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Phase 1b - Cardiovascular & Respiratory Medicine > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is a restrictive lung disease?

A
  • Expansion of the lung is restricted decreasing lung volume
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2
Q

What is the aetiology of a restrictive lung disease (2)?

A
  • Intrinsic lung disease:
    • Alterations to lung parenchyma interstitial lung disease (ILD)
  • Extrinsic disorders:
    • Compress lungs or limit expansion
      • Pleural
      • Chest wall
      • Neuromuscular (decrease ability of respiratory muscles to inflate / deflate the lungs)
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3
Q

What are the important cellular components of the lung parenchyma (4)?

Lung parenchyma: the alveolar regions of the lung

A
  • Alveolar type 1 epithelial cell - gas exchange surface (approx. 70m2)
  • Alveolar type 2 epithelial cell - surfactant to reduce surface tension, stem cell for repair
  • Fibroblasts - produce extracellular matrix (ECM) e.g Collagen type 1
  • Alveolar macrophages - phagocytose foreign material, surfactant
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4
Q

What is the function of the interstitial space (3)?

Interstitial space: space between alveolar epithelium and capillary endothelium

A
  • Contains lymphatic vessels, occasional fibroblasts and ECM
  • Structural support to lung
  • Very thin (few micrometers thick) to facilitate gas exchange
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5
Q

What is the aetiology of interstitial lung disease (ILD) (6)?

A
  • Idiopathic
    • IPF / NSIP / DIP etc.
  • Autoimmune-related
    • CTD associated (RA-ILD, SSc-ILD) etc.
  • Exposure related
    • Hypersensitivity pneumonitis (HP) / Drug-induced etc.
  • With cysts or airspace filling
  • Sarcoidosis
  • Others
    • Eosinophilic pneumonia etc.
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6
Q

What is the clinical presentation of interstitial lung disease (ILD) (7 symptoms / 4 signs)?

A

Symptoms:
* Progressive breathlessness
* Non-productive cough
* Limitation in exercise tolerance
* Symptoms of connective tissue disease
* Occupational and exposure history
* Medication history (i.e. drug induced ILD)
* Family history (up to 20% of idiopathic ILDs are familial)

Signs:
* Low oxygen saturations (resting or exertion)
* Fine bilateral inspiratory crackles
* Digital clubbing
* (+/- features of connective tissue disease - skin, joints, muscles)

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7
Q

Outline the pathophysiology of interstitial lung disease (ILD).

A
  1. Scarring makes the lung stiff
  2. Decreased lung compliance
  3. Decreased lung volumes (TLC, FRC, RV)
  4. Decreased FVC
  5. Decreased diffusing capacity of lung for carbon monoxide (DLCO)
  6. Decrease arterial PO2 – particularly with exercise
  7. Normal or increased FEV1/ FVC ratio
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8
Q

What investigations are suggested in suspected interstitial lung disease (ILD) (8)?

A
  • Blood tests:
    • Anti-nuclear antibody (ANA)
    • Rheumatoid factor (RhF)
    • Anti-citrullinated peptide (CCP)
  • Pulmonary function tests
  • 6-minute walk test (6MWT) - SpO2 ≤ 88% associated with increased risk of death
  • High-resolution CT scan (HRCT)
  • Invasive testing:
    • Bronchoalveolar lavage (BAL)
    • Surgical lung biopsy (2-4% mortality)
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9
Q

What is a high-resolution CT (HRCT)?

HRCT is essential for ILD diagnosis.

A
  • CT uses X-rays to obtain cross-sectional images
  • Rotating X-ray source and detectors spin around the patient gathering data
  • HRCT - thin slices and high-frequency reconstruction - gives good resolution at level of secondary pulmonary lobule (smallest functional lung unit identifiable on CT)

  • High: density substances e.g. bone absorb more x-rays and appear whiter
  • Low: density substances e.g. air absorb few x-rays and appear darker
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10
Q

How is suspected interstitial lung disease (ILD) diagnosed?

A
  • Integration of clinical, radiological +/- pathological information to make a diagnosis
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11
Q

How is early interstitial lung disease (ILD) managed (7)?

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
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12
Q

How is late interstitial lung disease (ILD) managed (3)?

A
  • Supplemental oxygen
  • Lung transplant
  • Paliative care (symptom management / end-of-life care)
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13
Q

Outline the epidemiology of idiopathic pulmonary fibrosis (IPF) (5).

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  • Progressive, scarring lung disease of unknown cause
  • 6,000 new cases diagnosed each year
  • 1% of all deaths in UK
  • Incidence increases with age - most >60yrs
  • More common in men
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14
Q

What are the risk factors of idiopathic pulmonary fibrosis (IPF) (7)?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  • Genetic susceptibility
    • MUC5B
    • DSP
  • Environmental triggers
    • Smoke
    • Viruses
    • Pollutants
    • Dusts
  • Cellular ageing
    • Telomere attrition
    • Senescence
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15
Q

What is the clinical presentation of idiopathic pulmonary fibrosis (IPF) (7 symptoms / 4 signs)?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A

Symptoms:
* Progressive breathlessness
* Non-productive cough
* Limitation in exercise tolerance
* Symptoms of connective tissue disease
* Occupational and exposure history
* Medication history (i.e. drug induced ILD)
* Family history (up to 20% of idiopathic ILDs are familial)

Signs:
* Low oxygen saturations (resting or exertion)
* Fine bilateral inspiratory crackles
* Digital clubbing
* (+/- features of connective tissue disease - skin, joints, muscles)

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16
Q

Outline the pathophysiology of idiopathic pulmonary fibrosis (IPF) (4).

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  1. IPF is initiated by alveolar epithelial injury
    -> Denuded alveolar epithelium seen by electron microscopy
  2. Targeted injury to AECIIs (in mouse model)
  3. Increased collagen deposition
  4. Re-epithelialization disturbed in IPF
Proposed mechanisms of IPF (Still unclear)
17
Q

What investigations are suggested in suspected idiopathic pulmonary fibrosis (IPF) (8)?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  • Blood tests:
    • Anti-nuclear antibody (ANA)
    • Rheumatoid factor (RhF)
    • Anti-citrullinated peptide (CCP)
  • Pulmonary function tests
  • 6-minute walk test (6MWT) - SpO2 ≤ 88% associated with increased risk of death
  • CT scan
  • Invasive testing:
    • Bronchoalveolar lavage (BAL)
    • Surgical lung biopsy (2-4% mortality)
18
Q

How would the lung histopathology of idiopathic pulmonary fibrosis (IPF) be different from a normal lung pathology (2)?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A

Presence of:
* Microscopic honeycomb cyst
* Fibroblastic foci

19
Q

How would idiopathic pulmonary fibrosis (IPF) present on a CT scan (3)?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  • Subpleural honeycombing
  • Traction bronchiectasis
  • Basal predominance
20
Q

How is idiopathic pulmonary fibrosis (IPF) management different to the interstitial lung disease (ILD) management?

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
  • Immunosuppression eventhough helpful in ILD, it is harmful in IPF
  • Antifibrotics slow disease progression in IPF but do not cure
21
Q

Name 3 drugs currently in trial for the management of idiopathic pulmonary fibrosis (IPF).

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD).

A
22
Q

What is hypersensitivity pneumonitis (HP)?

A
  • ILD caused by immune - mediated response in susceptible and sensitised individuals to inhaled environmental antigens
23
Q

What is acute hypersensitivity pneumonitis (HP) (4)?

Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Intermittent
  • High-level exposure
  • Abrupt symptom onset
  • Flu-like syndrome 4-12 hrs after exposure
24
Q

What is chronic hypersensitivity pneumonitis (HP)? What are the 2 subtypes?

Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Long-term, low-level exposure
    • Nonfibrotic (purely inflammatory)
    • Fibrotic - associated with higher mortality
25
Q

Outline the epidemiology of hypersensitivity pneumonitis (HP).

Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Rare -> incidence in UK ~ 1 per 100,000
    • Worldwide prevalence varies due to differences in antigen exposure, agricultural, industrial practices etc.
  • Mean age onset 50-60 yrs
  • M=F
  • Less frequent in smokers
  • 3- fold increased risk of death compared to general population
26
Q

What are the risk factors of hypersensitivity pneumonitis (HP) (2)?

Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Genetic susceptibility
  • Inhaled environmental factors
27
Q

Outline the pathophysiology of hypersensitivity pneumonitis (HP).

Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Antigen exposure and processing by the innate immune system
  • Inflammatory response mediated by T-helper cells and antigen-specific immunoglobulin (Ig) G antibodies
  • Accumulation of lymphocytes and formation of granulomas
28
Q

What is the clinical presentation of hypersensitive pneumonitis (HP) (7 symptoms / 4 signs)?

Hypersensitive pneuomonitis (HP) is a type of interstitial lung disease (ILD).

A

Symptoms:
* Progressive breathlessness
* Non-productive cough
* Limitation in exercise tolerance
* Symptoms of connective tissue disease
* Occupational and exposure history
* Medication history (i.e. drug induced ILD)
* Family history (up to 20% of idiopathic ILDs are familial)

Signs:
* Low oxygen saturations (resting or exertion)
* Fine bilateral inspiratory crackles
* Digital clubbing
* (+/- features of connective tissue disease - skin, joints, muscles)

29
Q

What investigations are suggested in suspected hypersensitive pneumonitis (HP) (5)?

Hypersensitive pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Detailed exposure history
    • Antigen not identified in ~50%
  • Blood tests:
    • IgG antibodies circulating
    • Bronchoalveolar lavage (BAL) lymphocyte count >30%
  • High resolution CT scan (HRCT)
  • Clinical examination:
    • Inspiratory ‘squeaks’ on auscultation
      • Caused by the coexisting bronchiolitis
30
Q

What is the recommended management of hypersensitive pneumonitis (HP) (non-fibrotic) (3)?

Hypersensitive pneumonitis (HP) is a type of interstitial lung disease (ILD).

A
  • Complete antigen removal / avoidance is crucial
  • Corticosteroids often used
  • Immunosuppressants e.g. mycophenolate mofetil (MMF) and azathioprine used but poor evidence base
31
Q

What additional medication is required for fibrotic hypersensitive pneumonitis (HP)?

Hypersensitive pneumonitis (HP) is a type of interstitial lung disease (ILD).

A

Nintedanib (antifibrotic)

32
Q

What is systemic sclerosis associated - interstitial lung disease (SSc-ILD)?

A
  • SSc is an autoimmune connective tissue disease characterised by immune dysregulation and progressive fibrosis that affects skin, with variable internal organ involvement
33
Q

Outline the epidemiology of systemic sclerosis associated - interstitial lung disease (SSc-ILD) (5).

A
  • Rare: 10-50 cases per 1,000,000 per year
  • Affects young, middle-aged women
  • ILD develops in 30-40% of SS patients and is most common cause of death
    • 10-year mortality of 40%
  • Slow indolent course vs. rapid progression
  • Worse survival when:
    • Male
    • Older age
    • Smoker
    • 20% extent on HRCT
    • FVC < 70%
34
Q

What is the clinical presentation of systemic sclerosis associated - interstitial lung disease (SSc-ILD) (5)?

A
  • Sclerodactyly
  • Raynaud’s phenomenon
  • Telengectasias
  • Abnormal nailfold capillaroscopy
  • Digital ulcer
35
Q

Systemic sclerosis associated - interstitial lung disease (SSc-ILD) is classified based on skin involvement. What are the 2 classifications?

A
  • Limited cutaneous SSc -> Pulmonary hypertension more common
  • Diffuse cutaneous SSc -> Interstitial lung disease (ILD) more common
36
Q

What autoanitbodies would be elevated in systemic sclerosis associated - interstitial lung disease (SSc-ILD) (2)?

A
  • Anti-centromere
  • Anti-Scl-70
37
Q

Outline the pathogenesis of systemic sclerosis associated - interstitial lung disease (SSc-ILD).

A
38
Q

What HRCT patterns would present in systemic sclerosis associated - interstitial lung disease (SSc-ILD)?

A
  • Non-specific interstitial pneumonia (NSIP) pattern is the most common pattern
39
Q

What is the management of systemic sclerosis associated - interstitial lung disease (SSc-ILD) (3)?

A
  • Immunosuppressives:
    • Cyclophosphamide
    • Mycophenolate mofetil (MMF)
  • Nintedanib (antifibrotic) in progressive fibrotic phenotype

  • Determined by disease extent on HRCT and lung function trajectory (monitor every 3-6 months)
  • Corticosteroid use is controversial and risk of renal crisis with high doses (>10mg/day)

Phase 1b - Cardiovascular & Respiratory Medicine (21 decks)

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