Restrictive Lung dz packet - Ms. Anna Flashcards
(62 cards)
What is restrictive lung dz characterized by?
Decreased lung volume and decreased lung capacity due to either alteration in the lung parenchyma or disease of the pleura, chest wall, or neuromuscular apparatus
Describe how alteration in lung parenchyma relates to restrictive lung dz?
“intrinsic” or “interstitial” dz
Disease causes inflammation, scarring, or results in filling of air spaces with exudate or debris
Describe how diseases of pleura, chest wall, or neuromuscular apparatus relate to restrictive lung disease?
“extrinsic”
Chest wall, pleura, or respiratory muscles are not functioning normally
What are some examples of extrinsic disease that involve the chest wall?
Trauma
Kyphoscoliosis
Ankylosing spondylitis
Neuromuscular disease like myasthenia gravis/guillain barre
morbid obesity
scleroderma
What are some drug induced causes of interstitial lung disease?
Chemotherapy
Methotrexate
Amiodarone
Macrobid
What are some autoimmune related causes of interstitial lung disease?
RA
SLE
Scleroderma
Polymyositis
Dermatomyositis
Sjogrens syndrome
What is the most common hypothesized etiology of interstitial lung disease?
Both environmental and genetic factors contribute
Usually idiopathic
Most identifiable causes include infectious, drug-related, or environmental/occupational
What does interstitial lung disease cause?
thickening of the interstitium, a part of the lung’s structure
Inflammation, scarring, or extra fluid can result in the thickening
Can be acute or chronic
What are the pathophysiological changes in ILD?
After injury/exposure:
-A repair process is initiated resulting in progressive, patchy, and diffuse thickening of the alveolar walls with connective tissue and inflammatory cells
What is the functional abnormality of ILD?
Reduced lung volume/capacity
What are the symptoms of ILD?
Dyspnea**
-usually insidious and progressive
Dry chronic cough
Extrapulmonary symptoms may be noted depending on dz
What are the signs of ILD?
Early - normal
Advanced:
-tachypnea
-bilateral crackles
-clubbing
-right sided heart failure
What all should be in the workup for ILD?
CXR
PFTs
CT chest
Blood tests
Bronchoscopy with biopsy
Surgical biopsy
What are the common CXR findings in ILD?
Low lung volumes
Patchy “ground glass” appearance
Hallmark: reticular or nodular opacities
What are the common Chest CT findings in ILD?
HRCT:
Reticular opacities
interlobular septal thickening
Honeycombing (late)
Nodules
Traction bronchiectasis
What will be seen on spirometry in ILD?
Decrease lung volumes
Decrease vital capacity
What is the use of Bronchoscopy with biopsy and surgical biopsy for ILD?
Bronchoscopy with biopsy is diagnostic for sarcoidosis
Surgical biopsy is the definitive diagnostic study
What are the pharmacological treatment options for ILD?
Corticosteroids
Cytotoxic agents
Immunosuppressive agents
What are the non-pharmacological treatment options for ILD?
Smoking cessation
Supplemental O2 therapy
Avoid exposure/DC meds
Tx pulmonary infections
Pulmonary rehabilitation
Lung transplant (definitive)
Define idiopathic pulmonary fibrosis?
A subtype of interstitial lung disease that results in fibrosis of the lungs without a known cause
What is the etiology of idiopathic pulmonary fibrosis?
Cause is unknown
More commonly effects older adults (65+)
Hypothesized genetic link
What is the pathogenesis of idiopathic pulmonary fibrosis?
An inciting incident disrupts homeostasis of alveolar epithelial cells causing diffuse epithelial cell activation and abnormal cell repair
Exaggerated accumulation of extracellular matrix - destruction of the lung parenchyma
Idiopathic pulmonary fibrosis clinical manifestations?
limited to lungs - systemic symptoms rare
Onset is gradual and not often diagnosed for 1-2 years
Most common complaint is dyspnea, 2nd is non-productive cough
What is found on PE for idiopathic pulmonary fibrosis?
End-respiratory crackles