Restrictive Lung Diseases Flashcards
A synonym for intrinsic restrictive lung disease is?
interstitial
How is restrictive lung disease defined?
less than 80% (predicted) TLC (problems inflating the lungs) via a PFT
How is obstructive lung disease defined?
FEV1/FCV less than 70%
What are the most and least common cells in the lungs?
most-macrophages
least-neutrophils
middle- lymphocytes
What is a normal CD4/CD8 ratio in the lungs?
2:1
What is sarcoidosis?
multisystem disorder of unidentified etiology characterized by non-caveating granulomas
What patient population is common for sarcoidosis?
young AA female adults (20-40 yo)
What happens to the size of alveoli in obstructive disease?
increases. EXPANDED lung disease
What happens to the size of conducting airways in restrictive disease?
decreases (or may not change) BUT expiratory velocity is increased
T or F. Lung volumes DECREASE in restrictive lung disease
T. Elastic recoil is increased
What is Type I restrictive disease?
disease involves lung (intrinsic)- ILD and lung resection
What is Type II restrictive disease?
disease involves pleura chest wall, muscles, ribs, etc. (extrinsic) and neuromuscular disorders
Interstitial lung disease (ILD) predominantly involve what part of the lung?
connective tissue of the alveolar wall (in between the epithelium and endothelium)
How would a person with restrictive lung disease breath?
short, small breaths (panting) because its too hard to inflate the lungs
What cases the hypoxemia of ILD (interstitial lung disease)?
V/Q mismatch (NOT diffusion defect because there is plenty of reserve length available)
What are some granulomatous causes of ILD?
- Berylliosis
- Hypersensitivity pneumonitis
- Sarcoidosis
What are some Non-granulomatous causes of ILD?
- asbestos, coal, silica
- radiation
- CIGS
- Idiopathic Interstitial Pneumonia
- ILD with connective tissue disease
What are some drug causes of ILD (non-granulomatous)?
Bleomycin, amiodarone, nitrofurantoin
What are some types of ILD with connective tissue disease?
- Rheumatoid lung
- Lupus lung
- Mixed CT disease
Symptoms of ILD?
- DYSPNEA, progressive
- dry cough
- non-specific chest pain
- fatigue, weight loss
Signs of of ILD?
- Tachypnea, rapid-shallow
- VELCRO crackles
- digital clubbing
- Cor-pulmonale
- Pulmonary HTN
Why is digital clubbing seen in ILD?
fibrosis also occurs in the nail bed
T or F. Expiratory airflow is normal in ILD
T. TLC and VC are reduced though
Most of the diagnosis of ILD can be made by what?
high-resolution CT scan
What radiological feature is characteristic of early-stage ILD?
ground glass appearance (can be reversed)
What radiological feature is characteristic of mid-stage ILD?
reticulations and nodulations (most common)
What radiological feature is characteristic of late-stage ILD?
honeycombing
How is ILD diagnosed?
- Extensive history (genetics, occupation, meds, etc.)
- H&P (velcro crackles)
- imaging
- PFTS
- BAL or lung biopsy
What is a bronchoalveolar lavage (BAL)?
Using a scope to sample alveolar cell populations
Normal distribution of cells in a BAL?
Macro-85%
Lymph-20%
PMNS-2%
What is a common pleural diseases?
Mesothelioma (tumor compresses the lung- will present will pleural effusion)
What is a new treatment for IPF?
Pirfenidone
What is a key to early diagnosis of IPF?
bilateral velcro (aka fine-high pitched) crackles prominent at the lung BASE upon auscultation
T or F. In asbestosis, crackles are present before abnormalities are detected by chest radiograph, and are thus useful for screening populations exposed to asbestos
T.
Are crackles ever a normal physiologic phenomenon?
Yes, in elderly patients BUT they typically disappear after couple breaths
What is unique about the crackles heard in IPF?
they are pan-inspiratory (heard throughout the entire duration of inspiration)
T or F. In the early stages of restrictive lung disease, there is no hypoxia at rest but desaturation occurs during exercise
T.
PFTs of RLD?
- decreased FVC, DLCO (in intrinsic)
- normal FEV1:FVC
How can you differentiate between intrinsic and extrinsic RLD?
DLCO low in intrinsic (interstitial lung disease and lung resection) and normal in extrinsic (disease of the pleura, chest wall, neuromuscular disorders)
What will the DLCO/Va (correction for the amount of lung) show in lung resection?
100% (assuming no other disease)- keep in mind that DLCO will be 50% and other stats may look restrictive