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BRS Flashcards (Y2) > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is restrictive lung disease?

A

Lung volumes are small, expansion is limited by
- Alterations to lung parenchyma - intrinsic disorders, e.g. ILD
- Compression/limited expansion of the lungs (pleural, chest wall, neuromuscular) - extrinsic disorders

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2
Q

What is the lung parenchyma?

A

Alveolar regions of the lung

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3
Q

What the different cellular components of the lung parenchyma and what are their functions?

A

Alveolar type 1 epithelial cell - gas exchange surface
Alveolar type 2 epithelial cell - surfactant to reduce surface tension; stem cell for repair
Fibroblasts - produce extra cellular matrix e.g. collagen type 1; also plays a role in cell signalling
Alveolar macrophages - phagocytose foreign material; surfactant

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4
Q

What is the interstitial space and what is its function?

A

Space between alveolar epithelium and capillary endothelium
Contains lymphatic vessels, occasional fibroblasts and ECM
Provides structural support to lung
Very thin (few micrometers thick) to facilitate gas exchange

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5
Q

What are interstitial lung diseases characterised by?

A

Inflammation or fibrosis in the interstitial space

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6
Q

What are the different categories of interstitial lung diseases?

A

Idiopathic
Autoimmune-related
Exposure related, e.g. hypersensitivity pneumonitis
With cysts or airspace filling
Sarcoidosis
Others e.g. eosinophilic pneumonia

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7
Q

Different patterns of fibrosis influence survival. True or false?

A

True

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8
Q

What may be present in a history suggestive of ILD?

A

Progressive breathlessness
Non-productive cough
Limitation in exercise tolerance
Symptoms of connective tissue disease (joint problems, rashes, dry eyes/mouth)
Occupational and exposure history
Medication history (drug induced ILD)
Family history (up to 20% idiopathic ILDs are familial)

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9
Q

What may you find on clinical examination of someone with suspected ILD?

A

Low oxygen saturations (resting or exertion)
Fine bilateral inspirations crackles
Digital clubbing
(+/- features of connective tissue disease)

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10
Q

What are some investigations used in ILD?

A

Blood tests - anti-nuclear antibody (ANA), rheumatoid factor (RhF), anti-citrullinated peptide (CCP)
Pulmonary function tests
6-minute walk test (6MWT)
High resolution CT scan (HRCT)
Invasive testing: bronchoalveolar lavage (BAL) and surgical lung biopsy

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11
Q

Why is a high-resolution CT scan useful in ILD diagnosis?

A

Can allow you to see the pattern of fibrosis - useful for distinguishing between disease types

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12
Q

What oxygen saturation is associated with an increased risk of death in the 6MWT?

A

SpO2 </= 88%

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13
Q

Describe the lung physiology in ILD

A

Scarring makes lungs stiff - reduced lung compliance
Reduced lung volumes (TLC, FRC, RV)
Reduced FVC
Reduced diffusing capacity of lung for carbon monoxide (DLCO)
Reduced arterial PO2 - particularly with exercise
Normal or increased FEV1/FVC ratio

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14
Q

What type of ILD does this image show and what are the features of disease?

A

Usual interstitial pneumonia
Honeycomb cysts - advanced fibrosis

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15
Q

What does this image show and what are the features of disease?

A

Non-specific interstitial pneumonia
Greyer areas - ground glass (hazy) opacifications

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16
Q

What is show in this image and what are the features of disease?

A

Organising pneumonia
Consolidation (dense, white area)

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17
Q

Which HCP may be involved in a diagnosis of ILD?

A

Integration of clinical, radiological +/- pathological information:
- Pulmonologist
- Radiologist
- Respiratory physiologist
- Rheumatologist
- Clinical nurse specialist
- Physiotherapist/occupational therapist

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18
Q

What are the general principles of ILD management in early disease?

A

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

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19
Q

What are the general principles of ILD management in late disease?

A

Supplemental oxygen
Lung transplantation
Palliative care - symptom management, end-of-life care

20
Q

What is idiopathic pulmonary fibrosis?

A

Progressive, scarring lung disease or unknown cause
Average decline in FVC = 150-200mls/year

21
Q

What is the epidemiology of IPF?

A

6,000 new cases diagnosed each year
1% of UK deaths
Incidence increases with age - most >60yrs
More common in men

22
Q

How common are acute exacerbations of IPF and what is the median survival?

A

Occur in 5-15% of patients
Median survival -> 3-4 months
In-hospital mortality ~50%

23
Q

What is the median untreated survival of IPF?

A

3-5 years

24
Q

What factors may predispose to IPF?

A

Genetic susceptibility
- MUC5B (airway mucin gene), DSP
Environmental triggers
- Smoke, viruses, pollutants, dust
Cellular ageing
- Telomere attrition, senescence

25
Q

What is the proposed mechanism of IPF?

A

Injury to epithelium (type 1 alveolar cells)
Injured cells die and release profibrotic factors which recruit fibroblasts
Fibroblasts lay down scar tissue - limits/prevents gas exchange
Remodelling of lung tissue - honeycomb cyst formation

26
Q

Describe the meaning of spatial and temporal heterogeneity in the histopathology of IPF

A

Classic feature of usual interstitial pneumonia
Areas of normal lung tissue juxtaposed with areas of diseased lung tissue

27
Q

Label this image

A
28
Q

These scans are characteristic of which condition?

A

Idiopathic pulmonary fibrosis

29
Q

Immunosuppressants slow the progression of IPF. True or false?

A

False, actually harmful - increased risk of death and hospitalisation

30
Q

What drugs are used to manage IPF?

A

Antifibrotics:
- Nintedanib - tyrosine kinase inhibitor
- Pirfenidone - a pyridine compound

31
Q

Does antifibrotics treat IPF?

A

No, slow disease progression - not a cure

32
Q

What is hypersensitivity pneumonitis?

A

ILD caused by immune-mediated response in susceptible and sensitised individuals to inhaled environmental antigens
Genetic and host factors may explain why only a few exposed individuals get HP
Involves small airways and parenchyma

33
Q

How is hypersensitivity pneumonitis classified?

A

Acute HP
- Intermittent, high level exposure
- Abrupt symptom onset
- Flu-like syndrome 4-12 hours after exposure
Chronic HP
- Long-term, low-level exposure
- Nonfibrotic (purely inflammatory)
- Fibrotic - increased risk of mortality

34
Q

Describe HP epidemiology

A

Rare - incidence 1/100,000 (UK)
Mean age onset 50-60 years
M=F
Less frequent in smokers
3-fold increased risk of death compared to general population

35
Q

Describe the pathophysiology of HP

A

Antigen exposure and processing by the innate immune system
Inflammatory response mediated by T-helper cells and antigen-specific immunoglobulin (IgG) antibodies (formation of immune complexes)
Accumulation of lymphocytes and formation of granulomas

36
Q

What are the key diagnostic elements in HP?

A

Detailed exposure history - antigen not identified in ~50% of cases
Inspiratory ‘squeaks’ on auscultation (caused by existing bronchiolitis)
Specific circulating IgG antibodies to potential antigens
HRCT
Bronchoalveolar lavage - lymphocyte count >30%

37
Q

How is HP treated?

A

Complete antigen removal/avoidance
Corticosteroids
Immunosuppressants e.g. mycophenolate mofetil (MMF) and azathioprine (poor evidence base)
Progressive, fibrotic HP - Nintedanib

38
Q

What is systemic sclerosis associated (SSc) ILD?

A

SSc - autoimmune connective tissue disease characterised by immune dysregulation and progressive fibrosis that affects skin with variable organ involvement
ILD develops in 30-40% of cases - most common cause of death
Slow indolent course vs rapid progression (track lung function)

39
Q

What is the epidemiology of SSc?

A

10-50/1,000,000
Affects young, middle-aged women

40
Q

What factors may worsen prognosis of SSc ILD?

A

Male
Older age
Smoker
>20% extent on HRCT
FVC <70%

41
Q

What are the two main types of SSc and how are they classified?

A

Classified based on skin involvement:
- Limited cutaneous SSc (up to elbow) - pulmonary hypertension more common
- Diffuse cutaneous SSc (more generalised, e.g. face involvement) - ILD more common

42
Q

Label these features. What condition are they characteristic of?

A

Systemic sclerosis

43
Q

What autoantibodies are associated with SSc?

A

Anti-centromere
Anti-Scl-70 - increased risk of ILD

44
Q

Describe the pathogenesis of SSc-ILD

A

Tissue injury -> vascular injury -> autoimmunity -> fibrosis -> inflammation-> more fibrosis

45
Q

What type of ILD pattern is show in these HRCTs and what condition are they associated with?

A

Non-specific interstitial pneumonia (NSIP)
Most common SSc-ILD pattern

46
Q

How is SSc-ILD managed?

A

Determined by disease extent on HRCT and lung function trajectory (monitored every 3-6 months)
Corticosteroids - controversial, risk of renal crisis (high doses)
Immunosuppressants- cyclophosphamide, mycophenolate mofetil (MMF)
Progressive fibrotic phenotype - Nintedanib

Typically not suitable for lung transplant due to oesophageal dysmotility and reflux

BRS Flashcards (Y2) (79 decks)

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