SFP: restrictive lung disease Flashcards

1
Q

Generally, describe restrictive airway diseases

A

The expansion of the lung is hindered either from something external to the lung or within the lung (often the latter)

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2
Q

What do restrictive lung diseases do to FVC, FEV1, and the FEV1/FVC ratio

A

Decrease FVC and FEV1 about equally, so the ratio is preserved

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3
Q

What is diffuse alveolar damage (DAD)

A

The pathological pattern caused by ARDS that is characterized by rapid onset of life-threatening respiratory failure

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4
Q

What do we see in the exudative (early) stage of DAD

A

Edema; Heavy and wet lungs with hyaline membranes

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5
Q

What tends to happen to alveoli in the exudative stage of DAD

A

They collapse

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6
Q

What do we see in the proliferative stage of DAD

A

Type II pneumocyte proliferation, interstitial fibroblasts, edema; grayish lungs

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7
Q

What do we see in the fibrotic stage of DAD

A

Fibrosis and honeycomb appearance; dense interstitial collagen

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8
Q

In what stage of DAD do macrophages accumulate

A

Proliferative

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9
Q

What is nonspecific interstitial pneumonia

A

A pneumonia usually found in younger patients that is associated with autoimmune diseases

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10
Q

What is the onset of non-specific interstitial pneumonia (NSIP)

A

Acute

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11
Q

What is the histology of NSIP

A

Lymphocytes, plasma cells, macrophages can be seen as well as foci of cryptogenic organizing pneumonia

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12
Q

What may cause NSIP

A

Drugs or autoimmune diseases

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13
Q

What is the prognosis of NSIP

A

It varies, but better than UIP

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14
Q

What is usual interstitial pneumonia

A

An idiopathic pneumonia in older patients that is difficult to treat; it’s the worst of the fibrosing interstitial pneumonias. It progresses over months to years.

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15
Q

What is seen on x-ray in UIP

A

Bilateral interstitial disease

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16
Q

Describe the histology of UIP

A

Temporal heterogeneity (fibrosis of different ages), honeycombing, minimal inflammation, fibroblast

17
Q

How do we treat UIP

A

We kinda don’t…many get transplants or die

18
Q

What is cryptogenic organizing pneumonia

A

Polyps of young connective tissue in airspaces or airways

19
Q

Why is cryptogenic organizing pneumonia (COP) considered interstitial?

A

It leads to interstitial inflammation

20
Q

What is seen microscopically in COP

A

Fibroblasts and collagen

21
Q

What is the pattern of desquamative interstitial pneumonia

A

Diffuse, bilateral, and in the lower lobes

22
Q

What are the smoking related interstitial lung diseases

A

Desquamative interstitial pneumonia and respiratory bronchiolitis

23
Q

Describe the histology of desquamative interstitial pneumonia (DIP)

A

Accumulation of macrophages and some lymphocytes and eosinophils. There is fine lamellar fibrosis

24
Q

How can we treat DIP

A

Steroids

25
Q

What is respiratory bronchiolitis interstitial lung disease

A

Often develops before DIP, has the same underlying cause, but is just less widespread.

26
Q

What are the two granulomatous interstitial lung diseases

A

Sarcoidosis and hypersensitivity pneumonia

27
Q

Describe hypersensitivity pneumonia

A

Looks like NSIP but with granulomas. It may lead to interstitial fibrosis and may have features of COP

28
Q

What are common clues for hypersensitivity pneumonia

A

Farmers, hot tubs, working with or having birds, humidifiers

29
Q

What types of hypersensitivity reactions are seen in hypersensitivity pneumonia

A

3 (serum specific antibodies) and 4 (granuloma)

30
Q

What is the key treatment for HP

A

Limiting exposure to antigen

31
Q

Describe sarcoidosis

A

Usually in younger patients and involved granulomas in the lungs that follow lymphatic distribution. May also be big lymph nodes in the mediastinum

32
Q

What is distinctive about sarcoid

A

It tends to be systemic!

33
Q

What are associated findings of sarcoid

A

Hypercalcemia from granulomas, elevated ACE, T cells that are reduced in blood and elevated in lungs