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Respiratory System DO > Pathology of Restrictive Lung Disease > Flashcards

Pathology of Restrictive Lung Disease Flashcards

1
Q

What is the interstitium of the lung

A

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

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2
Q

What is in direct contact in normal alveolar walls

A

Most of the alveolar epithelial (pneumocyte) and interstitial capillary endothelial cell basement membranes

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3
Q

What can enter the alveolar wall space in restrictive lung diseases

A

Inflammatory cells, fibroblasts and collagen

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4
Q

What does the presence of inflammatory cells, fibroblasts and collagen in the alveolar wall space cause

A

Increased diffusion distance of air

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5
Q

What do inflammatory cells, fibroblasts and collagen in the alveolar wall develop into and what does this cause

A

Fibrosis causing the lungs to become stiff due to an increase in the amount of elastic tissue and reduction in stretch ability due to the excess components present in the alveoli wall

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6
Q

What are the 4 main features of restrictive lung diseases

A

Reduced lung compliance (stiff lungs)
Low FEV1 and FVC but FEV1/FVC ratio is normal
Reduced gas transfer (Tco or Kco) – diffusion abnormality
Ventilation/perfusion imbalance – when small airways are affected by pathology

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7
Q

Why is there a reduction in FEV1 and FVC

A

Because there is a lung volume reduction

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8
Q

Do restrictive lung diseases have air flow limitation

A

No

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9
Q

How can gas transfer be measured

A

By the movement of CO that is breathed in in a single breath and can be measured in the blood to calculate the transfer of CO from air to the blood

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10
Q

How much V/Q imbalance is seen in some restrictive lung diseases

A

Small amount

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11
Q

How are diffuse lung diseases normally found

A

Abnormal CXR

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12
Q

How will diffuse lung diseases present

A

Dyspnoea

Type 1 respiratory failure leading to heart failure

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13
Q

Is the dyspnoea found in diffuse lung disease upon exertion or at rest

A

Upon exertion but as the disease progresses it will be present at rest also

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14
Q

How many ribs will be seen on a chest X-Ray of interstitial lung disease

A

Less than 10 with more markings between the ribs

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15
Q

Where is the site of inflammation in interstitial lung disease

A

Parenchymal

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16
Q

What type of inflammation can parenchymal lung injury cause

A

Acute or Chronic

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17
Q

How many pathways does chronic inflammation have. Name them

A

3
Usual interstitial pneumonitis (UIP)
Granulomatous responses
Other patterns

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18
Q

What do all of the chronic inflammation pathways lead to

A

Fibrosis or End-stage Honeycomb Lung

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19
Q

What can end stage pulmonary fibrosis lead to

A

Respiratory failure

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20
Q

Which process is most likely to lead to end stage pulmonary fibrosis

A

UIP

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21
Q

Which process is least likely to lead to end stage pulmonary fibrosis

A

Granulomatous response

22
Q

Give an example of acute inflammation due to parenchymal lung injury

A

Diffuse Alveolar Damage DAD

23
Q

What is DAD associated with

A 
Major trauma
Chemical injury / toxic inhalation
Circulatory shock
Drugs 
Infection
Auto(immune) disease
Radiation
But it can be idiopathic
24
Q

What does DAD cause

A

A large leak in the capillaries of the alveolar walls

The damage to the capillaries is most likely mediated by neutrophil polymorphs

25
Q

What do some patients with DAD present with

A

Severe respiratory failure and admitted straight to ITU

Over 50%

26
Q

Where is fibrosis found in DAD

A

Interstitial space and alveoli walls

27
Q

What are the histological features of DADS

A 
Protein rich oedema
Fibrin
Hyaline membranes
Denuded basement membranes
Epithelial proliferation
Fibroblast proliferation
Scarring - interstitium and airspaces
28
Q

Why is proliferation seen in DADS

A

To try and repair the damage caused

29
Q

What is the fluid and protein found in DADS transformed to

A

Fibrous tissue which forms a solid lung

30
Q

What can a granulomatous response cause

A

Sarcoidosis

Hypersensitivity pneumonitis

31
Q

What is sarcoidosis

A

A multisystem granulomatous disorder of unknown atiology

32
Q

What are the characteristic features of sarcoidosis in histopathology

A

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

33
Q

Can sarcoidosis lead to fibrosis

A

Yes but unlikely to lead to end-stage fibrosis

34
Q

Who is commonly affected by sarcoidosis

A

Young adults

Females more than males

35
Q

What is the incidence of sarcoidosis in the UK

A

3-4/100,000

36
Q

What is the incidence of sarcoidosis in Afro-Americans the USA

A

20/100,000

37
Q

What will a young adult with sarcoidosis show

A

Acute arthralgia
Erythema nodosum
Bilateral hilar lymphadenopathy

38
Q

Can sarcoidosis be discovered when there are no symptoms

A

Yes in an incidental abnormal CXR

May resolve, persist or progress

39
Q

What else can patients with sarcoidosis present with

A

SOB
Cough
Abnormal CXR
May resolve, persist or progress

40
Q

Do young patients presenting with acute arthralgia, erythema nodosum and bilateral hilar lymphadenopathy require treatment

A

No

It is a self-limiting condition which resolves within 2 years

41
Q

How is sarcoidosis treated with

A

Corticosteroids

42
Q

What 4 things can be used to diagnose sarcoidosis

A

Clinical findings
Imaging findings
Elevated serum Ca++ and ACE
Biopsy

43
Q

What is hypersensitivity pneumonitis

A

A reaction to inhaled antigens (normally of organic origin)

44
Q

Name some type of antigens which can cause hypersensitivity pneumonitis

A 
Thermophilic actinomycetes (e.g. Micropolyspora faeni, Thermoactinomyces vulgaris)
Bird/Animal proteins - faeces, bloom
Fungi  - Aspergillus spp
Chemicals
Others
45
Q

What do you see in the acute presentation of hypersensitivity pneumonitis

A

Fever, dry cough, myalgia,
Chills 4-9 hours after antigen exposure
Crackles, tachyopnoea, wheeze
Precipitating antibody

46
Q

What do you see in the chronic presentation of hypersensitivity pneumonitis

A

Insidious
Malaise, SOB, cough
Low grade illness
Crackles and some wheeze

47
Q

What is one of the most important aspects when seeing a patient with suspected hypersensitivity pneumonitis

A

History

48
Q

Is acute or chronic hypersensitivity pneumonitis seen more often

A

Chronic

49
Q

What type of hypersensitivity reaction is hypersensitivity pneumonitis

A

Immune complex mediated

Type III and type IV hypersensitivity reaction

50
Q

What can hypersensitivity pneumonitis lead to

A

Soft centriacinar epithelioid granulomata
Interstitial pneumonitis
Foamy histiocytes
Bronchiolitis obliterans

51
Q

Which part of the lung does hypersensitivity pneumonitis affect

A

Upper Zone

Respiratory System DO (33 decks)

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