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RESPIRATORY > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What types of cells are found in the pulmonary interstitium?

A
  • Type I and II alveolar cells

- Thin elastin-rich connective tissue (contains capillaries)

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2
Q

Describe the difference between early and late stage Interstitial Lung Disease?

A

Early stage = alveolitis (injury with inflammatory cell infiltration)

Late stage = fibrosis

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3
Q

What are the potential causes of ILD?

A
  • Environmental (minerals, drugs etc)
  • Hypersensitivity (mouldy hay, avian proteins)
  • Unknown (idiopathic e.g. IPF)
  • Due to Connective tissue diseases
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4
Q

HOw is a biopsy completed when you suspect ILD in a patient?

A

Transbronchial biopsy – special forceps used at bronchoscopy

Thoracoscopic biopsy – more invasive but gets more tissue

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5
Q

Which types of interstitial lung disease are chronic?

A
  • Idiopathic pulmonary fibrosis (IPF)
  • Sarcoidosis
  • Hypersensitivity pneumonitis (extrinsic allergic alveolitis)
  • Pneumoconiosis
  • Connective tissue diseases
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6
Q

How does Idiopathic pulmonary fibrosis appear pathologically?

A
  • Subpleural and basal fibrosis (HONEYCOMBING)
  • may be associated inflammation
  • Terminal disease shows lung replaced by dilated spaces AND whole thing surrounded by fibrous walls
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7
Q

What sign in the hands can be caused by idiopathic pulmonary fibrosis?

A

Finger clubbing

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8
Q

What is Hypersensitivity Pneumonitis/ Extrinsic Allergic ALveolitis?

A 
Chronic inflammatory disease (not fibrotic)
Affects:
- Small airways
- Interstitium
- Causes occasional granulomas
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9
Q

What can cause Hypersensitivity Pneumonitis/ Extrinsic Allergic Alveolitis?

A
  • Thermophilic bacteria – Farmers lung
  • Avian proteins – Bird fanciers lung
  • Fungi – Malt workers lung
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10
Q

What is sarcoidosis?

A
  • Granulomas form in many body systems
  • cause is unknown
  • Pulmonary involvement is common
  • Most cases mild and self-limiting
  • granulomas are non-caseating (difference from TB)
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11
Q

Aside from granulomas, what else does sarcoidosis cause?

A
  • Uveitis (inflammation of iris)
  • Erythema nodosum (skin)
  • Lymphadenopathy (especially in hilum of lungs)
  • Hypercalcaemia
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12
Q

What pulmonary effects do connective tissue diseases cause?

A
  • Interstitial fibrosis (milder than IPF)
  • Pleural effusions
  • Rheumatoid nodules (can be seen on CXR)
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13
Q

What is pneumoconiosis and what can cause this condition?

A
  • lung disease caused by mineral dust exposure:
  • Asbestosis
  • Coal workers lung
  • Silicosis
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14
Q

What does the degree of pneumoconiosis depend on?

A
  • Particle size (1-5mm)
  • Reactivity of particle
  • Clearance of particle
  • Host response
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15
Q

Does ILD cause an obstructive or restrictive lung disease pattern on spirometry?

A

RESTRICTIVE

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16
Q

Describe the difference between acute and chronic sarcoidosis

A

ACUTE

  • erythema nodosum
  • bilateral hilar lymphadenopathy
  • arthritis
  • uveitis
  • fever

CHRONIC

  • lung infiltrates
  • peripheral lymphadenopathy
  • hypercalcaemia
  • Other organs: renal, myocardial, neurological, hepatitis, splenomegaly
17
Q

What are the potential differential diagnoses for sarcoidosis?

A
  • TB (tuberculin test -ve)
  • Lymphoma
  • Carcinoma
  • fungal infection
18
Q

How is chronic sarcoidosis treated?

A

ACUTE

  • supportive Tx as self limiting
  • oral steroids if vital organs are affected

CHRONIC

  • steroids
  • immunosuppression
19
Q

What type of Hypersensitivity reaction is responsible for extrinsic allergic alveolitis?

A

Type III

- immun complexes deposited in lung tissue

20
Q

How does Acute EAA present?

A
  • cough, breathless, fever, myalgia –
  • SOB
  • fever
  • myalgia
  • come on a few hrs after exposure
21
Q

How does Chronic EAA present?

A
  • progressive breathlessness and cough
  • crackles in chest
  • CXR pulmonary fibrosis in the upper zones
  • Restrictive defect
22
Q

How does idiopathic pulmonary fibrosis present?

A
  • progressive breathlessness
  • dry cough
  • clubbing
  • bilateral fine inspiratory crackles
  • restrictive defect
  • CxR = bilateral infiltrates
  • CT scan = fibrotic shadowing, worse at the lung bases
23
Q

What differentials should you check for before diagnosing a patient with IPF?

A
  • occupational disease (asbestosis, silicosis)
  • connective tissue disease (RhA, scleroderma, Sjogrens Disease, SLE)
  • left ventricular failure
  • sarcoidosis
  • extrinsic allergic alveolitis
24
Q

How is IPF treated?

A
  • Steroids and immunosuppressants do NOT change course of disease

=> New antifibrotic drugs – PIRFENIDONE and NINTEDANIB

  • these slow disease progression but dont reverse fibrosis
  • very expensive
  • Give O2 if pt is hypoxic
  • Lung transplantation in young patients
25
Q

What is the difference between simple and complicated pneumoconiosis?

A

Simple - CXR abnormality but lung function normal

Complicated - massive fibrosis (often in upper zone) causes breathlessness and restrictive pattern

26
Q

Prolonged asbestos exposure can cause pulmonary fibrosis. TRUE/FALSE?

A

TRUE - “Asbestosis”

  • Diffuse pulmonary fibrosis
  • restrictive defect
  • Asbestos bodies in sputum
  • Asbestos fibres in lung biopsy

RESPIRATORY (9 decks)

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