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1st Year - Clinical Respiratory > Restrictive lung disease > Flashcards

Restrictive lung disease Flashcards

1
Q

What is restrictive interstitial lung disease

A

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.

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2
Q

What does the patient present with in Restrictive lung disease

A

S.O.B at rest or on exertion (MRC Dyspnoea Scale)

Dry cough

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3
Q

What are the clinical signs of restrictive lung disease

A

Type 1 respiratory failure (cyanotic)
Abnormal CXR
Low FEV1 and FVC (normalish ratio)
increased respiratory rate

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4
Q

How does restrictive lung disease cause Hypoxaemia

A

Reduced gas transfer
Ventilation/perfusion imbalance
Reduced compliance

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5
Q

What happens in end stage restrictive lung diease

A

Fibrosis and End-stage honeycombing of the lung.

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6
Q

What is the common acute response to interstitial lung injury called?

A

Diffuse alveolar damage

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7
Q

What typically causes Diffuse alveolar damage

A 
Trauma
Chemical/toxic inhalation 
Circulatory shock
Drugs
Infection 
Auto-immune response
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8
Q

What happens to the lungs after diffuse alveolar damage

A

Its swells with protein rich oedema
Interstitial inflammation and fibrosis (scarring)
deposition of hyaline membranes causing alveolar collapse and non-compliant lungs

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9
Q

What are the 2 categories of response to chronic restrictive interstitial lung disease

A

Granulomatous

Usual Interstitial Pneumonitis

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10
Q

What are the granulomatous responses to interstitual lung injury

A

Sarcoidosis

Hypersensitivity pneumonitis

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11
Q

What is sarcoidosis

A

Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomas.

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12
Q

How long does it take to recover from sarcoidosis

A

usually around 1-2 years

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13
Q

What are the clinical signs of Sarcoidosis

A

Acute athralgia
Erythema nodosum (swollen fat under skin, (usually legs))
Bilateral hilar lymphadenopathy
Tender reddish bumps or patches on the skin

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14
Q

What investigations should you do if suspect sarcoidosis

A

X-ray
serum ca and angiotensin converting enzyme
EBUS (sample)

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15
Q

what causes sarcoidosis

A

unknown

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16
Q

What age group and sex is more succeptible to Sarcoidosis

A

Females

20-40 years

17
Q

What does a sarcoidosis positive Xray look like?

A

opaque

18
Q

What can be prescribed to treat sarcoidosis

A

Corticosteroids

19
Q

What is hypersensitivity pneumonitis

A

Hypersensitivity to inhaled organic dusts causing inflammation of the alveoli. It can lead to respiratory failure by reducing gas transfer.

20
Q

What does the patient present with when they have hypersensitivity pneumonitis

A

Fever
Dry cough
Increased breathing rate
Wheeze

21
Q

What are the clinical findings of hypersensitivity pneumonitis

A

Crackles

22
Q

What is Usual Interstitial pneumonitis

A

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.

23
Q

What does the patient present with in Usual interstitial pneumonitis

A 
S.O.B
Cough 
Basal Crackles
Cyanosis 
Clubbing
24
Q

What is a marker of lung restriction and reduces in people with restrictive interstitial lung disease and how do you measure it?

A

Vital capacity

25
Q

What areas commonly get Sarcoidosis

A

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.

26
Q

How do you investigate possible sarcoidosis

A

Pulmonary function tests
Bloods / urinalysis / ECG / TB skin test / eye exam

EBUS

27
Q

What are the stages of Sarcoidosis

A

1 - nodal
2 - nodal and parenchymal
3 - nodal, parenchymal and
4 - honey comb (massive fibrosis) - lethal

28
Q

How should you treat mild Sarcoidosis

A

No treatment

29
Q

How should you treat Erythema Nodosum and athralgia

A

Nonsteroidal anti-inflammatory drugs (NSAIDs)

30
Q

How should you treat Skin lesions / anterior uveitis / cough

A

Topical steroids

31
Q

How should you treat Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

A

Systematic steroids

32
Q

What are the presenting symptoms for Idiopathic pulmonary fibrosis

A

Chronic breathlessness & cough
Typically 60-70 years old, commoner in men
Failed prescription for “LVF” or infection
Clubbed & crackles

33
Q

What is the median survival rate for Idiopatathic pulmonary fibrosis

A

3 years

34
Q

What medical treatment can you give to people with ideopathic pulmonary fibrosis

A 
OAF (oral anti-fibrotic) - Pirfenidone, Nintedanib
Lung transplant (best prognosis)
35
Q

What are the most common restrictive lung diseases

A

sarcoidosis
ideopathetic pulmonary fibrosis
Hypersenstivity pneumontis

1st Year - Clinical Respiratory (11 decks)

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