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BRS - Year 2 > 🫀🫁Cardio & Resp🫀🫁 - Restrictive Lung Diseases > Flashcards

🫀🫁Cardio & Resp🫀🫁 - Restrictive Lung Diseases Flashcards

1
Q

What is a restrictive lung disease?

A

A disease that restricts the expansion/volume of the lungs

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2
Q

What are the 2 categories of intrinsic lung disease?

A

Intrinsic lung disease - alterations to lung parenchyma
Extrinsic disorders - compress lungs or limit expansion

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3
Q

What do extrinsic disorders usually effect?

A

Pleura
Chest wall
Neuromuscular (decrease ability of respiratory muscles to inflate / deflate the lungs)

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4
Q

What is the most significant cause of intrinsic lung disease?

A

Interstitial lung disease

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5
Q

Outline the cells found in the lung parenchyma

A
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6
Q

What is found within the interstitial space of the lungs

A

Interstitial space – space between alveolar epithelium and capillary endothelium.
Contains lymphatic vessels, occasional fibroblasts and ECM
Structural support to lung
Very thin (few micrometers thick) to facilitate gas exchange

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7
Q

What key point must be remembered about macrophages’ relationship with the lungs?

A
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8
Q

What are the categories if interstitial lung diseases (ILD)?

A

Idiopathic
Autoimmune-related
Exposure related
With cysts or airspace filling
Sarcoidosis
Others (eosinophilic pneumonia etc…)

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9
Q

Give some examples of idiopathic ILDs

A

Idiopathic pulmonary fibrosis (IPF)
Nonspecific interstitial pneumonia (NSIP)
Desquamative interstitial pneumonia (DIP)

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10
Q

Give some examples of autoimmune-related ILDs

A

Connective tissue disease (CTD) associated
Rheumatoid arthritis-associated interstitial lung disease (RA-ILD)
Systemic sclerosis interstitial lung disease (SSc-ILD)

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11
Q

Give some examples of exposure related ILDs

A

Hypersensitivity pneumonitis (HP)
Drug induced etc…

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12
Q

What is the classic clinical presentation for ILDs?

A

Progressive breathlessness
Non-productive cough
Limitation in exercise tolerance
(Symptoms of CTD)
Can be useful:
-Occupational and exposure history
-Medication history (drug induced ILD)
-Family history (strong familial connections with idiopathic ILDs)

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13
Q

What would be found upon clinical examination of a patient with ILD?

A

Low oxygen saturations (resting or exertion)
Fine bilateral inspiratory crackles
Digital clubbing
(+/- features of CTD - skin, joints, muscles)

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14
Q

What are the changes to the lung physiology in ILD?

A

Scarring makes lungs stiff - decrease in lung compliance
↓ Lung volumes (TLC, FRC, RV)
↓ FVC
↓ diffusing capacity of lung for carbon monoxide (DLCO)
↓ arterial PO2 – particularly with exercise
FEV1/ FVC ratio stays normal (sometimes even elevated)

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15
Q

What investigation is used for ILD diagnosis?

A

High-resolution CT (HRCT)
Rotating X-ray images to generate cross-sectional images
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
Same as a standard X-ray

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16
Q

What is usual interstitial pneumonia (UIP)?

A

Usual interstitial pneumonia (UIP) is a histologic pattern
Strongly associated with ILDs, such as IPF

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17
Q

What would you see in a HRCT of usual interstitial pneumonia (UIP)?

A

Honeycombing - Clustered cystic spaces with well-defined walls
Traction bronchiectasis due to fibrosis pulling on bronchi
Minimal or absent ground-glass opacity
Commonly associated with IPF
Predominantly in the posterior and basal lungs

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18
Q

What would you see in a HRCT of NSIP?

A

Predominant ground-glass opacities
Some honeycombing, but much less than UIP
Involves the bilateral lower lobes but more uniform and diffuse than UIP

19
Q

What would you see in organising pneumonia?

A

Patchy, consolidation-like opacities, often subpleural or around bronchioles
No honeycombing or significant reticulation
Seen in cryptogenic organising pneumonia (COP) or secondary to infections, drugs, or autoimmune conditions

20
Q

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

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

21
Q

What are the principles of late stage ILD treatment?

A

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

22
Q

What is idiopathic pulmonary fibrosis (IPF)?

A

Progressive, scarring lung disease of unknown cause
1% of all deaths in the UK
Average decline in FVC

23
Q

Summarise the clinical trajectory of IPF

A

Highly variable
Feature progressive decline, but also sudden extreme decline due to acute exacerbations possible

24
Q

What is the prognosis of IPF?

A

Poor
Mediant untreated survival 3-5years

25
Q

What are the predisposing factors for IPF?

A

Genetic susceptibility (MUC5B, DSP)
Environmental triggers (smoking, viruses, pollutants, dusts)
Cellular ageing (telomere attrition, senescence)

26
Q

What is the proposed mechanism for IPF?

A

Initial alveolar epithelial injury in the presence of an altered microbiome
Inflammatory response - profibrotic macrophages activated by injury - release fibrosis-promoting cytokines
Fibroblast activation - production of excess ECM, including collagen
Leads to remodelling and honeycomb cyst formation

27
Q

What do we know for sure about the initiation of IPF?

A

IPF is initiated by alveolar epithelial injury

28
Q

What are the key histopathological features of IPF?

A

Microscopic Honeycomb Cysts (Image b):
-Represents areas of advanced fibrosis with dilated, thick-walled airspaces.
-These cysts are a hallmark of chronic, irreversible lung damage in UIP.
Fibroblastic Foci (Image c):
-Clusters of proliferating fibroblasts and myofibroblasts, often found at the interface between normal lung tissue and fibrotic areas
-Indicate active fibrosis

29
Q

What is meant by spatial and temporal heterogeneity, in the context of IPF?

A

Spatial heterogeneity - alternating areas of normal lung tissue, active fibrosis, and honeycombing
Temporal heterogeneity - presence of both early fibrotic lesions (fibroblastic foci) and advanced fibrosis (honeycomb changes) in the same lung biopsy

30
Q

What is the effectiveness of immunosuppression in IPF?

A

HARMFUL effect

31
Q

How is IPF treated?

A

Antifibrotics
Treatment - no cure

32
Q

What is hypersensitivity pneumonitis (HP)?

A

ILD caused by immune-mediated response in susceptible and sensitised individuals - inhaled environmental antigens
Involves small airways and parenchyma

33
Q

What are the classifications of HP?

A

Acute HP - Intermittent, high-level exposure – abrupt symptom onset, flu-like syndrome 4-12 hrs after exposure
Chronic HP - Long-term, low-level exposure
-Nonfibrotic (purely inflammatory)
-Fibrotic – associated with higher mortality

34
Q

What is the epidemiology of HP?

A

(Rare - incidence in UK ~ 1 per 100,0001
Mean age onset 50 – 60 yrs
M = F
Less frequent in smokers
Worldwide prevalence varies due to differences in antigen exposure, agricultural, industrial practices etc.
3- fold ↑ risk of death compared to general population)

35
Q

What is the driving force behind HP?

A

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

36
Q

How is HP diagnosed?

A

Detailed exposure history – antigen not identified in approx. 50% of cases
Inspiratory ‘squeaks’ on auscultation - caused by the coexisting bronchiolitis
Specific circulating IgG antibodies (serum precipitins) to potential antigens
HRCT imaging
Bronchoalveolar lavage (BAL) - lymphocyte count >30% indicative of HP

37
Q

What would you expect to see in an HRCT of HP?

A

Bronchiolocentric inflammation (centrilobular ground-glass nodules)
Narrowing of small airways (air trapping)
Interstitial inflammation (ground-glass opacity)
Mosaic attenuation and three-density patterns

38
Q

What is the treatment for HP?

A

Complete antigen removal / avoidance is crucial
Corticosteroids often used
Immunosuppressants e.g. mycophenolate mofetil (MMF) and azathioprine used but poor evidence base
Progressive, fibrotic HP – Nintedanib (antifibrotic)

39
Q

What is SSc-ILD?

A

Systemic sclerosis associated ILD
Autoimmune connective tissue disease characterised by immune dysregulation and progressive fibrosis that affects skin, with variable internal organ involvement
Rare - 10 – 50 cases per 1,000,000 per year
ILD develops in 30-40 % and is most common cause of death – 10-year mortality of 40%

40
Q

What are the clinical features of SSc?

A

Classified based on skin involvement -
Limited cutaneous SSc
(Pulmonary hypertension more common)
OR
Diffuse cutaneous SSc
(ILD more common)

41
Q

What is the pathogenesis of SSc-ILD?

A

Endothelial injury - increased permeability and recruitment of inflammatory cells into lung tissue
Autoimmunity and Inflammation - activation of T cells, B cells, and macrophages - overproduction of autoantibodies and cytokines
Fibroblast activation - excessive ECM deposition
Leads to progressive fibrosis

42
Q

What is the difference between SSc-ILD and IPF?

A

SSc-ILD secondary to systemic sclerosis, IPF cause is unknown
SSc-ILD has major inflammatory factor, which IPF does not
SSc-ILD characterised by non-specific interstitial pneumonia (NSIP), IPF features usual interstitial pneumonia (UIP)

43
Q

What are the management options for SSc-ILD?

A

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)
Immunosuppressives – cyclophosphamide, mycophenolate mofetil
Progressive fibrotic phenotype– Nintedanib (antifibrotic)

BRS - Year 2 (46 decks)

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