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BRS YR2 > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is restrictive lung disease?

A

Diseases which make the lung volume small by restricting their expansion

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2
Q

What is intrinsic lung disease?

A
  • alteration in lung parenchyma
  • interstital lung disease
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3
Q

What is extrinsic lung disease?

A
  • compression or limited expansion of lungs
  • pleural, chest wall or neuromuscular
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4
Q

What is the lung parenchyma?

A

alveolar region of the lungs

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5
Q

What does the lung parenchyma consist of

A
  • alveolar type 1 epithelial cell
  • alveolar type 2 epithelial cell
  • fibroblasts
  • alveolar macrophages
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6
Q

What do alveolar epithelial cells do?

A
  • type 1 = gas exchange surface
  • type 2 = surfactant to reduce surface tension and stem cells for repair
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7
Q

What do fibroblasts do?

A

produce ECM

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8
Q

What do alveolar macrophages do?

A
  • phagocytosis or foreign material
  • produce surfactant
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9
Q

What is the interstital space?

A

space between alveolar epithelium and capillary endothelium

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10
Q

What is the function of the interstital space?

A
  • contains lymphatic vessels, fibroblasts and ECM
  • structural support for lungs
  • very thin to facillitate gas exchange
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11
Q

What are the different types of ILD?

A
  • idiopathic (IPF)
  • autoimmune (CTD)
  • exposure related (hypersensitivity pneumonitis)
  • and many more (200+)
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12
Q

What is ILD?

A

inflammation or fibrosis of the interstitial space

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13
Q

What is the presentation of ILD?

A
  • Progressive breathlessness
  • Non-productive cough
  • Limitation in exercise tolerance
  • Symptoms of connective tissue disease?
  • Occupational and exposure history
  • Medication history (drug induced ILD)
  • Family history (up to 20% of idiopathic ILDs are familial)
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14
Q

What are the clinical findings in ILD?

A
  • low oxygen saturation
  • fine bilateral inspiratory crackles
  • digital clubbing
  • features of connective tissue dieases
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15
Q

What are the investigations for ILD?

A
  • blood tests for anti-nuclear antibody (ANA), rheumatoid factor (RhF), CCP
  • pulmonary function test
  • 6-minute walk test
  • high resolution CT scan
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16
Q

Which invasive tests can be done for ILD?

A
  • bronchoalveolar lavage (BAL)
  • surgical lung biopsy (2-4% mortality)
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17
Q

What is the lung physiology in ILD?

A
  • decreased lung volume
  • decreased FVC
  • decreased diffusing capacity for lung CO (DLCO)
  • decreased arterial PO2
  • normal or increased FEV1/FVC
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18
Q

What is essential for ILD diagnosis?

A
  • high resolution CT scan
  • rotating xrays create high frequency reconstruction from many small slices
  • gives good resolution at level of secondary pulmonary lobule
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19
Q

How do high and low density areas appear on a HRCT?

A
  • high density = white
  • low density = dark
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20
Q

Which planes are viewed on an HRCT?

A
  • axial
  • sagittal
  • coronal
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21
Q

What are the three patterns of ILD?

A
  • usual interstitial pneumonia
  • non-specific interstitial pneumonia
  • organising pneumonia
22
Q

Which pattern is this?

A

usual interstitial pneumonia

23
Q

Which pattern is this?

A

non-specific interstitial pneumonia

24
Q

Which pattern is this?

A

organising pneumonia

25
Q

What is the treatment of early stage ILD?

A
  • Pharmacological therapy – immunosuppressive drugs, antifibrotics
  • Clinical trials
  • Patient education
  • Vaccination
  • Smoking cessation
  • Treatment of co-morbidities – gastroesophageal reflux, obstructive sleep apnoea, pulmonary hypertension
  • Pulmonary rehabilitation
26
Q

What is the treatment of late stage ILD?

A
  • Supplemental oxygen
  • Lung transplantation
  • Palliative care – symptom management, end-of-life care
27
Q

What is idiopathic pulmonary fibrosis?

A
  • Progressive, scarring lung disease of unknown cause
  • Incidence increases with age - most >60yrs
  • More common in men
  • Average decline in forced vital capacity (FVC) = 150 – 200mls / year
28
Q

What are acute exacerbations of IPF?

A
  • Occur in 5-15% of patients
  • Median survival 3-4 months
  • In-hospital mortality ~50%
29
Q

What is the median survival of untreated IPF?

A

3-5 years

30
Q

What are the predisposing factors of IPF?

A
  • genetic susceptibility (MUC5B, DSP)
  • environmental triggers (smoke, viruses, pollutants, dust)
  • cellular aging
31
Q

What are the characteristic features of IPF on a CT scan?

A
  • subpleural honeycombing
  • traction bronchiectasis
  • basal predominance
32
Q

What is harmful for people with IPF?

A

immunosuppression

33
Q

What is the treatment for IPF?

A
  • antifibriotics (nintedanib and pirfenidone)
  • slows progression but doesn’t cure or reverse damage
34
Q

What is hypersensitivity pneumonitis?

A
  • ILD caused by immune response to inhaled environmental anitgens
  • genetic and host factors contribute to who is susceptible to it
  • involves small airways and parenchyma
35
Q

What are the classifications of HP?

A

acute and chronic

36
Q

What is acute HP?

A
  • intermittent, high-level exposure
  • abrupt symptom onset
  • flu-like symptoms 4-12 hours after exposure
37
Q

What is chronic HP?

A
  • long-term, low-level exposure
  • two types: nonfibrotic and fibrotic
  • fibrotic has higher mortality
38
Q

What is the epidemiology of HP?

A
  • rare
  • mean onset age 50-60yrs
  • less frequent in smokers
  • 3x increase risk of death compared to gen pop
39
Q

What is HP driven by?

A
  • immune dysregulation
  • antigen exposure to the innate immune system leads to inflammatory response from Th and IgG
  • leads to accumulation of lymphocytes and granuloma formation
40
Q

What are some triggers of HP?

A
  • African parrot (droppings)
  • hay
  • plant and animal proteins
41
Q

How is HP diagnosed?

A
  • need history as an antigen is not identified in ~50% of patients
  • inspiratory squeaks caused by bronchiolitis
  • IgG to potential antigens
  • HRCT
  • BAL lymphocyte count > 30%
42
Q

What is the treatment of HP?

A
  • complete removal/avoidance of antigen
  • corticosteroids
  • immunosuppressants
  • antifibrotics (e.g. nintedanib) for progressive, fibriotic HP
43
Q

What are the connective tissue diseases associated with ILD?

A

systemic sclerois and rheumatoid arthiritis

44
Q

What is systemic sclerosis?

A
  • autoimmune connective tissue diesease
  • immune dysregulation and progressive fibrosis of skin and various internal organs
  • rare
  • affects young, middle-aged women
  • ILD develops in 30-40%
  • can progress slowly or quickkly
45
Q

What are the skin issues which occur in systemic sclerosis?

A
  • sclerodactyly
  • raynaud’s
  • telengectasias
  • digital ulcers
  • abnormal nailfold
46
Q

How is SSc classified based on skin involvement?

A
  • limited cutaneous SSc
  • diffuse cutaneous SSc
46
Q

Which type of SSc is ILD more common with?

A

Diffuse cutaneous SSc

47
Q

Which autoantibodies confirm SSc?

A
  • anti-centromere
  • anti-Scl-70 (also associated with ILD)
48
Q

What is the pahtogenesis of SSc-ILD?

A
  • tissue injury
  • vascular injury
  • autoimmunity
  • inflammation
  • fibrosis
49
Q

What are the HRCT patterns in SSc-ILD?

A

non-specific interstitial pneumonia is the most common

50
Q

How do you manage SSc-ILD?

A
  • treatment determined by disease extent on HRCT and lung function trajectory
  • corticosteroids are high risk for renal crisis
  • immunosuppressives (cyclophosphanamide, MMF)
  • nintedanib for progressive fibrosis

BRS YR2 (67 decks)

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