Interstitial Lung Diseases Flashcards
1
Q
What are interstitial lung diseases (ILDs)?
A
A group of disorders characterized by cellular infiltration, scarring and/or architectural disruption of the pulmonary parenchyma
2
Q
What are the ssx of ILD?
A
Dry cough with progressive DOE
3
Q
What are PFTs like with ILDs?
A
Restrictive pattern (decreased FEV, and normal FEV1/FVC)
4
Q
What are the CT/ CXR findings with ILDs?
A
Interstitial infiltrates and Ground glass appearance
5
Q
What are the major occupational exposures that lead to ILD? (4)
A
Silica
Asbestos
Farms
Fumes
6
Q
What are the two classic drugs that lead to ILD?
A
Amiodarone
Nitrofurantoin (macrobid)
7
Q
What are the connective tissue disorders that can lead to ILD?
A
SLE
RA
Scleroderma
8
Q
What are the signs of ILD?
A
Clubbing
Erythema/rash
Arthritis
9
Q
What are the lungs sounds with ILD?
A
Dry crackles
10
Q
What heart findings are found with ILD? (4)
A
Increased right heart pressure:
- increased P2 intensity
- TR murmur
- edema
- JVD
11
Q
What is usually the first change on a PFT with ILD? Why?
A
Decreased DLCO due to non homogeneous thickening of the alveoli
12
Q
What is usually the first change on a PFT with COPD? Why?
A
FEV/FVC ratio
13
Q
What are the two ILDs that have increased lung volumes?
A
LAM
Langerhans histiocytosis
14
Q
What is the granulomatous ILD?
A
Sarcoidosis
15
Q
What are the four known association ILDs?
A
CT diseases
Drugs
Occupational exposure
Hypersensitivity
16
Q
What are the idiopathic Interstitial pnuemonias?
A
- Idiopathic pulmonary fibrosis
- non-specific interstitial pneumonia
- Cryptogenic organizing pneumonia
- respiratory bronchiolitis
- desquamative interstitial pneumonia
- acute interstitial pneumonia
17
Q
What patients with idiopathic interstitial pneumonias respond to therapy?
A
No
18
Q
What is the prognosis (generally) for idiopathic pulmonary fibrosis?
A
Bad-3-5 year survival rate
19
Q
What is the history commonality for idiopathic pulmonary fibrosis?
A
Smoking
20
Q
Who usually gets idiopathic pulmonary fibrosis?
A
50-70 year old smokers
21
Q
What are the ssx of idiopathic pulmonary fibrosis?
A
Chronic and progressive DOE, and non-productive cough
22
Q
What are the lung sounds with idiopathic pulmonary fibrosis?
A
Bibasilar inspiratory “velcro-like” crackles
23
Q
What percent of patients with idiopathic pulmonary fibrosis have clubbing?
A
25%
24
Q
What causes the crackles with idiopathic pulmonary fibrosis?
A
Expanding alveoli
25
What are the CXR findings with idiopathic pulmonary fibrosis? (which lobe? central or peripheral distribution? What type of CT changes)?
Lower lobe and peripheral distribution of reticular infiltrates and fibrotic changes
26
What are the CT changes with idiopathic pulmonary fibrosis?
Patchy honeycombing and traction bronchiectasis
27
What are the tissue changes that can be seen in with idiopathic pulmonary fibrosis?
Usual interstitial pneumonitis
28
What are the drugs used to treat idiopathic pulmonary fibrosis? MOA?
Pirfenidone
Nintedanib
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
29
What is the MOA of etanercept? Is this effective in idiopathic pulmonary fibrosis?
Anti-TNF antibody
Not effective
30
What is N-acetylcysteine used for usually? Is it effective for idiopathic pulmonary fibrosis?
Acetaminophen overdose
Not effective
31
What is the MOA of Pirfenidone? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
32
What is the MOA of nintedanib? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
33
What are nonspecific interstitial pneumonitis (NSIP)?
Homogenic CT disease of the lungs
34
What is the prognosis (generally) with interstitial pneumonitis?
better than IPF
35
What are the imaging findings with nonspecific interstitial pneumonitis (NSIP)?
Ground glass appearance symmetrically in the lower lung fields
36
Which disease is honeycombing common in: idiopathic pulmonary fibrosis or nonspecific interstitial pneumonitis (NSIP)?
idiopathic pulmonary fibrosis
37
What is the therapy for nonspecific interstitial pneumonitis (NSIP)?
Steroids
38
What are the two ILDs that are definitively linked to smoking?
Desquamative interstitial pneumonitis (DIP)
Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)
39
What are the histological characteristics of Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?
Peribronchiolar accumulation of pigmented macrophages
40
What is the treatment for Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?
Stop smoking
| Steroids
41
What are the imaging findings with DIP and RB-ILD?
Diffuse, patchy areas of ground glass appearance
42
What is acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)
Sudden and fulminant form of diffuse lung injury that generally occurs in previously healthy individuals
43
What are the ssx ofacute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)
Fever
Cough
SOB
44
What is the prognosis for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
50% mortality, and those that survive develop fibrosis
45
What are the imaging findings with acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
Diffuse, bilateral air space and reticular infiltrates
46
What is the treatment for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
Supportive
47
What is cryptogenic organizing pneumonia (COP)?
a non-infectious pneumonia; specifically, an inflammation of the bronchioles (bronchiolitis[1]) and surrounding tissue in the lungs.
48
Who usually gets cryptogenic organizing pneumonia (COP)?s
Smokers
49
What is the clinical presentation of cryptogenic organizing pneumonia (COP)?
LRTI with lack of response to abx
50
LRTI with lack of response to abx = ?
cryptogenic organizing pneumonia (COP)
51
What is the treatment and prognosis for cryptogenic organizing pneumonia (COP)?
Corticosteroids
| Good prognosis
52
What is the best way to diagnose cryptogenic organizing pneumonia (COP)? (2)
Bronchoscopy
| Alveolar lavage
53
What are the imaging findings of cryptogenic organizing pneumonia (COP)? Where are they in the lungs?
Patchy infiltrates (unilaterally or bilaterally), with small nodular opacities
Usually subpleural
54
What is hypersensitivity pneumonitis?
Repeated exposure and immunologic sensitization to organic antigens
55
What are the risk factors for developing hypersensitivity pneumonitis? (3)
Bird feathers
Thermophilic bacteria
Farmers
56
What are the cells that mediate hypersensitivity pneumonitis? What do these do?
CD8 +
Macrophages
Granuloma formation
57
What is the usual acute presentation of hypersensitivity pneumonitis?
Abrupt onset of fever, chills, nausea, cough, chest tightness and dysponea
58
What are the PE findings with hypersensitivity pneumonitis?
Tachypnea
| Diffuse, fine crackles
59
What are the imaging findings of hypersensitivity pneumonitis?
Normal to patch, micronodular infiltrates
60
What is the treatment for hypersensitivity pneumonitis?
Remove antigen exposure
61
What is the usual SUBacute presentation of hypersensitivity pneumonitis? Treatment?
Gradual development of cough, SOB, weight loss
Corticosteroids
62
What is the usual presentation of CHRONIC hypersensitivity pneumonitis?
Insidious onset of cough, fatigue, SOB
Clubbing
63
What is the major complication of chronic hypersensitivity pneumonitis?
Irreversible Pulmonary fibrosis
64
What are the CT findings with hypersensitivity pneumonitis? (2)
Ground glass and centrilobular nodules
65
Why should you r/o other causes of lung disease prior to treating any of the ILDs?
Steroids will weaken the immune response, which will be bad with infectious causes
66
What is the lung disease that amiodarone causes? Who usually gets this?
Interstitial pneumonitis
Patients who are on the drug long term
67
What are the imaging findings with amiodarone toxicity? (2)
Ground glass appearance, with increased attenuation of the liver/spleen
68
What are the two presentations of lung toxicity induced by nitrofurantoin?
Acute after course of abx
Chronic after several months of abx
69
What are the peripheral blood findings with lung toxicity from nitrofurantoin?
Eosinophilia
70
What are the cytokines that are elevated with nitrofurantoin lung toxicity?
Serum gamma globulin
Serum transaminases
ANA
71
What is acute eosinophilic pneumonia? What causes it?
Acute febrile illness of 1-4 weeks duration with usual symptoms.
Associated with resumption of smoking, or inhalation exposure
72
What are the peripheral blood smear findings with acute eosinophilic pneumonia?
None
73
What are the histological findings with acute eosinophilic pneumonia?
Extensive eosinophilic infiltration in the interstitium
74
What are the CT findings of acute eosinophilic pneumonia?
Bilateral ground glass
75
Which is more likely to have eosinophilia: chronic or acute eosinophilic pneumonia?
Chronic
76
What is chronic eosinophilic pneumonia?
Idiopathic infiltration of eosinophils in the interstitial or alveolar spaces
77
What are the imaging findings of chronic eosinophilic pneumonia
bilateral *peripheral* pulmonary edema
| "negative of pulmonary edema"
78
What are the peripheral blood smear findings with chronic eosinophilic pneumonia?
High levels of eosinophils
79
What is lymphangioleiomyomatosis? Who is affected?
Progressive cystic lung disease that occurs in women of childbearing years
80
What are the histological findings of lymphangioleiomyomatosis?
Proliferation of atypical smooth muscle cells
81
What are the ssx of lymphangioleiomyomatosis?
DOE
Hemoptysis
Pneumothorax
82
What is the usual presenting sign of lymphangioleiomyomatosis?
Pneumothorax
83
What is the treatment for lymphangioleiomyomatosis?
Lung transplant
84
What is the best test to get to differentiate ILDs?
High res CT (HRCT)
85
Idiopathic pulmonary fibrosis and non-specific interstitial pneumonitis are very similar. What are the major differences between the two? (hint: age, CT findings, prognosis, pathological pattern)?
IPF = older smokers, honeycombing, bad prognosis, UIP
NSIP: Younger pts, non-honeycombing, better prognosis, temporal heterogeneity pattern,
