effects of asbestosis?

A

Asbestosis causes reduction in
Lung volume
Compliance
Gas transfer

36
Q

Differentiate between the early and late presentation of asbestosis

A
1. Insidious onset 
A. Early: 
DOE
↓ exercise tolerance
Cough

B. Late:
CP
Clubbing

37
Q

When do most ppl seek treatment for asbestosis?

A

Most seek medical attention 15 or > years after exposure

38
Q

What are the CXR results for asbestosis?

A

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

39
Q

What is the prophylaxis for asbestosis?

A

Dust suppression in work environment / Abatement teams

Smoking cessation

40
Q

What is the supportive treatment for asbestosis?

A

Oxygen
Immunizations
Prompt tx of resp infections

41
Q

What increases the risk of complications from asbestosis?

A

Smoking/tobacco use

42
Q

What are the complications of asbestosis?

A
  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