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28 Cards in this Set

  • Front
  • Back
What is the most common interstitial lung dz of the young? old?
sarcoidosis, IPF
Pts with obstructive lung dz, can't inhale/exhale? Restrictive?

What 3 general problems can cause restrictive dz?
exhale; inhale

Problems w/ inspiratory muscles, problems w/ lung compliance, and problems with thorax compliance.
Most common cause of decrease in thorax compliance --> restrictive lung dz?
Morbid obesity.
What are the characteristics of spirometry for someone with restrictive lung disease?

Can you differentiate between the three different causes of restrictive dz based on spirometry alone?
Reduced FVC
Reduced FEV1
**High** FEV1/FVC
- the airways are wide open
- the increased elasticity of the lung pushes all the air out more quickly.

No.
When looking at lung volumes, give the "patters" of TLC, FRC, and RV associated with:
ILD, Muscle weakness, and obseity
ILD: All 3 decreased

Muscle weakness: TLD down, FRC normal, and RV raised.

Obesity: isolated decrease in FRC, possible small decrease in TLC. (small tho)
What happens in pts with ILD during exercise?
marked exercise-induced O2 desaturation = related to the increased distance of gas exchange.
What is cor pulmonale?

In lung dz that increases the width of the interstitum, the same amount of blood still has to flow... this results in what?

What does hypoxia do to pulmonary artery vaso-tone?

What does this contriction do to Rt Ventricle?

What would you expect to see in in the lung of a pt with Cor Pulmonale on CXR?
Rt. ht failure

Pulmonary HTN

causes constriction
^^AL --> RVH concentric --> RVH eccentric --> Fail --> Tricuspid regurge, hepatomegaly, JVD

Problem is with Rt Ventricle --> parenchyma is clear, no issues.
What are the three categories of ILD etiologies?
Mimicks

DPLD known etiology

DLPD unknown etiology
What are the things that can mimick DPLD?
CHF, Some Pulmonary infection (PCP, CMV), Lymphangitic carcinomatosis,
Can CXR help diff b/t DPLD and CHF?
Yes, there will be *bilateral* congestion w/CHF, sometimes cardiomegaly too.
Asbestosis
Silicosis
HyperS Pneumonitis
Xrt
Drugs

... all fall under which of the 3 diff dx categories for DLPD?
Known etiology
Sarcoidosis
Eosinophilic granuloma
Wegener's Granulomatosis
IPF
Eosinophilic pneumonia
aveolar proteinosis
BOOP


... all fall under which of the 3 diff dx categories for DLPD?
unknown etiology
What are the cell types involved in the development of sarcoidosis?

Do we see necrosis in sarcoidosis?
Macrophages, Dendritic cells -> present to lymphocytes

No, rarely.
IL-4, IL-5, IL-13 --> associated with the Th1 or Th2 pathway?

InF-g, IL-2, IL-12?

Which pathway takes care of Bacteria, fungus, and viri? Asthma/Parasites?

Which pathway predominates in sarcoidosis? As such, what are two potential causes being investigated?
Th2
Th1
Th1 (bact, etc.)
Th2 (asthma, etc.)

Th1
Mycobacteria, Propionibacteria.
What syndrome is the most common presentation of sarcoidosis?

Does it need to be treated with steroids? Why?
Lofgren's Syndrome:
Fever
symmetrical ankle arthritis
Hylar/mediastinal lympadenopathy
& erythema nodosum

No; chance of spontaneous resolution is high.
Is sarcoidosis-caused fibrosis typically an upper lobe or a lower lobe disease?
upper lobe.
What is lupus pernio? What is it associated with?
disfiguring skin lesions which develop primarily around the nose/face

chronic fibrotic lung disease caused by sarcoidosis.
How do you Dx sarcoid?
Have to find the non-caseating granulomas in tissue (and make sure there is not other cause of granuloma)
- skin lesions count
- probably going to have to do a bronchoscopy --> good idea because this is a peribronchial dz, and often the granulomas are not too hard to get at.
What is the mainstay of sarcoid txt in those pts that need it?
Corticosteroids (prednisone)
What is the prog for IPF?
not good; most will be dead w/i 5 years, and a good deal won't make it past 2.
DIP = ? RB-ILD?

What can make these dz go away?

Why is it important to differentiate these from UIP, BOOP, etc.?
disquamative interstitial pneumonia

respiratory bronchiolitis - interstitial lung dz

Stop smoking.

For the sake of prog: UIP is baaaAAd.
What is defined as:

A specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histological appearance of usual interstitial pneumonia (UIP)
IPF
Is IPF associated with cigarette smoking?
Yes. Not as profound as COPD tho'.
What are features of presentation for IPF?
Age > 50
Gradual onset of sx
Progressive Dypsnea on exertion
nonproductive paroxysmal cough
**classic 'velcro' crackles in bilateral lung bases
Digital Clubbing (25-50%)
PFT on IPF patients:
Spirometry
Lung volumes
Gas exchange
Blood gas
exercise oximetry
Low FVC, FEV1. High FEV1/FVC.

Reduced TLC, FRC, and RV.

Decreased diffusion capacity --> Decreased DLCO

Decreased PaO2, increased AA gradient

Desaturation on exercise oximetry.
What type of bronchiectasis can pts with fibrotic lung dz get?
Traction bronchiectasis - fibrotic tiss pulls the airways apart.
How do we test for IPF?
Can we use a transthroacic bronch?

Can it be dx'ed clinically?

Is IPF temporally homogenous?
Video-assisted thoracoscopic (VATS) Biopsy.

No, it doesn't give enough tissue.

No, heterogenous (insults occuring in different areas over time)

These days, yes, it can.
How do we Tx IPF?
Oxygen
- prevents/delays the development of RHF
Combined immunocuppressive therapy (Azathioprine/Predisone)
Antioxidants (N-acetylcysteine (NAC)
Anti-fibrotic agents
Lung Txplt