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Respiratory > EXAM #1: PATHOLOGY OF ILD > Flashcards

EXAM #1: PATHOLOGY OF ILD Flashcards

1
Q

What external pathologies can cause restrictive lung disease?

A

1) Deformed chest wall i.e. scoliosis or obesity

2) Pleural space filled

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2
Q

What is the defining feature of the Interstitial Lung Diseases (ILDs)?

A

Inflammation and fibrosis of pulmonary connective tissue/ peripheral interstitium between alveolar walls

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3
Q

What are the PFT findings in the ILDs?

A

1) Decreased TLC
2) Decreased FEV1
3) Dramatically decreased FVC

*Normal to increased FEV1/FVC ratio

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4
Q

How do the ILDs present?

A

1) Dyspnea
2) Hypoxia
3) End-inspiratory crackles

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5
Q

What is the pathognomonic description for the end-stage of the ILDs?

A

“Honeycomb lung”

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6
Q

Where is the pathologic disturbance occurring in ALI or ARDS?

A

Inflammatory damage at the alveolar-capillary interface

*Consequently, this is called non-cardiogenic pulmonary edema or DaNang Lung

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7
Q

What is the clinical features of ALI or ARDS?

A
  • Rapid onset dyspnea

- Refractory to oxygen therapy

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8
Q

What causes ALI or ARDS?

A

1) Sepsis
2) Infection
3) Shock
4) Trauma
5) Aspiration
6) Pancreatitis
7) DIC
8) Hypersensitivity
9) Drugs
10) Transfusion i.e. “TRALI”

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9
Q

What PaO2/FiO2 ratios define ALI and ARDS?

A 
ALI= less than 300 
ARDS= less than 200
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10
Q

What causes the damage seen in ARDS?

A

Neutrophil secreted proteases

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11
Q

What is the cause of neonatal ARDS?

A

Lack of surfactant

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12
Q

How is ARDS treated?

A

1) Address the underlying cause
2) NO to reduce resistance in pulmonary artery
3) PEEP

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13
Q

What are the complications of ARDS in those that survive?

A

Scarring and fibrosis leading to chronic ILD

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14
Q

What are the phases of ARDS recovery?

A

1) Exudative stage with hyaline membranes

2) Fibrotic stage

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15
Q

What is TRALI? What causes TRALI?

A
  • Transfusion-related ALI

- Caused by anti-HLA or anti-HNA antibodies

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16
Q

What is the treatment for TRALI?

A

Immediately stop tranfusion

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17
Q

Generally, what is the progression seen in diffuse interstitial disease?

A

1) Alveolitis
2) Leukocytes accumulate
3) End-stage fibrotic lung i.e. honeycomb lung

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18
Q

What cell plays a key role in mediating the pathology seen in diffuse interstitial disease?

A

M2 macrophage

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19
Q

What is the histological pattern associated with Idiopathic Pulmonary Fibrosis (IPF)?

A

UIP– “usual interstitial pneumonia”

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20
Q

What is the definitive treatment for IPF?

A

Lung transplant

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21
Q

What is the current theory of IPF pathogenesis?

A

“Repeated cycles” of epithelial activation/injury with an OVERWHELMING healing response

22
Q

What cytokine is implicated in the overwhelming healing response seen in IPF?

A

TGF-B1

23
Q

What is “temporal heterogeneity” referring to in IPF?

A
  • Cyclic healing causes collagen deposition
  • B/c it is cyclic, different stages of collagen will be present

Thus, temporal heterogeneity is referring to different stages of collagen deposition.

24
Q

What is essential to remember about IPF or seeing a UIP pattern in regards to treatment?

A

This requires lung transplant

*Other ILDs are responsive to steroids

25
Q

What collagen vascular disorders are associated with ILD?

A

1) RA
2) Scleroderma
3) SLE

26
Q

What is a pnemoconiosis?

A

Interstitial fibrosis caused by an occupational exposure

27
Q

What is a key factor in pneumoconiosis?

A

Capacity of the inhaled particle to stimulate alveolar macrophages that initiate inflammation and fibrosis

28
Q

What is Coal Worker’s Pneumonconiosis (CWP)?

A

Pneumoconiosis caused by inhalation of carbon dust

29
Q

What are the three degrees of CWP?

A

1) Asymptomatic anthracosis= no sx.
2) Simple CWP= minimal dysfunction
3) Complicated CWP= severe degree of dysfunction

30
Q

Is CWP associated with an increased risk for cancer or TB?

A

NO

31
Q

What is PMF?

A

Pulmonary Massive Fibrosis

  • severe fibrosing reaction that can complication ANY pneumoconiosis
  • massive amounts of carbon dust= major culprit
32
Q

What is Caplan Syndrome?

A

CWP + RA

33
Q

What is Silicosis?

A

Pneumoniosis associated with scilica i.e. sandblasting, masonry, and fine stone particles

34
Q

What particle is most fibrogenic and thus most dangerous in terms of Scilicosis progression?

A

Quartz

35
Q

What is unique about the risk profile associated with Scilicosis?

A

This is the ONLY pneumoconiosis that increases the risk of cancer AND TB

*Scilica impairs the formation of the phagolysosome, which is what increases the risk for TB

36
Q

Histologically, what is pathognomonic for Scilicosis?

A

“Whorls of collagen” in the fibrosis seen with Scilicosis

37
Q

What test is a good test to confirm Scilicosis?

A

Polarized light–the rock particles will light up

38
Q

What are the two types of particles seen in Asbestosis?

A 
Serpentines= more common, less pathogenic 
Amphiboles= less common, more pathogenic
39
Q

What types of occupations were historically most exposed to Asbestos?

A

1) Construction workers
2) Plumbers
3) Shipyard workers

40
Q

What type of cancers are associated with asbestos exposure?

A

1) Lung carcinoma
2) Mesothelioma

Mesothelioma occurs later than the more generic lung carcinomas; thus, lung carcinoma is MORE COMMON

41
Q

What is an asbestos body?

A

Dumbbell shaped golden-brown fibers coated with iron

42
Q

Describe the pathogenesis of the pleural plaques seen in asbestosis.

A

Larger asbestos fibers penetrate the alveolar sacs and pleura, forming fibrotic plaques in the pleura

43
Q

How much does asbestos increase the risk of lung carcinoma? How if the individual smokes in addition to the exposure?

A
  • 5x increase if just asbestos

- 55x increase if patient smokes too

44
Q

List the drugs that classically cause drug-induced pulmonary disease. What conditions are caused by these drugs?

A

1) Bleomycin= pneumonitis and fibrosis
2) Methotrexate= hypersensitivity pneumonitis
3) Amiodarone= pneumonitis and fibrosis
4) Nitrofurantoin= hypersensitivity pneumonitis
5) Aspirin= bronchospasm
6) Beta-antagonists= bronchospasm

45
Q

What are the two types of radiation induced lung disease?

A
  • Acute= occurs 1-6 months post treatment

- Chronic= failure of acute disease to resolve, causing pneumonitis and pulmonary fibrosis

46
Q

What medication is indicated for the treatment of radiation-induced lung disease?

A

Steroids

47
Q

What is the mesothelioma?

A

Cancer of the pleura associated with asbestos exposure

48
Q

What is Berylliosis?

A

Pneumoconiosis caused by exposure to Beryllium

49
Q

What types of workers are exposed to Beryllium?

A

Miners and aerospace workers

50
Q

What does Berylliosis cause?

A

Non-caseating granulomas of the lung, hilar lymph nodes, and systemic organs

*Note that this strongly resembles SARCOIDOSIS

51
Q

What disease do you want to be sure not to confuse Berylliosis with?

A

Sarcoidosis

Respiratory (35 decks)

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