interstitial lung dz Flashcards
(49 cards)
1
Q
interstitial lung disease is also known as
A
pulmonary fibrosis
2
Q
in interstitial lung disease, the lung is affected in what 3 ways
A
- lung parenchyma is damaged
- walls of alveoli become inflamed
- scarring (fibrosis) begins and lungs become stiff
3
Q
interstitial lung disease has what primary clinical presentation
A
- progressive dyspnea on exertion and nonproductive cough
- wheezing and CP are uncommon
4
Q
PE is consistent with
- crackles at lung bases
- inspriatory squeaks
- cor pulmonale
- cyanosis and digital clubbing (advanced dz)
A
interstitial lung disease
5
Q
what abnormal CXR findings are consistent with interstitial lung disease
A
- ground-glass appearance: often early finding
- bilateral opacities, reticular “net-like” (most common)
- honeycombing indicated poor prognosis
6
Q
pulmonary function tests reveal that most interstitial lung disease are . TLC will be? FEV1/FVC ratio?
A
- restrictive
- TLC decreased
- FEV1 and FVC decreased but ratio is normal
7
Q
contrary to other interstitial lung diseases, sarcoidosis has what pattern on PFT
A
obstructive
8
Q
expected diffusing capacity of lung for CO (DLCO) in interstitial lung disease
A
- reduced
- ability of gas to cross from air -> interstitium -> blood is diminished due to inflammation of alveolar wall
9
Q
expected ABG in interstitial lung disease
A
- may be normal
- may show hypoxemia or respiratory alkalosis
- increased RR -> decreased PaCO2
10
Q
what is bronchoalveolar lavage
A
- extension of bronchoscopy
- allows for cellular analysis
- may help narrow DDx, define stage of disease, assess progress or response to therapy
11
Q
what is the gold standard to diagnose interstitial lung disease
A
lung biopsy
12
Q
provide indications when lung biopsy is indicated
A
- atypical or progressive symptoms
- age >50 yo
- fever, weight loss, hemoptysis
- ILD symptoms with normal or atypical CXR
13
Q
list complications of interstitial lung disease
A
- pulmonary HTN -> Cor pulmonale (rt ventricular hypertrophy) -> Rt heart failure
- pneumothorax (alveoli become so stiff, they rupture)
- elevated CA risk
- progressive respiratory insufficiency
14
Q
what is often the initial procedule of choice for lung biopsy
A
transbronchial lung biopsy
- less invasive, less tissue for analysis
15
Q
known occupation and environmental causes of interstitial lung disease
A
- inorganic dust (asbestos, silica, hard metals)
- organic dust (bacteria, animal proteins)
- gases, fumes
16
Q
known drug cause of interstitial lung disease
A
- chemo
- abx: macrobid
- radiation treatment
17
Q
interstitial lung disease is associated with what diseases
A
- sarcoidosis
- connective tissue or autoimmune
- scleroderma
- SLE
- RA
- polymyositis
- systemic vasculitis (granulomatosis with polyangitis)
18
Q
what is Pneumoconiosis
A
- any disease of the respiratory tract due to inhalation of dust particles
- asbestosis, silicosis
19
Q
when does asbestosis present
A
- due to chronic inhalation of asbestos fibers
- usually presents 10-15 yrs of exposure
- age 40-75 yo
- m>f
20
Q
asbestos is linked to causing what conditions
A
- bronchogenic (lung) CA
- smoking increases risk
- malignant mesothelioma
21
Q
What is mesothelioma
A
- form of CA almost always associated with asebestos exposure
- CA in mesothelium (protective lining that covers most organs)
- most common in pleura
22
Q
What CXR findings are consistent with asbestosis
A
- opacities in lower lungs, thickened pleura, pleural plaques
23
Q
how is asbestosis diagnosed?
A
- consistent hx, PE, symptoms and CXR
- open lung bx will provide definitive diagnosis but usually not indicated
24
Q
asbestosis will have what findings on PFT
A
- restrictive
- decreased TLC
- FEV1/FVC normal
25
asbestosis management
* consult pulmonology
* smoking cessation
* no effective therapy
* vaccinate: influenza, pneumovax
26
* male
* 50ish
* pipefitter
* smoker
* CXR: pleural plaques
asbestosis
27
prognosis of asbestosis
* lung damage is irreversible
* smoking + asbestosis = 59 x more likely to get lung CA
28
which type of pneumoconiosis is characterized by fibronodular lung disease
silicosis
29
what causes silicosis
inhalation of silica dust
* occupations: mining, construction, granite cutting, pottery making
30
presentation
* 10-12 years exposure
* may be asymptomatic
* non-progressive once exposure elimiated
* **hilar node calcification (eggshell pattern**)
* small round opacities on CXR
chronic **simple** silicosis
31
presentation
* \>20 years exposure
* progressive even after exposure eliminated
* tachypnea, prolonged expiration, rhonchi, wheezing
* digital clubbing (uncommon)
* cyanosis (advanced)
* cor pulmonale (advanced)
* CXR: enlarging opacities that can cavitate
* PFT: restrictive
chronic complicated silicosis
32
management of chronic complicated silicosis
* consult pulmonologist
* smoking cessation
* no specific therapy
* vaccinate: influenza, pneumovax
33
which condition is associated with **noncaseating granulomas**, predominantly in the **lungs**, but can affect heart, liver, spleen, joints, bone
sarcoidosis
34
sarcoidosis normally affects what patient population
* african american
* 20-40 yo
* slight female predominance
35
clinical presentation
* fever, anorexia, arthralgias (45% of people)
* dyspnea on exertion, cough, CP
* arthritis, cranial nerve palsies, visual disturbances, **erythema nodusum**
* PFT: restrictive
sarcoidosis
36
what is the most common pattern of lymphadenopathy in sarcoidosis
* bilateral symmetric hilar and right paratracheal mediastinal adenopathy
37
increased serum ACE is associated with what ILD condition
sarcoidosis
38
managment of sarcoidosis
* consult pulmonologist
* 75% require only symptomatic -\> NSAIDs
* corticosteroids: mainstay of therapy
39
what is granulomatosis with polyangitis
* immune-mediated systemic vasculitis
40
which ILD condition is associated with **necrotizing granulomas** of the upper and lower respiratory tract and **glomerulonephritis**
granulomatosis with polyangitis
41
granulomatosis with polyangitis usually affects what patient population
* white
* male
* presents 40-50s
42
clinical presentation
* upper airway
* rhinorrhea, purulent/bloody nasal dx
* oral/nasal ulcers
* lower airway
* dyspnea, cough, pleuritic pain, hemoptysis
* PFT: restrictive or obstructive; decreased DLCO
* CT: blood vessels leading to nodules and cavities
granulomatosis with polyangitis
43
which condition is associated with a + ANCA: antineutophil cytoplasmic antibody, usually C-ANCA
granulomatosis with polyangitis
44
managment of granulomatosis with polyangitis
* consult rheumatologist
* tx: cyclophosphamide (immunosuppressant) and glucocorticoid
45
what is the most common idiopathic interstitial pneumonias
idiopathic pulmonary fibrosis
46
how is idiopathic pulmonary fibrosis diagnosed
* diagnosis made after excluding other causes of ILD
* lung biopsy
47
what is interstitial pneumonitis
another name for idiopathic pulmonary fibrosis
48
what patient population is associated with idiopathic pulmonary fibrosis
* male
* 60s
* a common man
* smoker
49
managment of idiopathic pulmonary fibrosis
* consult pulmonologist
* eval for lung transplant
