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Medicine - Year 1 - Term 2 > W4 - Pathology of Restrictive Lung Diseases > Flashcards

W4 - Pathology of Restrictive Lung Diseases Flashcards

1
Q

The interstitium of the lung is comprised of connective tissue space around what 3 things?

A

Airways
Vessels
Basement membranes of alveolar walls

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2
Q

In normal alveolar walls, what 2 things are in direct contact with each other?

A

Alveolar epithelial (pneumocyte)

Interstitial capillary endothelial cell basement membranes

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3
Q

Histologically, how do healthy alveoli vs alveoli in restrictive lung disease appear? What’s the physiological relevance of this difference?

A

In healthy tissue, alveolar walls are 2 cells thick.

In restrictive lung disease, they are thickened by interstitial infiltrate

The thickening means gas exchange is much more difficult

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4
Q

What does FEV1, FVC and the ratio look like in restrictive lung disease?

A

FEV1 reduced
FVC reduced
FEV1/FVC ratio normal

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5
Q

How does restrictive lung disease affect compliance and why?

A

Reduced lung compliance as fibrous material prevents lungs stretching

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6
Q

Reduced gas transfer from restrictive lung disease is an abnormality of what?

A

Diffusion

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7
Q

Describe ventilation/perfusion balance in restrictive lung disease

A

Imbalance when small airways are affected by pathology

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8
Q

There’s more than 100 restrictive lung diseases, so it’s tricky to generalise. But what 4 symptoms are typical of restrictive lung disease?

A

Incidentally discovered on CXR or CT (asymptomatic)

Dyspnoea, initially SOB on exertion, then at rest as disease progresses

Type 1 Resp Failure

Heart failure, caused by cor pulmonale

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9
Q

An acute response to interstitial lung injury is called what?

A

Diffuse Alveolar Damage (DAD)

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10
Q

Name 3 types of chronic responses to interstitial lung disease

A

Usual Interstitial Pneumonitis (UIP)

Granulomatous Responses

Other Patterns

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11
Q

Chronic responses to interstitial lung disease lead to what?

A

Fibrosis or end-stage honeycomb lung

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12
Q

Define the natural history of restrictive lung disease generally, IPF and sarcoidosis

A

Generally variable

IPF - 3-6 year survival

Sarcoidosis - relatively benign self-limiting course. 20% develop pulmonary fibrosis

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13
Q

Name 3 examples of occupational lung diseases

A

Coal Worker’s pneumoconiosis
Silicosis
Asbestosis

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14
Q

Name 2 granulomatous diseases of restrictive lung disease

A

Sarcoidosis

Hypersensitivity pnemonitis

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15
Q

Give 3 examples of types of hypersensitivity pneumonitis

A

Farmer’s Lung
Bird Fancier’s Lung
Bark Stripper’s Lung

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16
Q

Give an example of idiopathic pulmonary fibrosis

A

Usual Interstitial pneumonia

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17
Q

Name 8 causes of DADS

A

Major trauma
Chemical injury/toxic inhalation
Circulatory shock, cardiac failure, blood loss
Drugs
Infection inc viruses
Autoimmune disease
Radiation
Idiopathic

18
Q

What is DADS?

A

Diffuse Alveolar Damage Syndrome - acute inflammatory response in lung interstitium

19
Q

Name 6 histological features of DADS

A

Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial or fibroblast proliferation
Scarring in interstitium and airspaces (fatal)

20
Q

What 2 things peak in the exudative stage of DADS, then which 2 things rise in the proliferative stage?

A

Exudative - oedema then hyaline membranes

Proliferative - interstitial inflammation and interstitial fibrosis

21
Q

What is sarcoidosis in 3 words? Where is it more common? Who does it mainly affect in terms of age and gender?

A

Multisystem granulomatous disorder
More common in temporate climates
Mainly young adults, F>M

22
Q

Name 4 histopathological features of sarcoidosis

A

Epithelioid and giant cell granulomas
Unlikely to have necrosis
Little lymphoid infiltrate
Variable associated fibrosis

23
Q

In what 3 ways does sarcoidosis present?

A

Young adult with acute arthralgia, erythema nodosum or bilateral hilar lymphadenopathy

Asymptomatic with incidental abnormal CXR/CT

SOB, cough, abnormal CXR

24
Q

In what 4 ways is sarcoidosis diagnosed?

A

Clinical findings
Imaging findings
Serum Ca++ and ACE
Biopsy or EBUS cytology

25
Q

How is sarcoidosis treated?

A

Corticosteroids

26
Q

How do you distinguish between sarcoidosis and hypersensitivity pneumonitis in histology?

A

Sarcoidosis - distinct granulomas

HP - thickening of alveolar walls and softer, more diffuse granulomas

27
Q

Define hypersensitivity pneumonitis

A

Granulomatous condition from hypersensitivity reaction to exogenous inhaled materials

28
Q

Name 5 antigens that can lead to HP

A

Thermophilic actinomycetes (micropolyspora faeni, thermoactinomyces vulgaris)
Bird/animal proteins
Fungi (aspergillus)
Chemicals
Other

29
Q

In what 8 ways does HP present if acute?

A

Fever, dry cough, myalgia, chills 4-9 hrs after Ag exposure, crackles, tachyopnoea, wheeze, precipitating antibody

30
Q

In what 6 ways does HP present if chronic?

A

Insidious
Malaise
SOB
Cough
Low grade illness
Crackles and some wheeze

31
Q

Name 6 histopathology features of HP

A
  • Immune complex mediated combined Type III and IV Hypersensitivity reaction
    • Soft centriacinar epithelioid granulomata
    • Interstitaial pneumonitis
    • Foamy histiocytes
    • Bronchiolitis obliterans
      Upper zone disease
32
Q

Name 3 types of HP

A

Farmer’s Lung
Bird Fancier’s Lung
Bark Stripper’s Lung

33
Q

What 2 other diseases may UIP present in?

A

Scleroderma
Rheumatoid disease

34
Q

What 5 things may cause UIP?

A

Connective tissue disease
Drug reaction
Post infection
Industrial exposure (asbestos)
Other

35
Q

What age and sex is UIP more prevalent in?

A

> 50, M>F

36
Q

In what 5 ways does UIP present?

A

Dyspnoea
Cough
Basal crackles
Cyanosis
Clubbing

37
Q

What 4 things does CXR of UIP show?

A

Basal/posterior
Diffuse infiltrates
Cysts
“ground glass”

38
Q

Name 5 histopathology features of UIP

A
  • Patchy interstitial chronic inflammation
    • Type II pneumocyte hyperplasia
    • Smooth muscle and vascular proliferation
    • Evidence of old and recent injury (temporal heterogeneity or spatial heterogeneity)
    • Proliferating fibroblastic foci
39
Q

What does UIP stand for?

A

Usual Interstitial Pneumonitis

40
Q

What 4 treatments can be used for UIP and what is their effectiveness? What is the prognosis for UIP?

A

Steroids or anti-inflammatories (don’t work very well)
Anti-angiogenic agents (minor improvement)
Transpulmonary transplantation

5 year survival

41
Q

What is the endstage of restrictive lung diseases? What causes it and what do the lungs look like?

A

Endstage lung fibrosis/honeycomb lung

Here, progressive fibrosis damages more and more of the lungs. The lungs try to repair themselves but fail, leading to shrunken lung with non-smooth surface.

42
Q
A

Medicine - Year 1 - Term 2 (13 decks)

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