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Pathoma - Respiratory Tract Pathology > Restrictive Diseases > Flashcards

Restrictive Diseases Flashcards

1
Q

What are Restrictive Diseases characterized by?

A

Restricted filling of the lung (problem filling the lung)

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2
Q

What do Restrictive Diseases show on spirometry?

A

Dec. TLC
Dec. FEV1
Dec. (ALOT) FVC
FEV1:FVC ratio is Increased!

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3
Q

What is normal FEV1:FVC ratio? What is this ratio in restrictive diseases?

A

80%

Restrictive diseases are >80%

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4
Q

What most commonly causes Restrictive Diseases?

A

Interstitial diseases of he lung

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5
Q

What may also cause Restrictive Disease?

A

Chest wall abnormalities (such as massive obesity)

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6
Q

What is the pathogenesis behind interstitial diseases of the lung?

A
  1. Fibrosis of interstitium
  2. Thickening of the barrier
  3. Hard to open airway/sacs
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7
Q

What are the four main Restrictive Diseases?

A
  1. Idiopathic Pulmonary Fibrosis
  2. Pneumoconioses
  3. Sarcoidosis
  4. Hypersensitivity Pneumonitis
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8
Q

What is Idiopathic Pulmonary Fibrosis?

A

Fibrosis of lung interstitium

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9
Q

What is the etiology of Pulmonary Fibrosis?

A

Unknown

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10
Q

What is Idiopathic Pulmonary Fibrosis likely related to?

A

Cyclical lung injury

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11
Q

What induces fibrosis in Idiopathic Pulmonary Fibrosis?

A

TGF-beta from injured pneumocytes induces fibrosis.

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12
Q

What are secondary causes of Interstitial Fibrosis?

A
  • Drugs (Bleomycin and Amiodarone)

- Radiation therapy

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13
Q

What are two symptoms of Idiopathic Pulmonary Fibrosis?

A
  1. Progressive dyspnea

2. Cough

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14
Q

What is seen in Idiopathic Pulmonary Fibrosis on Lung CT?

A

Fibrosis

-> Initially seen in sub pleural patches but eventually results in diffuse fibrosis with end-stage ‘honeycomb’ lung

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15
Q

What is the treatment for Idiopathic Pulmonary Fibrosis?

A

Lung transplantation

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16
Q

What is Pneumoconiosis?

A

Interstitial fibrosis due to occupational lung exposure.

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17
Q

What is required for Pneumoconiosis to occur?

A

Chronic exposure to small particles that are fibrogenic

-Particles small enough that they can get into distal lung and induce fibrosis (through macrophages)

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18
Q

What causes the fibrosis in Pneumoconiosis?

A

Alveolar macrophages engulf foreign particles and induce fibrosis, trying to ‘wall off’ the foreign substance.

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19
Q

What exposure causes Coal Workers’ Pneumoconiosis?

A

Carbon dust; seen in coal miners

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20
Q

What does massive exposure to carbon dust (coal miners) lead to?

A

Diffuse fibrosis (‘black lung’) and shrunken lung.

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21
Q

What is Coal Workers’ Pneumoconiosis associated with?

A

Rheumatoid Arthritis (Caplan syndrome)

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22
Q

What is Anthracosis?

A

Collections of carbon-laden macrophages.

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23
Q

What causes Anthracosis?

A

Mild exposure to carbon (e.g. pollution)

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24
Q

Is Anthracosis clinically significant?

A

NO

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25
Q

What exposure causes Silicosis?

A

Silica

26
Q

What populations is Silicosis seen in?

A

Sandblasters and silica miners

27
Q

What is seen in the lungs of patients with Silicosis?

A

Fibrotic nodules in upper lobes of the lung

28
Q

What are patients with Silicosis at increased risk for?

A

TB

29
Q

What does silica impair in the lung?

A

Silica impairs phagolysosome formation by macrophages

30
Q

What exposure causes Berylliosis?

A

Beryllium

31
Q

In what populations is Berylliosis seen in?

A

Beryllium miners and workers in the aerospace industry (NASA)

32
Q

What pathologic findings are seen in Berylliosis?

A

Noncaseating granulomas in the lung, hilar lymph nodes and systemic organs.

33
Q

What are the pathological findings in Berylliosis similar to?

A

Sarcoidosis

34
Q

What are patients with Berylliosis at an increased risk for?

A

Lung cancer!

35
Q

What exposure causes Asbestosis?

A

Asbestos fibers

36
Q

What populations is Asbestosis seen in?

A

Construction workers, Plumbers, Shipyard workers

37
Q

What type of cancer is more common in Asbestos exposed individuals?

A

Lung carcinoma is more common than mesothelioma.

38
Q

What pathologic findings are associated with Asbestosis?

A

Fibrosis of lung and pleura (plaques) with increased risk of lung carcinoma and mesothelioma.

39
Q

What findings confirm exposure to asbestos in lung lesions?

A

Lesions containing long, golden-brown fibers with associated iron (asbestos bodies)

40
Q

What is Sarcoidosis?

A

Systemic disease characterized by noncaseating granulomas in multiple organs.

41
Q

What does Noncaseating mean?

A

All cells in granuloma are alive

42
Q

What is the most common site for noncaseating granulomas in Sarcoidosis?

A

Lungs & hilar lymph nodes

43
Q

What population is Sarcoidosis most commonly seen in?

A

African American Females

44
Q

What is the Etiology of Sarcoidosis?

A

Unknown

45
Q

What is the proposed pathogenesis of Sarcoidosis?

A

Likely due to CD4+ helper T-cell response to an unknown antigen.

46
Q

What do granulomas most commonly involve in Sarcoidosis? What do they lead to?

A

Hilar lymph nodes and lung.

Restrictive lung disease.

47
Q

What is often seen in the granulomas of Sarcoidosis?

A

Epitheliod histiocytes & giant cells

48
Q

What is often seen within the giant cells of Sarcoidosis granulomas?

A

Stellate inclusions (‘asteroid bodies’)

49
Q

What are other commonly involved tissues in Sarcoidosis?

A
  • Uvea (uveitis)
  • Skin (cutaneous nodules or erythema nodosum)
  • Salivary and lacrimal glands (mimics Sjogren syndrome)
  • Almost any tissue can be involved
50
Q

What are the common symptoms of Sarcoidosis?

A
  • Dyspnea

- Cough (most common presenting symptom)

51
Q

What do you see in the serum of Sarcoidosis?

A

Elevated ACE

52
Q

What does the noncaseating granuloma in Sarcoidosis cause in the serum?

A

Hypercalcemia

53
Q

How does noncaseating granulomas lead to Hypercalcemia?

A

1-alpha hydroxylase activity of epithelioid histiocytes converts vitamin D to its active form

54
Q

What is the treatment for Sarcoidosis?

A

Steroids. Often resolves spontaneously without treatment.

55
Q

What is Hypersensitivity Pneumonitis?

A

Granulomatous reaction to inhaled organic antigens

56
Q

What will you see on a slide of Hypersensitivity Pneumonitis?

A

Granulomas and Eosinophils

57
Q

What is an example of an organic antigen that causes Hypersensitivity Pneumonitis?

A

Pigeon Breeder’s Lung

58
Q

What does Hypersensitivity Pneumonitis present with?

A

-Fever
-Cough
-Dyspnea
. . .hours after exposure

59
Q

When does Hypersensitivity Pneumonitis resolve?

A

With removal of the exposure.

60
Q

What does chronic exposure to the inhaled organic antigen lead to in Hypersensitivity Pneumonitis?

A

Interstitial Fibrosis

Pathoma - Respiratory Tract Pathology (9 decks)

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