SM_168a: IIPs

Question 1

What is interstitial lung disease?

Answer 1

Disease in the interstitial space around alveolar capillary units

Question 2

This shows diffuse pulmonary metastases, which _____ DPLD

Answer 2

This shows diffuse pulmonary metastases, which are not DPLD

Question 3

This is multifocal pneumonia, which _____ DPLD

Answer 3

This is multifocal pneumonia, which is not DPLD

Question 4

What are the 6 questions for suspected IIP?

Answer 4

Question 5

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

Answer 5

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

Question 6

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

Answer 6

Can tell whether lund disease is idiopathic or not

• Pets
• Mold
• Dust

Question 7

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

Answer 7

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

Question 8

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

Answer 8

• 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

Question 9

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

Answer 9

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

(this is drug-induced ILD)

Question 10

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

Answer 10

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

(this is hypersensitivity pneumonitis - see ground glass)

Question 11

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

Answer 11

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

(this is silicosis)

Question 12

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

Answer 12

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

(this is RA-ILD)

Question 13

What is sarcoidosis?

Answer 13

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

Question 14

Sarcoidosis manifests as ________ on HRCT

Answer 14

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

Question 15

Sarcoidosis manifests as _______ on pathology

Answer 15

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

Question 16

Outcomes are _____ with IPF versus other IIPs

Answer 16

Outcomes are notably worse with IPF versus other IIPs

(median survival of 3.5 years)

Question 17

Therapy for IPF involves ______, while ______ is harmful

Answer 17

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

Question 18

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

Answer 18

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

Question 19

Describe the clinical and radiographic features consistent with IPF

Answer 19

• 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)

Question 20

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

Answer 20

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

Question 21

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

Answer 21

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

No further testing required

Question 22

Strongly discordant HRCT features mean the patient _____ IPF

Answer 22

Strongly discordant HRCT features mean the patient does not have IPF

Question 23

Is this UIP?

Answer 23

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

Question 24

Is this UIP?

Answer 24

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

Question 25

Is this UIP?

Answer 25

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

Question 26

Is this UIP?

Answer 26

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

Question 27

Is this UIP?

Answer 27

This is not UIP because there are diffuse micronodules

Question 28

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

Answer 28

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)

Question 29

\_\_\_\_\_\_ is the pathologic correlate of clinical IPF

Answer 29

UIP is the pathologic correlate of clinical IPF * Spacial heterogeneity * Fibroblastic foci * Microscopic honeycombing

Question 30

IPF is treated with ______ and \_\_\_\_\_\_

Answer 30

IPF is treated with Pirfenidone and Nintedanib (anti-fibrinolytics, which slow decline of lung function but do not improve mortality)

Question 31

Pirfenidone, which is used to treat IPF, acts by \_\_\_\_\_\_\_

Answer 31

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

Question 32

Nintedanib, which is used to treat IPF, is a \_\_\_\_\_\_\_

Answer 32

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