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Pulm > SM_168a: IIPs > Flashcards

SM_168a: IIPs Flashcards

1
Q

What is interstitial lung disease?

A

Disease in the interstitial space around alveolar capillary units

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2
Q

This shows diffuse pulmonary metastases, which _____ DPLD

A

This shows diffuse pulmonary metastases, which are not DPLD

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3
Q

This is multifocal pneumonia, which _____ DPLD

A

This is multifocal pneumonia, which is not DPLD

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4
Q

What are the 6 questions for suspected IIP?

A
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5
Q

If there is an _________ for a patient’s ILD, they do not have idiopathic ILD regardless of CT or pathology findings

A

If there is an identifiable trigger for a patient’s ILD, they do not have idiopathic ILD regardless of CT or pathology findings

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6
Q

Why is a detailed occupational and exposure history important for evaluating a person with lung problems?

A

Can tell whether lund disease is idiopathic or not

  • Pets
  • Mold
  • Dust
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7
Q

If a patient with connective tissue disease (autoimmune disease) comes in with lung problems, think that the patient might have ______

A

If a patient with connective tissue disease (autoimmune disease) comes in with lung problems, think that the patient might have ILD

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8
Q

What is the basic lab work for a patient with lung issues?

A
  • Connective tissue disease labs
    • ANA panel (ANA, SSA/SSB, Scl-70, RNP)
    • Rheumatoid arthritis: RF, anti-CCP
    • Myositis: CPK, aldolase, myositis panel
  • Hypersensitivity pneumonitis panel
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9
Q

This is DPLD in a patient on amiodarine, which ____ an IIP

A

This is DPLD in a patient on amiodarine, which is not an IIP

(this is drug-induced ILD)

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10
Q

This is DPLD in a 75 yo female w/ 3 cockatiels, which ____ an IIP

A

This is DPLD in a 75 yo female w/ 3 cockatiels, which is not an IIP

(this is hypersensitivity pneumonitis - see ground glass)

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11
Q

This is progressive fibrosis in a patient with an occupational silica exposure, which _____ an IIP

A

This is progressive fibrosis in a patient with an occupational silica exposure, which is not an IIP

(this is silicosis)

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12
Q

This is fibrosis in a 65 yo female w/ rheumatoid arthritis, which ____ an IIP

A

This is fibrosis in a 65 yo female w/ rheumatoid arthritis, which is not an IIP

(this is RA-ILD)

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13
Q

What is sarcoidosis?

A

Sarcoidosis is a multi-system idiopathic granulomatous disease

  • More common in younger patients (20-40 years old)
  • Bilateral hilar adenopathy and upper lobe parenchymal disease (often nodules)
  • Well-formed non-caseating granulomas on pathology
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14
Q

Sarcoidosis manifests as ________ on HRCT

A

Sarcoidosis manifests as bilateral hilar adenopathy and upper lobe parenchymal disease (often nodules) on HRCT

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15
Q

Sarcoidosis manifests as _______ on pathology

A

Sarcoidosis manifests as well-formed non-caseating granulomas on pathology

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16
Q

Outcomes are _____ with IPF versus other IIPs

A

Outcomes are notably worse with IPF versus other IIPs

(median survival of 3.5 years)

17
Q

Therapy for IPF involves ______, while ______ is harmful

A

Therapy for IPF involves antifibrotic therapy (pirfenidone or nintedanib), while immunosuppresion is harmful

18
Q

Therapy for HP, CT-ILD, sarcoidosis, AEP/CEP, COP, and NSIP involves ______

A

Therapy for HP, CT-ILD, sarcoidosis, AEP/CEP, COP, and NSIP involves immunosuppression (steroids +/- steroid-sparing agents)

19
Q

Describe the clinical and radiographic features consistent with IPF

A
  • Indolent cough and dyspnea
  • No obvious trigger
  • Older (generally > 65)
  • History of or current smoker
  • Family history (genetic component)
  • Usual interstitial pneumonia (UIP) pattern on HRCT: sub-pleural basilar-predominant fibrosis, reticulation, honeycombing, traction bronchiectasis (not mandatory)
20
Q

The UIP pattern of IPF on HRCT consists of ______, ______, ______, and ______

A

The UIP pattern of IPF on HRCT consists of sub-pleural basilar-predominant fibrosis, reticulation, honeycombing, traction bronchiectasis (not mandatory)

21
Q

What do you information need to be sure that someone has IPF?

A

Consistent story + consistent imaging + consensus at multidisciplinary conference = IPF

No further testing required

22
Q

Strongly discordant HRCT features mean the patient _____ IPF

A

Strongly discordant HRCT features mean the patient does not have IPF

23
Q

Is this UIP?

A

This is not UIP because there too much ground glass (too hazy)

24
Q

Is this UIP?

A

No, this is not UIP because this is upper-lobe predominant fibrosis

25
Q

Is this UIP?

A

This is not UIP because this is consolidation (not fibrosis)

26
Q

Is this UIP?

A

This is not UIP because there is peribronchovascular distribution (not subpleural)

27
Q

Is this UIP?

A

This is not UIP because there are diffuse micronodules

28
Q

When there are some features consistent and some features inconsist with IIP, further testing involves ______

A

When there are some features consistent and some features inconsist with IIP, further testing involves lung biopsy

(little ground glass, some upper lobe fibrosis and some lower lobe fibrosis, no honeycombing, younger age)

29
Q

______ is the pathologic correlate of clinical IPF

A

UIP is the pathologic correlate of clinical IPF

  • Spacial heterogeneity
  • Fibroblastic foci
  • Microscopic honeycombing
30
Q

IPF is treated with ______ and ______

A

IPF is treated with Pirfenidone and Nintedanib

(anti-fibrinolytics, which slow decline of lung function but do not improve mortality)

31
Q

Pirfenidone, which is used to treat IPF, acts by _______

A

Pirfenidone, which is used to treat IPF, acts by inhibiting TGF-beta mediated collagen synthesis

  • Decrease rate of FVC decline, may reduce mortality
  • Side effects: nausea, photosensitivity rash, dyspepsia, elevated LFTs
32
Q

Nintedanib, which is used to treat IPF, is a _______

A

Nintedanib, which is used to treat IPF, is a multiple tyrosine kinase inhibitor

  • Decrease rate of FVC decline, may reduce exacerbations
  • Side effects: diarrhea, nausea, vomiting, elevated LFTs

Pulm (32 decks)

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