DILD- Pulmonary Fibrosis, Pneumonitis Patho, Drugs, Treatment

Question 1

Pulmonary fibrosis, pneumonitis pathology

Answer 1

Usual or nonspecific interstitial pneumonia, can be drug-induced or idiopathic

Question 2

Pulmonary fibrosis, pneumonitis: can fibrosis occur with these drugs?

Answer 2

Yes, and can be preceded by an acute pneumonitis, fibrosis can lead to pulmonary HTN

Question 3

Pulmonary fibrosis, pneumonitis: onset

Answer 3

acute, subacute, chronic

Question 4

Pulmonary fibrosis, pneumonitis: symptoms

Answer 4

nonproductive cough and sudden onset dyspnea (hours), fever, rash, eosinophilia

chronic Sx: slowly progressing breathlessness, decreased physical activity

Question 5

Pulmonary fibrosis, pneumonitis chest CT

Answer 5

fibrosis- decreased lung volumes, BL diffuse ground-glass opacities

(Diagnosis of exclusion- I don’t think we need to know this)

Question 6

Pulmonary fibrosis, pneumonitis PE

Answer 6

crackles on expiration, clubbing

Question 7

Pulmonary fibrosis, pneumonitis: purpose of BAL

Answer 7

distinguishes between cell differential, rules out malignancy or infection

(Used to rule out other causes- I don’t think we need to know this)

Question 8

Nitrofurantoin DILD mechanism

Answer 8

Imbalance of oxidant/antioxidant

Question 9

Nitrofurantoin DILD presentation

Answer 9

Acute eosinophilic pneumonia
Chronic pulmonary fibrosis: 8 months-16 years

Question 10

Leflunomide, methotrexate DILD mechanism

Answer 10

Hypersensitivity

Question 11

Leflunomide, methotrexate DILD presentation

Answer 11

onset of days-years

Question 12

Bleomycin DILD mechanism

Answer 12

Cytokine, inflammatory cells, free O2 radial induction

Question 13

Belomycin DILD presentation

Answer 13

Weeks-months, can progress to fibrosis

Well known side effect of bleomycin!

Question 14

Busulfan DILD mechanism

Answer 14

Direct alveolar injury

Question 15

Busulfan DILD presentation

Answer 15

4 years after monotherapy or months after a high dose

Question 16

Carmustine DILD presentation

Answer 16

Months-years after initiation
Can progress to fibrosis after years

Question 17

Cyclophosphamide DILD mechanism

Answer 17

Direct alveolar injury

Question 18

Cyclophosphamide DILD presentation

Answer 18

Months of initiation (early)
Months-years (late)

Question 19

Gemcitabine DILD mechanism

Answer 19

Endothelial dysfunction after cytokine release

Question 20

EGFRIs DILD presentation

Answer 20

Within 1 month

Question 21

ICPIs DILD presentation

Answer 21

Onset within 3 months but may be faster if used in combination with other medications

Question 22

mTORIs DILD mechanism

Answer 22

DAD or hypersensitivity

Question 23

mTORIs DILD presentation

Answer 23

51-104 days, daily regimens more likely than weekly ones

Question 24

Taxanes DILD mechanism

Answer 24

Hypersensitivity, direct toxicity to organ, pneumonia

Question 25

Taxanes DILD presentation

Answer 25

During Cycle 2 of treatment or within 18 days after last cycle ends

Question 26

Amiodarone DILD mechanism

Answer 26

Direct toxic effect

Question 27

Amiodarone DILD presentation

Answer 27

4 weeks-6 years

Question 28

Is amiodarone DILD dose-dependent?

Answer 28

YES! Smaller doses over years (>2 years) or larger doses over a shorter period of time (400mg/day for >2 months) are at risk Patients over 60 have a 3x increased risk of toxicity each subsequent decade

Question 29

Treatment of DIILD: Immunotherapy Check-Point Inhibitors (ICPis), Grade 1 event

Answer 29

consider holding meds and reassess in 1-2 weeks

Question 30

Treatment of DIILD: Immunotherapy Check-Point Inhibitors (ICPis), Grade 2 event

Answer 30

hold meds, prednisone/methylprednisone 1-2mg/kg/day if you improve to grade 1, taper over 4-6 weeks, if no improvement in 48-72 hours, treat as grade 3

Question 31

Treatment of DIILD: Immunotherapy Check-Point Inhibitors (ICPis), Grade 3 or 4 event

Answer 31

permanent D/C, methylprednisone 1-2mg/kg/day Taper over 4-6 weeks no improvement in 48 hours- infliximab, IVIG, MMF

Question 32

Treatment of DIILD: mTORIs, Grade 1 event

Answer 32

Nothing, it's fine

Question 33

Treatment of DIILD: mTORIs, Grade 2 event

Answer 33

Dose reduce or hold medication

Question 34

Treatment of DIILD: mTORIs, Grade 3 event

Answer 34

hold med

Question 35

Treatment of DIILD: mTORIs, Grade 4 event

Answer 35

permanently D/C medication

Question 36

For ALL Grade events (except for 1) of mTORI treatment, what else do you give them?

Answer 36

Prednisone 0.75-1mg/kg/day and treat until they're a Grade 1

Question 37

Bleomycin treatment

Answer 37

Prednisone 0.75mg/kg/day for 4-6 days, then taper

Question 38

Carmustine treatmentt

Answer 38

Prednisone 60mg PO BID, then 30mg PO QD then tapered by 10mg PO QW, then 5mg PO QW

Question 39

Amiodarone treatment

Answer 39

Prednisone 0.5-1mg/kg/day, continue for several months up to 1 year