Restrictive lung disease Flashcards
What is restrictive interstitial lung disease
A large group of disorders that caues progressive scarring/thickening of the interstitium of the lung that prevents it from fully expanding with air. The scarring is irreversible.
What does the patient present with in Restrictive lung disease
S.O.B at rest or on exertion (MRC Dyspnoea Scale)
Dry cough
What are the clinical signs of restrictive lung disease
Type 1 respiratory failure (cyanotic)
Abnormal CXR
Low FEV1 and FVC (normalish ratio)
increased respiratory rate
How does restrictive lung disease cause Hypoxaemia
Reduced gas transfer
Ventilation/perfusion imbalance
Reduced compliance
What happens in end stage restrictive lung diease
Fibrosis and End-stage honeycombing of the lung.
What is the common acute response to interstitial lung injury called?
Diffuse alveolar damage
What typically causes Diffuse alveolar damage
Trauma Chemical/toxic inhalation Circulatory shock Drugs Infection Auto-immune response
What happens to the lungs after diffuse alveolar damage
Its swells with protein rich oedema
Interstitial inflammation and fibrosis (scarring)
deposition of hyaline membranes causing alveolar collapse and non-compliant lungs
What are the 2 categories of response to chronic restrictive interstitial lung disease
Granulomatous
Usual Interstitial Pneumonitis
What are the granulomatous responses to interstitual lung injury
Sarcoidosis
Hypersensitivity pneumonitis
What is sarcoidosis
Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomas.
How long does it take to recover from sarcoidosis
usually around 1-2 years
What are the clinical signs of Sarcoidosis
Acute athralgia
Erythema nodosum (swollen fat under skin, (usually legs))
Bilateral hilar lymphadenopathy
Tender reddish bumps or patches on the skin
What investigations should you do if suspect sarcoidosis
X-ray
serum ca and angiotensin converting enzyme
EBUS (sample)
what causes sarcoidosis
unknown
What age group and sex is more succeptible to Sarcoidosis
Females
20-40 years
What does a sarcoidosis positive Xray look like?
opaque
What can be prescribed to treat sarcoidosis
Corticosteroids
What is hypersensitivity pneumonitis
Hypersensitivity to inhaled organic dusts causing inflammation of the alveoli. It can lead to respiratory failure by reducing gas transfer.
What does the patient present with when they have hypersensitivity pneumonitis
Fever
Dry cough
Increased breathing rate
Wheeze
What are the clinical findings of hypersensitivity pneumonitis
Crackles
What is Usual Interstitial pneumonitis
Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the supporting framework (interstitium) of the lung.
What does the patient present with in Usual interstitial pneumonitis
S.O.B Cough Basal Crackles Cyanosis Clubbing
What is a marker of lung restriction and reduces in people with restrictive interstitial lung disease and how do you measure it?
Vital capacity
What areas commonly get Sarcoidosis
The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver, heart, and brain. Any organ, however, can be affected.
How do you investigate possible sarcoidosis
Pulmonary function tests
Bloods / urinalysis / ECG / TB skin test / eye exam
EBUS
What are the stages of Sarcoidosis
1 - nodal
2 - nodal and parenchymal
3 - nodal, parenchymal and
4 - honey comb (massive fibrosis) - lethal
How should you treat mild Sarcoidosis
No treatment
How should you treat Erythema Nodosum and athralgia
Nonsteroidal anti-inflammatory drugs (NSAIDs)
How should you treat Skin lesions / anterior uveitis / cough
Topical steroids
How should you treat Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia
Systematic steroids
What are the presenting symptoms for Idiopathic pulmonary fibrosis
Chronic breathlessness & cough
Typically 60-70 years old, commoner in men
Failed prescription for “LVF” or infection
Clubbed & crackles
What is the median survival rate for Idiopatathic pulmonary fibrosis
3 years
What medical treatment can you give to people with ideopathic pulmonary fibrosis
OAF (oral anti-fibrotic) - Pirfenidone, Nintedanib Lung transplant (best prognosis)
What are the most common restrictive lung diseases
sarcoidosis
ideopathetic pulmonary fibrosis
Hypersenstivity pneumontis
