Restrictive (Interstitial) Lung Diseases Flashcards
What is interstitium?
connective tissue space around airways and vessels
Space between alveolar epithelium and capillary endothelium
space between basement membranes of the alveolar walls
Describe the relationship between the normal alveolar wall and most of the alveolar epithelial (pneumocyte) & interstitial capillary endothelial cell basement membranes
They are in direct contact
What is the material which impedes the elasticity of the alveoli (reducing compliance)?
Collagen
What are the characteristics of Interstitial Lung Disease?
•Reduced Lung Compliance
- Stiff Lungs
•Low FEV1 & Low FVC but FEV1/FVC normal ratio
•
•Reduced Gas Transfer (Tco or Kco)
-Diffusion abnormality
•Ventilation/Perfusion Imbalance
When small airways affected by pathology
Is there airflow limitation in restrictive lung disease?
NO
What are the clinical presentations for restrictive lung disease?
Which one is Normal? Emphysema? Interstitial Lung Disease?
What are the outcomes of Parenchymal (Interstitial) Lung Injury?
Acute inflammation (can develop into chronic response in rare circumstances) or Chronic inflammation
What are the three outcomes of Chronic response?
Which Chronic restrictive disease is most likely to develop end - stage honeycomb lung?
Usual Interstitial Pneumonitis
What does DAD stand for?
Difuse alveolar damage
What is DAD asociated with?
What are the exudative stages of DADS?
Edema - Vessels become leaky - more than normal response
Hyaline membranes - lots of proteins
What are the proliferative stages of DADS?
Interstitial inflammation
Interstitial fibrosis
What are the histological features of DADS?
- Protein rich oedema
- Fibrin
- Hyaline membranes
- Denuded basement membranes
What is the commonest interstitial lung disease that you will encounter?
Sarcoidosis
Apart from sarcoidosis what is the other granulomatous respnse?
Hypersensitivity pneumonitis
What is the histopathology of sarcoidosis?
Why is sarcoidosis called multisystem?
Involves many organs
Do most patients with sarcoidosis reach end stage fibrotic lung disease?
No ???
What is the presentation of sarcoidosis?
What is the diagnosis of sarcoidosis?
What is ACE?
A converting enzyme, converts angiotensin 1 to angiotensin 2.
Angiotensin II helps increase blood pressure by causing small blood vessels in the body to tighten or narrow.
Doctors most often use the ACE level test to monitor a disease called sarcoidosis. This condition causes inflammatory cells called granulomas to form in the body, leading to organ inflammation.
The granulomas associated with sarcoidosis increase the amount of ACE in the blood.
What are the hypersensitivity pneumonitis antiges?
What is the accute presentation for Hypersensitivity Pneumonitis?
What is the chronic presentation of Hypersensitivity pneumonitis?
What type of hypersensitivity causes Hypersensitivity Pneumonitis?
•Immune complex mediated combined Type III and Type IV Hypersensitivity reaction
Describe the granulomata in Hypersensitivity Pneumonitits
•Soft centriacinar epithelioid granulomata, causing interstitial pneumonitis
Hypersensitivty pneumonitis is said to cause Bronchiolitis obliterans, what does this mean?
Inflammatory obstruction of bronchioles
Bronchioles become damaged and inflamed by chemical particles/respiratory infections
Extensive scarring blocks airways
What are the causes of Usual interstitial pneumonitis?
Connective tissue diseases - •scleroderma and rheumatoid disease
Drugs
Asbestos
Viruses
Idiopathic/cryptogenic reasons
What is cryptogenic fibrosing alveolitis?
Old fashioned name for UIP
Describe the interstitium of UIP?
Patchy chronic inflammation
Type 2 pneumocytes hyperplasia
Smooth muscle and vascular proliferation
Proliferating fibroblastic foci
What are Proliferating Fibroblastic Foci?
Nodules of fibrous tissue in walls of alveoli
Reflects severity of disease
Who usually suffers from Idiopathic Pulmonary Fibrosis?
What is the clinical presentation of Idiopathic Pulmonary Fibrosis?
What is the prognosis for Idiopathic Pulmonary Fibrosis?
Usually dead within 5 years
What will you see in the ches X-ray for someone with Idiopathic Pulmonary Fibrosis?
•CXR : Basal/Posterior,
Diffuse infiltrates,
Cysts,
‘ Ground Glass ’
What is UIP Basal and posterior fibrosis with honeycombing often complicated with?
Peripheral adenocarcinoma
Describe the airway flow in interstitial lung disease
Normal air flow, diffusion is altered
