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Respiration > Pathology of Restrictive Lung Diseases > Flashcards

Pathology of Restrictive Lung Diseases Flashcards

1
Q

What happens to lung compliance in restrictive disease and why?

A

Reduced compliance
- Stiff lungs due to thicker interstitium
- Inflammatory process eventually leading to collagen being laid down which doesn’t stretch

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2
Q

What happens to FEV1 and FVC in restrictive lung disease?

A

Low FEV1 and FVC
FEV1/FVC normal or increased ratio
Reduced gas transfer
Ventilation/perfusions imbalance

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3
Q

What is the presentation of diffuse lung disease?

A

Discovery of abnormal CXR or CT scan
Dyspnoea
- SOB of exertion
- Progressing to SOB at rest
Respiratory failure
Heart failure

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4
Q

What are the main features of a CXR of interstitial lung disease?

A

Compressed lungs
Fluffy heart border

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5
Q

What is the pathology of diffuse alveolar damage (DAD or ARDS)

A
  1. Acute inflammatory response involving lots of tissue damage
  2. Early vascular phase producing lots of fluid (exudate stage) - may result in haemorrhage
  3. Cellular and proliferation - chronic inflammatory process
  4. Disease evolves at different stages in different parts of the lung
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6
Q

What is DADs associated with?

A

Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection including viruses
Autoimmune disease
Radiation
Idiopathic

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7
Q

What is the histology of DADs like?

A

Protein rich
Fibrin
Hyaline membranes
Denuded basement membranes
Attempt to remodel lung
Scarring of the lung

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8
Q

What is a hyaline membrane?

A

Layer of proteinates material becoming layered on alveolar walls.

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9
Q

What occurs when the lung attempts to remodel?

A

Epithelial proliferation
Fibroblast proliferation

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10
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder of unknow aetiology

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11
Q

What is the prognosis of sarcoidosis?

A

Relatively good
- There is only a small possibility it will progress to end stage fibrosis

Stage 1 usually resolves after 2 years
Stages 2&3 may resolve, persist or progress

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12
Q

What is the histopathology of sarcoidosis?

A

Epithelioid and giant cell granulomas
Necrosis/cessation very unusual
Little lymphoid infiltrate
Variable associated fibrosis

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13
Q

What is the incidence of sarcoidosis worldwide and what does this suggest?

A

3-4/100,000 in UK
20/100,000 in African-Americans
Low in equatorial regions

*A disease of temperate climate

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14
Q

What organs can be involved in sarcoidosis and what is the likelihood of involvement?

A

Lymph node= almost 100%
Lungs >90%
Spleen 75%
Liver 70%
Skin, eyes, skeletal muscle 50%
Bone marrow 20%
Salivary glands up to 50%

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15
Q

How does sarcoidosis present?

A

Acute arthralgia
Erythema nodosum
Bilateral hilar lymphadenopathy
Incidental abnormal CXR or CT scan - if asymptomatic
SOB
Cough

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16
Q

What is the treatment for sarcoidosis?

A

Corticosteroids

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17
Q

How do you diagnose sarcoidosis?

A

Clinical findings
Imaging findings
Serum Ca++ and ACE
Biopsy

18
Q

What is the interstitium?

A

The connective tissue potential space around the airways and vessels and the space between the basement membranes of the alveolar walls.

19
Q

What does the interstitium look like in normal cells?

A

Very thin (see sheet) to allow for gas exchange

20
Q

What happens in interstitial disease?

A

Interstitium is thickened by interstitial infiltrate making gas exchange harder/impossible

21
Q

What is hypersensitivity pneumonitis?

A

Patient is exposed to particular antigen to which they are sensitive to leading to hypersensitive reaction.

22
Q

How does a hypersensitive reaction come about?

A

Inhaled antigen that patient is exposed to
Coupled with immunogenetic make up

23
Q

What are some common antigens that cause hypersensitivity pneumonitis?

A

Thermophilic actinomycetes (in mouldy hay)
Bird/animal proteins
Fungi
Chemicals

24
Q

What is the acute presentation of hypersensitivity pneumonitis? (8)

A

Fever
Dry cough
Myalgia
Chills 4-9 hours after Ag exposure
Crackles
Tachypnoea
Wheeze
Precipitation antibody

25
Q

What is the chronic presentation of hypersensitivity pneumonitis? (7)

A

Insidious
Malaise
SOB
Cough
Low grade illness
Crackles
Wheeze

26
Q

What mediates the immune complex of hypersensitivity pneumonitis?

A

Combined Type 3 and 4 hypersensitivity reactions

27
Q

What is the histopathology of hypersensitivity pneumonitis?

A

Soft centriacinar epithelioid granulomata
Interstitial pneumonitis
Foamy histiocytes
Bronchiolitis obliterans

28
Q

What lung zones does hypersensitivity pneumonitis effect most?

A

Upper zones
(as this is where inhaled bacteria/antigen usually end up)

29
Q

What are the response from type 3 and 4 hypersensitivity reactions?

A

Type 3 = lymphocytes
Type 4 = granuloma

30
Q

How can idiopathic pulmonary fibrosis (IPF) be diagnosed?

A

Clinically - may be associated with radiological features

31
Q

What is the pathology of IPF?

A

Usual interstitial pneumonitis

32
Q

What is the aetiology of IPF? (5)

A

Connective tissue diseases
Drugs
Asbestos
Viruses
Idiopathic

33
Q

What are the treatment options for IPF?

A

No treatment
One way journey to resp failure - probability of developing end stage fibrosis is very high

34
Q

What is the histology of IPF? (5)

A

Patchy interstitial chronic inflammation
Type 2 pneumocyte hyperplasia
Smooth muscle and vascular proliferation
Evidence of old/recent injury
Proliferating fibroblastic foci

35
Q

What are the signs of old/recent injury and what does this show?

A

Temporal heterogeneity
Spatial heterogeneity

**disease is evolving: new - moderate - old - very old all seen within the same area

36
Q

What are the clinical signs of IPF? (5)

A

Dyspnoea
Cough
Basal crackles
Cyanosis
Clubbing

37
Q

What is the prognosis of IPF?

A

Not good - usually ends with end stage fibrosis

Progressive disease = most dead in 5 years

38
Q

What does a CXR show for IPF patients?

A

Basal/posterior
Diffuse infiltrates
Cysts
‘Ground glass@

39
Q

What are the investigation results for IPF?

A

Restrictive pulmonary function tests
Reduced gas transfer

40
Q

What is honeycombing of the lung?

A

End stage fibrotic areas of lung as a result of many different fibrosing conditions

41
Q

What does an end stage lung look like?

A

Shrunken - fibrotic so cant expand/stretch
Non functioning

Respiration (27 decks)

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