Diffuse Interstitial Disease Flashcards
1
Q
A group of chronic disorders characterized by inflammation and fibrosis of the pulmonary interstitial tissue
A
diffuse interstitial disease
2
Q
Known collectively as restrictive lung disease
A
diffuse interstitial disease
3
Q
What are diffuse interstitial diseases caused by?
A
Many causes, poorly understood
4
Q
Diffuse interstitial diseases are responsible for about __% of __________ pulmonary diseases.
A
Diffuse interstitial diseases are responsible for about 15% of noninfectious pulmonary diseases.
5
Q
What are some fibrosing diseases? (7)
A
- Idiopathic pulmonary fibrosis
- Nonspecific interstitial pneumonia
- Cryptogenic organizing pneumonia
- Pulmonary fibrosis associated with connective tissue diseases
- Pneumoconiosis
- Drug reactions
- Radiation pneumonitis
6
Q
What is a granumlomatous disease?
A
tuberculosis
7
Q
What are types of diffuse interstitial disease?
A
- fibrosing dz
- granulomatous dz
- pulmonary eosinophilia
8
Q
What is the pathogenesis of interstitial fibrosis?
A
- endothelial damage and activation
- fibroblast migration and proliferation, basement membrane disruption
- angiogenesis and myofibroblast foci formulation
- pulmonary fibrosis
9
Q
chronic process of pulmonary fibrosis or scarring due to unknown causes
A
idiopathic pulmonary fibrosis
10
Q
What is idiopathic pulmonary fibrosis cause by?
A
Caused by recurrent inflammation and repair in susceptible persons
11
Q
With IPF, what is the cause the susceptible persons?
A
hereditary
12
Q
What does IPF result in?
A
decreased lung compliance
13
Q
What will IPF show with spirometry
A
decreased lung volume
14
Q
What is IPF associated with?
A
- pulmonary HTN
- right HF
15
Q
Does IPF occur abruptly or insidiously?
A
insidiously
16
Q
What are the initial symptoms of IPF?
A
gradually increasing dyspnea on exertion and dry cough
17
Q
What are the later symptoms of IPF?
A
- hypoxemia
- cyanosis
- clubbing
18
Q
What is the typical age range for presentation with IPF?
A
40-70 yo
19
Q
What are some general symptoms of IPF? (5)
A
- Shortness of breath
- Chronic dry, hacking coughing
- Fatigue and weakness
- Chest discomfort
- Loss of appetite and rapid weight loss
20
Q
What are some CXR features of IPF?
A
- Reduced lung volume
- Reticular opacities
- Lower lobe and peripheral field predominance
- Honeycombing may be visible (fibrocystic appearance)
21
Q
What imaging technique can you best se honeycombing?
A
better on CT versus xray
22
Q
diseases that can involve the lung, resulting in different presentations including pulmonary interstitial fibrosis (other manifestations include vascular sclerosis, pneumonia, and bronchiolitis)
A
autoimmune connective tissue diseases
23
Q
What are autoimmune connective tissue diseases associated with?
A
- pulmonary hypertension
- right HF
24
Q
What are the causes of autoimmune connective tissues diseases? (3)
A
- Rheumatoid arthritis
- Systemic sclerosis (scleroderma)
- Lupus erythematosus
25
What percent of patients is rheumatoid arthritis the cause of autoimmune connective tissue diseases?
30-40%
26
Lung fibrosis in response to inhaled particles and aerosols
pneumoconiosis
27
With pneumoconiosis, ____________ ingest particles, die and release ________ contents and cytokines causing _______ without clearing ________ stimulus.
With pneumoconiosis, macrophages ingest particles, die and release lysosomal contents and cytokines causing fibrosis without clearing inflammatory stimulus.
28
What size are particles that are most likely to reach terminal alveoli?
1-5 microns
29
Pneumoconiosis has a similar pathology as which condition?
IPF
30
What are the causes of pneumoconiosis?
- mineral dust
- organic dust
- chemical fumes
31
What are some examples of mineral dust?
- coal
- silica
- asbestos
- iron oxide
32
What are organic dusts also associated with?
- hypersensitivity pneumonitis
| - asthma
33
What are some examples of hypersensitivity pneumonitis causes?
- moldy hay
| - bird droppings
34
What are examples of asthma causes?
- cotton
- flax
- hemp
- red cedar dust
35
What are some examples of chemical fumes?
- nitrous oxide
- sulfur dioxide
- ammonia
- benzene
- insecticides
36
What describes when certain drugs may be associated with acute and chronic alterations in respiratory structure and function?
drug-induced pulmonary disease
37
Drug-induced pulmonary disease has similar process as what condition?
IPF
38
What are some drugs that can cause drug-induced pulmonary disease? (6)
- bleomycin
- methotrexate
- amiodarone
- nitrofurantoin
- aspirin
- beta antagonists
39
What are 2 examples of granulomatous diseases?
- sarcoidosis
| - hypersensitivity pneumonitis
40
a firm nodule resulting from chronic inflammation, often with a fibrous capsule
granuloma
41
What does a granuloma contain?
-macrophages
-lymphocytes
-plasma cells
-multinucleated giant cells
(all of these are associated with chronic inflammation)
42
Granulomas may contain ______ organisms and may have central ______.
Granulomas may contain infectious organisms and may have central necrosis.
43
What organism is caseating associated with specifically?
mycobacterium
44
What are examples of infectious causes of granulomatous diseases?
- bacteria
- fungi
- parasites
45
What are examples of noninfectious causes of granulomatous diseases?
- sarcoidosis
| - hypersensitivity pneumonitis
46
An immunologically-mediated systemic disease of unknown cause characterized by non-caseating granulomas in many tissues and organs
sarcoidosis
47
What organs and tissues are most commonly affected by sarcoidosis?
- lungs
- LN/spleen
- eyes
- skin
48
How do you differentiate sarcoidosis from infectious causes?
biopsy will show an absence of microbes
49
What are CXR findings of sarcoidosis?
- bilateral hilar lymph node enlargement
| - milia-size and larger granulomas that may cavitate
50
What percentage of sarcoidosis cases will you see bilateral hilar lymph node enlargement?
90% of cases
51
What may the granulomas in the lung initiate?
initiate interstitial fibrosis and pulmonary edema
52
What are some other signs of sarcoidosis?
- skin granulomas
| - eye granulomas
53
immunologically-mediated inflammatory lung disorders caused by intense, usually prolonged exposure to inhaled animal, microbial, and some chemical antigens
hypersensitivity pneumonitis
54
What does hypersensitivity pneumonitis causes diffuse inflammation of and what can that result in?
Causes diffuse inflammation of interstitial lung, terminal bronchioli, and alveoli, can result in fibrosis
55
What does hypersensitivity pneumonitis require?
Requires abnormal sensitivity or heightened reactivity to the antigen = susceptible individuals
56
What are the 2 primary types of hypersensitivity reactions that cause hypersensitivity pneumonitis?
- type 3 (immune complex)
| - type 4 (delayed hypersensitivity)
57
Acute attacks, which follow inhalation of antigenic dust in sensitized patients
hypersensitivity pneumonitis
58
With hypersensitivity pneumonitis, what are there recurring episodes of?
- fever
- dyspnea
- cough
- leukocytosis
59
with hypersensitivity pneumonitis, when do symptoms usually appear?
usually appear 4-6 hours after exposure
60
What may hypersensitivity pneumonitis progress to?
may progress to:
- respiratory failure
- dyspnea
- cyanosis
- decrease in total lung capacity and compliance
61
How can hypersensitivity pneumonitis be prevented?
Can be prevented by removal of the stimulus, if recognized early
62
What are the common sensitizing agents for hypersensitivity pneumonitis?
Common sensitizing agents include:
- organic dust with bacterial spores
- fungi
- animal proteins
- bacterial products
63
results from exposure to dusts generated from harvested humid, warm hay that permits the rapid proliferation of the spores of thermophilic actinomycetes
Farmer's lung
64
What are the spores associated with Farmer's lung?
thermophilic actinomycetes
65
provoked by proteins from serum, excreta, or feathers of birds
bird breeder's lung/bird fancier's disease
66
caused by thermophilic bacteria in heated water reservoirs
Humidifier or air-conditioner lung
67
What are CXR findings for hypersensitivity pneumonitis?
diffuse and nodular infiltrates
68
Several pulmonary disorders characterized by an infiltration of eosinophils into alveoli, resulting in intraseptal inflammation, fibrosis
pulmonary eosinophilia
69
What does pulmonary eosinophilia result in?
- intraseptal inflammation
| - fibrosis
70
What is the cause of pulmonary eosinophilia?
frequently idiopathic
71
Assessment of diffuse interstitial disease
- History and physical examination
- Spirometry
- CXR or CT
- Oximetry, arterial blood gas, acid-base balance
- Endoscopic bronchial biopsy
- Laboratory assessment for autoimmune diseases (rheumatoid arthritis, lupus erythematosus, scleroderma, sarcoidosis)
72
What are symptoms of diffuse interstitial disease?
- dyspnea
| - tachypnea
73
What are signs of diffuse interstitial disease?
- End-inspiratory crackles (without wheezing or other evidence of airway obstruction)
- Cyanosis
- Increased blood CO2 (hypercapnia) and reduced O2 (hypoxia)
- Reduced lung volume (FVC, TLC) and compliance (FEV1) by spirometry
74
What are CXR findings for diffuse interstitial disease?
CXR shows bilateral small nodules, irregular lines, ground-glass shadows
75
What does spirometry show with diffuse interstitial disease?
- Reduced lung volume (FVC, TLC)
| - Reduced compliance (FEV1)
76
What does diffuse interstitial disease frequently result in?
Frequently results in:
- secondary pulmonary hypertension
- cor pulmonale
77
Why are end-stage diffuse interstitial diseases hard to differentiate?
hard to differentiate because they result in similar scarring and destruction of the lung (called end-stage lung or honeycomb lung)
