Interstitial Lung Disease 2 Flashcards

1
Q
A
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2
Q

What structure is affected in interstitial lung disease?

A

Interstitial lung disease (ILD)

aka Diffuse Parenchymal Lung Disease (DPLD)

• a group of diseases affecting the interstitium (tissue and space around the alveolar sacs of the

lung)

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3
Q

Pathophysiology of interstitial Lung disease

A
  • injury to the lung —> abnormal healing response —> incorrect repair process —> tissue around #
  • the alveoli become scarred and thickened
  • scarred and thickened tissue —> more difficult for the oxygen to pass into blood stream
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4
Q

Symptoms of Interstitial Lung Disease?

A

SOB on expiration, non-productive paroxysmal cough, restrictive pulmonary, spirometry, abnormal breath sounds, decreased tolerance to exercise

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5
Q

What can be seen on examination of a patient with Interstitial Pulmonary Disease?

A

O/E: inspiratory crackles, cyanosis and finger clubbing (later stage)

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6
Q

Management of Interstitial Pulmonary Disease

A
  • if specific environmental factor is a contributor —> a person should avoid that environment
  • if a drug is suspected to be a cause —> discontinuation of a drug
  • many cases of an unknown cause or connective tissue disorder —> corticosteroid treatment

(e.g. prednisolone) ; some respond to immunosuppressants

  • supportive treatment with oxygen
  • Pulmonary rehabilitation
  • Lung transplant - if other forms of treatments fail and if there are no other contradictions
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7
Q

Examples of substances potentially causing Idiopathic Lung Disease re to occupation

A

asbestosis, berylliosis, silicosis, cotton worker’s lung

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8
Q

Examples of substances potentially causing Idiopathic Lung Disease re to drugs

A

nitrofurantoin, bleomycin

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9
Q

Examples of substances potentially causing Idiopathic Lung Disease re to allergy

A

extrinsic allergic alveolitis

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10
Q

Examples of substances potentially causing Idiopathic Lung Disease re to infections

A

TB, fungi, viral

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11
Q

What other systemic diseases ILD can be associated with?

A
  • Sarcoidosis
  • Rheumatoid arthritis
  • Systemic sclerosis, connective tissue disease, Sjogren’s syndrome
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12
Q

Pathophysiology of Idiopathic Pulmonary Fibrosis

A
  • idiopathic trigger of the process
  • Fibrosis occurs usually after tissue damage —> ongoing repair process —> excess collagen/ scar tissue in the interstitial space of the lungs —> chronic process —> progressive loss of lung tissue
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13
Q

Risk factors for Idiopathic Pulmonary Fibrosis (3)

A

old age, being male, tobacco smoking

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14
Q

What is the location of Idiopathic Pulmonary Fibrosis and cells involved

A

Interstitial tissue (contains macrophages and fibroblasts)

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15
Q

What happens (pathology) in Idiopathic Pulmonary Fibrosis?

A
  • Damage to epithelium —> type 2 pneumocyte over- proliferate (during the repair process)—> too many myofibroblasts —> too much collagen
  • Myofibroblasts do not undergo apoptosis —> more and more collagen is produced —> collagen accumulates —> interstitial layer between alveoli and capillary forms —> problems with ventilation and oxygenation
  • Excess collagen (also causes)—> lungs becoming stiff —> hard for air to flow in and out —>

restricted lung expansion (due to fibrosis in interstitium)

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16
Q

What results (3) would be seen on spirometry in Idiopathic Pulmonary Fibrosis

A

A) decrease in TLC (max amount of air lungs
can hold)

B) decrease in FVC (amount of the air exhaled after a deep breath)

C) decrease in FEV1 (air exhaled with max effort in first second)

17
Q

Why in FEV1/|FVC is over 0.7 or above in restrictive disease?

A

Decreased FVC and FEV1’ but restrictive still is >/= 0.7 of predictive value as it is a ratio o FVC/FEV1 so if both components of the fraction are decreased; overall ratio will not change

18
Q

A characteristic feature of X-ray/ CT in Idiopathic Pulmonary Fibrosis?

A

Excess collagen —> loss of alveoli —> fluid- filled

spaces = cysts created = ‘HONEYCOMBING’ pattern

20
Q

Symptoms of Idiopathic Pulmonary Fibrosis?

A
  • progressive process —> symptoms will worse over time
  • problems with oxygenation and ventilation —> SOB, coughing, cynosis, digital clubbing
  • over time —> respiratory failure (as lung loses more and more functional tissue)
21
Q

Diagnosis of Idiopathic Pulmonary Disease?

A

A) Chest imaging: CT scan - honeycomb appearance and thickening of interstitial walls

B) Spirometry - decrease in: TLC, FVC and FEV1 (characteristics of restrictive lung diseases)

22
Q

Management of Idiopathic Pulmonary FIbrosis

A

A) Supplemental oxygen

B) Antifibrotic medication - slow the progression but does not stop disease

C) Lung transplant - the only available treatment for severe cases

23
Q
A
24
Q

What’s Extrinsic Allergic Alveolitis?

A

Inflammation of the alveoli within the lung caused by hypersensitivity to inhaled organic dusts

25
Q

Cause of Extrinsic Allergic Alveolitis

A

Cause: provoking agents

  • inhalation of fine particles
  • oral drugs

Example:

• Bird fancier’s lung:

antigen: avian (relating to birds) proteins
exposure: feathers or bird droppings

• Farmer’s lung:

26
Q

What are the characteristic features in lung appearance in Extrinsic allergic alveolitis:

A. Acute

B. Subacute

C. Chronic

A

Acute: lungs are infiltrated with neutrophils and mononuclear cells

Subacute: lungs are infiltrated with lymphocytes

Chronic: Non-caseating epithelioid granulomas develop + progressive lung fibrosis

27
Q

Mechanism of Extrinsic Allergic alveolitis

A
  • Inhalation of antigen —> hypersensitivity reaction (type III and/or type IV) —> exaggerated immune response

*Hypersensitivity type response depends on cause -> can occur in both forms