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Pulm > Lecture 8: Interstitial Lung Disease > Flashcards

Lecture 8: Interstitial Lung Disease Flashcards

1
Q

What is the interstitium of the lungs?

A

Region between alveolar wall and pulmonary capillary epithelial cells.

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2
Q

What characterizies Interstitial Lung Disease (ILD)?

A
  • Damaged alveoli and surrounding tissue
  • DOE
  • Persistent dry cough
  • Late inspiratory rales on PE (forced opening of alveoli)
  • CXR: septal thickening and reticulonodular damage.
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3
Q

What structures are affected by ILDs?

A
  • Alveolar epithelium
  • Pulmonary capillary endothelium
  • Alveolar basement membrane
  • Perivascular tissues
  • Perilymphatic tissues

The tissue and airspace around the air sacs of the lungs.

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4
Q

What are the primary causes of ILDs?

A
  • Idiopathic pulmonary fibrosis (MC)
  • Occupational and environmental
  • Sarcoidosis
  • CT diseases
  • Drug and radiation
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5
Q

Describe the pathophysiology of ILDs.

A
  • Injury to alveolar epithelium or capillary endothelial cells.
  • Leads to progressive, irreversible scarring and stiffness of lung parenchyma.
  • Poor O2 exchange.
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6
Q

What is the proposed mechanism of ILD?

A

Repetitive and/or excessive injury, followed by dysregulation of tissue repair

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7
Q

What are the two pathophysiologic presentations for ILD?

A
  1. Granulomatous lung disease (T cells, macrophages, and epithelioid cells)
  2. Inflammation and fibrosis (fibrotic alveoli)
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8
Q

When do ILDs tend to appear?

A

Ages 20-40, with an insidious onset.

>60 would be idiopathic pulmonary fibrosis

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9
Q

What is the general presentation of an ILD?

A
  • Fatigue
  • Weight loss
  • Dyspnea
  • Cough (dry)

Extrapulmonary symptoms can be seen if they have a CT disorder.

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10
Q

What would be expected on a respiratory exam for ILD?

A
  • Tachypnea
  • Late inspiratory rales (bibasilar, posterior axilla)
  • Rhonchi (associated bronchiolitis)
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11
Q

What would be expected on a CXR for ILD?

A
  • Bibasilar reticular or reticulonodular with honeycombing in late stage.
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12
Q

What is an alternative to CXR that helps narrow ILD diagnosis?

A

High-res CT (HRCT)

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13
Q

How do most ILDs present on PFT?

A

Restrictive, with TLC reduction first and then FEV1/FVC reductions.

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14
Q

Which ILD could present as an obstructive pattern on PFT?

A

Sarcoidosis

50%

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15
Q

Which test would be used to confirm results of decreased pulse oximetry for an ILD patient?

A

ABG.

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16
Q

At what point does medicare reimburse for oxygen supplementation?

A
  • 88% or less SaO2 on 6MWT.
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17
Q

What is the function of bronchoalveolar lavage?

A

Obtain samples of cells and fluid for assessment of cell count, cultures, and cystologic analysis.

Usually non-specific for ILD.

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18
Q

When is lung biopsy indicated for in ILD?

A

Last resort to confirm diagnosis or stage disease.

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19
Q

What is the goal SaO2 for oxygen supplementation in ILD and when is it indicated?

A
  • Indication: hypoxemia <= 88%
  • Goal: 90-92%

Documentation: 2L O2 via NC

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20
Q

What is the first-line pharmacotherapy for ILD?

A

Glucocorticoids, usually prednisone.

Re eval after 4-12 wks.

Starting dose: 0.5-1mg/kg PO daily.

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21
Q

If an ILD patient has no improvement on glucocorticoids, what should be added?

A

Add immunosuppressant, such as cyclophosphamide, azathioprine, or mycophenolate mofetil (cellcept)

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22
Q

What patient education should be provided regarding ILDs?

A
  • Fibrosis is an irreversible condition
  • Compliance is vital to slowing disease progression.
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23
Q

What is the theory behind idiopathic pulmonary fibrosis? (IPF)

A
  • Homeostasis of alveolar epithelial cells is disrupted, leading to abnormal cell repair and fibrosis.
  • Excessive production and dysregulation of myofibroblasts occur.
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24
Q

What are the clinical findings suggestive of IPF?

A
  • Gradual onset of exertional dyspnea with dry cough.
  • 55-60 y/o males
  • Fine inspiratory crackles/rales w or w/o digital clubbing.
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25
Q

What should a PFT show for IPF?

A
  • Restrictive lung pattern
  • Reduced DLco
  • Hypoxemia that worsens on exertion.
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26
Q

What should a HRCT of IPF show?

A
  • Bibasilar, reticular opacities
  • Traction bronchiectasis
  • Honeycombing
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27
Q

What histiologic findings are characteristic of IPF?

A

Alternating areas of healthy, inflammation, fibrosis, and honeycomb change.

Fibrosis is dominant over inflammation

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28
Q

What are the primary treatment agents for IPF?

A
  • Nintedanib: Tyrosine kinase inhibitor
  • Pirfenidone: anti-inflammatory agent; antifibrotic

Prevents further scarring. Efficacy similar.

Caution in liver injury! Need LFTs

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29
Q

What should be done in terms of management for IPF to help treat it besides pharmacotherapy?

A
  • Early referral for lung transplant.
  • Encourage clinical trial participation

Disease is irreversible!

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30
Q

What drug class is being researched for IPF?

A

Phosphodiesterase 4B inhibitors

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31
Q

What is sarcoidosis?

A
  • Inflammatory disease of unknown etiology.
  • Noncaseating (non-necrotizing) granulomas involving 2+ organ systems.
  • MC organ: lungs.
  • 2nd MC: Skin and eyes
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32
Q

What demographic is the most susceptible to sarcoidosis?

A

Young, female, African-American

Will present more acute and severely.

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33
Q

How do Northern europeans present with sarcoidosis?

A

Much more milder and chronic.

34
Q

What are the primary clinical findings in sarcoidosis?

A
  • Dyspnea & cough
  • Insidious fatigue, fevers, night sweats, wt loss
  • Lung exam will most likely be normal unless advanced.
35
Q

What are the skin clinical findings associated with sarcoidosis?

A
  • Erythema nodosum (LE panniculitis)
  • Lupus Pernio (violaceous rash on cheeks or nose)
  • Maculopapular lesions (MC in chronic sarcoidosis)
36
Q

What are the ocular clinical findings associated with sarcoidosis?

A
  • Anterior/posterior granulomatous uveitis
  • Anterior: insidious onset, pain, slight photophobia, blurred vision.
  • Posterior: Painless, floaters, loss of visual field, scotomas, decreased VA
  • Conjunctival lesions and scleral plaques
37
Q

What abnormal lab findings are associated with sarcoidosis?

A
  • Hypercalcemia (granulomas make 1,25-dihydroxyvitamin D)
  • ESR (general inflammation)
  • ACE (granulomas secrete ACE)

ACE elevation is non-specific and non-sensitive.

ACEI use can produce low ACE levels even in the presence of sarcoidosis.

Lungs naturally create ACE!

38
Q

What is the most sensitive pulmonary test for sarcoidosis?

A

DLco to check for diffusing capacity.

39
Q

Describe the staging of sarcoidosis based on CXR presentation.

A
  1. Stage 1 is isolated hilar adenopathy
  2. Stage 2 is combination of adenopathy + hilar infiltrates
  3. Stage 3 is infiltrates alone
  4. Stage 4 consists of fibrosis
40
Q

What specific part of the lung is used to support the diagnosis of sarcoidosis?

A
  • Adenopathy size.
  • > 2 cm in the short axis supports Dx of sarcoidosis over other ILDs.

Requires HRCT and only used to compare vs other ILDs.

41
Q

What is the confirmatory histiologic finding for sarcoidosis?

A

Non-caseating granuloma.

Histology is the most confirmatory test for sarcoidosis.

42
Q

When is thoracoscopy used over transbronchial biopsy?

A

Atypical imaging or less invasive testing was indeterminate.

43
Q

What might a broncheoalveolar lavage show for sarcoidosis?

A
  • Increased lymphocytes
  • High CD4/CD8 ratio

Not confirmatory

44
Q

What is the only finding in sarcoidosis that does not require treatment?

A

Erythema nodosum.

Very rare to be an isolated finding.

45
Q

For acute sarcoidosis, what is the treatment?

Acute = < 2 years for sarcoidosis.

A
  • None-minimal symptoms: observe
  • Single organ: systemic therapy unless anterior eye only.
  • Multi-organ: Systemic glucocorticoids +/- immunomodulators

Anterior eye is only topical corticosteroids.

46
Q

What is the treatment for chronic sarcoidosis?

Chronic is 2-5 years without symptom resolution.

A
  • If tolerable of glucocorticoids: stay
  • If intolerable: use immunomodulators
  • If not effective: taper off +/- immunosuppressants
47
Q

What is the only efficacious treatment for advanced pulmonary fibrosis?

A

Lung transplant

48
Q

What is the primary contraindication to ophthalmic corticosteroids?

A

Dendritic lesions

49
Q

What are the 3 primary substances that cause pneumoconiosis?

A
  • Coal mining
  • Silicosis
  • Asbestosis
50
Q

How can PFTs vary for occupational/environmental lung disease?

A
  • Uncomplicated: restrictive with decreased DLco
  • Complicated: obstructive with decreased DLco
51
Q

Define pneumoconiosis.

A

Chronic, fibrotic lung disease caused by the inhalation of inorganic dusts.

52
Q

What is the treatment for pneumoconiosis?

A

Supportive. No medication has proven efficacy.

53
Q

What is the pathophysiology of black lung?

A

Alveolar macrophages ingest inhaled coal dust leading to the formation of coal macules 2-5 mm in diameter.

Macrophages then die in the lungs, releasing all the coal they ingested.

54
Q

What is the simple presentation of black lung?

A
  • Asymptomatic
  • Minimal change on PFTs
  • Small, rounded opacities on CXR.
55
Q

What is the complicated presentation of black lung?

A
  • Symptomatic
  • Diminished function on PFT
  • Nodules >= 1cm in upper half of lungs.
56
Q

Define silicosis.

A

Fibronodular lung disease caused by inhalation of dust containing crystalline silica.

57
Q

How does acute silicosis present?

A
  • Occurs in heavily exposed environments.
  • Few weeks to years after exposure
  • Cough, SOB, pleuritic pain, wt loss, faitgue
58
Q

How does chronic silicosis present?

A
  • Simple: asymptomatic or DOE and cough w/ sputum.
  • Complicated: Cough, SOB, appetite loss, wt loss, malaise/faitgue

Takes 15-20 years to develop changes.

59
Q

What abnormal breath sounds are expected in silicosis lungs?

A

Rales

60
Q

What is the expected finding on CXR/HRCT for simple silicosis?

A

Multiple, small (< 1cm) nodules scattered diffusely but more prominent in upper lung fields.

61
Q

What is the expected finding on CXR/HRCT for complicated silicosis?

A

Bilateral upper lobe masses due to coalescence of nodules

62
Q

What is the secondary concern for people with silicosis specifically?

A

Increased risk for pulmonary TB.

63
Q

What is the pathophysiology of asbestosis?

A
  • Asbestos fibers are inhaled, becoming ingested by macrophages.
  • Results in inflammatory response, fibroblast proliferation, and chronic scarring.

Asbestos stabs the macrophages

64
Q

How does asbestosis present?

A
  • Takes 20 years to appear usually.
  • DOE is the MC symptom
  • Bibasilar, fine end-inspiratory crackles
  • Clubbing (< 50% of pts)
65
Q

What is unique about asbestosis on CXR?

A

Formation of pleural plaques, generally on the parietal pleura.

Highly susceptible to mesothelioma.

66
Q

Why is HRCT preferred over CXR for asbestosis?

A

Far more sensitive, as people can present with normal CXR but abnormal HRCT.

67
Q

When is BAL used? What should it show for asbestosis?

A
  • Non-diagnostic HRCT.
  • Will show asbestos bodies
68
Q

What must a patient with asbestosis be counseled on?

A

Smoking cessation, because it will increase their risk of developing mesothelioma greatly.

69
Q

What is hypersensitivity pneumonitis?

A

Extrinsic inflammatory alveolitis due to exposure to inhaled, organic antigens leading to acute illness.

Nonatopic, nonasthmatic!

70
Q

What pathophysiology characterizes hypersensitivity pneumonitis?

A

Diffuse inflammation of interstitial lungs, terminal bronchioles, and alveoli.

71
Q

How does acute hypersensitivity pneumonitis present?

A
  • FLS
  • Should improve if exposure is removed.
  • CXR: poorly defined micronodular/diffuse interstitial pattern.

Onset is within hours.

72
Q

How does subacute/chronic hypersensitivity pneumonitis present?

A
  • Insidious onset of wet cough, dyspnea, fatigue, anorexia, and wt loss.
  • CXR: Fibrotic changes with loss of lung volume and coarse, linear opacities.
73
Q

What secondary treatment can be provided for hypersensitivity pneumonitis besides removing the offending agent?

A

Oral corticosteroids in severe/protracted cases.

74
Q

What factors contribute to radiation lung injury?

A
  • Volume of lung irradiated
  • Dose/rate of exposure
  • Concurrent chemo, previous therapy, and withdrawal of corticosteroid therapy.
75
Q

How does radiation pneumonitis present?

A
  • Insidious onset of dyspnea, intractable dry cough, chest fullness, pain, weakness, and fever.
  • Severe would present with respiratory distress or cyanosis
  • Inspiratory rales
  • CXR: Alveolar or nodular opacities limited to irradiated area + air bronchograms.

Presents 2-3 months after radiation therapy completion.

76
Q

How do we treat radiation pneumonitis?

A

Supportive. Steroids may be given.

77
Q

What kind of patients are most susceptible to pulmonary radiation fibrosis?

A

Patients who received a full course of radiation therapy for lung or breast cancer.

78
Q

What is the primary clinical symptom seen in pulmonary radiation fibrosis?

A

Slow, progressive dyspnea

79
Q

What CXR findings are seen in pulmonary radiation fibrosis?

A
  • Obliteration of normal lung markings
  • Dense interstitial and pleural fibrosis
  • Reduced lung volumes
  • Tenting of the diaphragm
  • Sharp delineation of the irradiated area
  • Diminished R lung volume
80
Q

What is the treatment for pulmonary radiation fibrosis?

A

Supportive. Steroids have no efficacy

81
Q

What are the common CT diseases associated with ILDs?

A
  • Progressive systemic sclerosis
  • RA
  • SLE
  • Sjogren’s
  • Polymyositis and dermatomyositis

Always evaluate for CTD if someone has an ILD.

Pulmonary symptoms can precede the CTD.

82
Q

What are the MC symptoms associated with drug-induced ILDs?

A
  • DOE
  • Non-productive cough

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