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TXP: Pulm/ Tox. And Rheumatology > Week 3: Cystic Fibrosis/ Drug Induced Pulmonary Disease > Flashcards

Week 3: Cystic Fibrosis/ Drug Induced Pulmonary Disease Flashcards

1
Q

Root Cause of CF

A

defect in cystic fibrosis transmembrane (CFTR)conductance regulator. Transports Cl- and bicarbonate.

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2
Q

organs typically involved in cf

A

skin

pancreas lung

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3
Q

Epidemiology

A

most common lethal genetically inherited disease affecting caucasions

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4
Q

outcome of cf dependent on ..

A

disease/genotype

pt mangement

compliance w. therapies

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5
Q

CFTR mutation classes and order of Prevalence

A

Class I mutation: nucleus

Class II mutation: Golgi apparatus

both effect synthesis of CTFR mutation, and cannot reach epithelial surface

Class III, IV, V, : dysfunctional CFTR proteins. little to no Cl can be transfered outside of cell

Class II»>Class I>Class III~Class IV>Class V

Classes IV and V cause milder disease because of little transport

classes I-III have no cloride transport

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6
Q

Most common mutation of CFTR

A

F508del

can either be homozygous or heterozygous

homozygous F508del=44.2%
heterozygous F08del= 40.5%

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7
Q

CF patho

A

mutated CFTR gene

loss of CFTR function

Impaird Bacterial Eradication

Infection

Inflammation

Airway Remodeling

Airway Obstruction

Broncheiectasis

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8
Q

Most common pathogen affecting young children and adulthhod

A

children: staph. Aureus

1.s.aureus
2.H. influenzae
3.ps aeruginosa

adults: psudamonas aeruginosa

1.p. aeuginosa
2.s. aureus
3. MRSA

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9
Q

chronic infection in CF triggers inflammation involving what mediators

A

tnf-a
IL-
GM-CSF
Leukotrienes
Neutrophils etc.

further worsens obstruction and creates better environment for infection

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10
Q

Exocrine dysfunction due to CF

A

occurs in 85-90% of CF pts.

results in obstruction and consequently deficient in ..
Protease, amylase, lipase
bicarbonate

causes poor absoprtion of…
*fat soluble vitamins (ADEK),vit. b12, zinc

Long term xocrine dysfunction: Acinar celldestruction->fibrosis->progressive adipose replacement of pancreatic tissue

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11
Q

SS of poor digestoin and complications

A

abdominal distention,increased stool frequency w.loose consistency/foul odor

increased fecal fat content

endocrine dysfunction: CF related diabetes (5-30% prevelance)

complication:mal nutrition and poor weightgain (predictor of mortality)

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12
Q

CF comorbidities

A

depression
anxiety

manifestations: asthma, acid reflux, CF related diabetes, sinus disease

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13
Q

CFTR Modulators

ex: Ivacafter (Kalydeco)
moa:
age indication:
Class mutation indications:
specific mutation indications:

A

ex:
moa: Facilitates opening of the chloride channel (CFTR potentiator)”

age indication: >/4 months

Class mutation indications: class III-IV when used alone

specific mutation indications: G551D, R117H, etc.

considerations: can be used as monotherapy or combo

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14
Q

CFTR modulators

ex: Lumacaftor/ Ivacaftor (Orkambi)

moa:

age indication:

Class mutation indications:

specific mutation indications:

pearls:

A

CFTR modulators

ex:

moa: fixes the defective CFTR protein so it can move to the proper place on the airway cell surface (CFTR corrector). Ivacaftor serves as potentiator

age indication: >/2 years old

Class mutation indications: Class Ii

specific mutation indications: homozygous F508del

pearls:

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15
Q

CFTR modulators

ex: Tezacaftor/ Ivacaftor (Symdeko)

moa:

age indication:

Class mutation indications:

specific mutation indications:

pearls:

A

CFTR modulators

ex:

moa:T.fixes the defective CFTR protein so it can move to the proper place on the airway cell surface (CFTR corrector). Ivacaftor serves as potentiator

age indication: >/6 y.

Class mutation indications:

specific mutation indications: homo or hetero mutation of F508del CFTR, 3849+10kbC->T

pearls:
*MUST INCLUDE A mutation that is responsive to symdeko

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16
Q

CFTR modulators

ex: Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)

moa:

age indication:

Class mutation indications:

specific mutation indications:

pearls:

A

CFTR modulators

ex: Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)

moa: E and T fixes the defective CFTR protein so it can move to the proper place on the airway cell surface (CFTR corrector). Ivacaftor serves as potentiator

age indication: >/6 years old

Class mutation indications:

specific mutation indications: homo or hetero F508 delta

pearls: doesnt have limitations like symdeko has as far as specific mutation response

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17
Q

Ivacaftor Studies

A

STRIVE trial: ivacaftor improved FEV1

ENVISION trial: improved fe1 at 24 and 48 wks

PERSIST study:

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18
Q

Lumacaftor/ Ivacaftor

A

TRAFFIC &TRansport trials

improved FEV1 @ 24 weeks

PROGRESS studies:

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19
Q

Tezacaftor + Ivacaftor trials

A

EVOLVE trials: improvement of fev1 @24 WEEKS

EXPAND trial:

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20
Q

Considerations for general CFTR modulator use

A

must take w. fat containing meal.

get basline ast/alt levels

ast/alt q 3 mo. first 1 year, annually therafter

dose reduction required in mod-severe hepatic dysfunction

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21
Q

DDIs of CFTR modulators

Ivacaftor, Tezacaftor, Elexacaftor

mechanism of DDI:
EX:
Affect on AUC and CMAX:
dose adjustment :

A

I, Tz, El: cyp3a4 substrates
mechanism of DDI: moderate cyp3a4 inhibitors EX: erythromycin and fluconazole
Affect onAUC and CMAX: increase
dose adjustment :
*I once daily,
* Tz, El: qod alternating btw T+I( or E/T/I) and I

mechanism of DDI: STRONG cyp3a4 inhibitors EX: clarithromycin and itroconazole
Affect onAUC and CMAX: significant increase
dose adjustment :
*I : twice weekly
* Tz, El: twice weekly T/I or E/T/I

mechanism of DDI: CYP3A4 induction
EX: rifampin, carbamezapine, phenobarbital, pheytoin, st johns wort
Affect onAUC and CMAX: significant decrease
dose adjustment :
*T : avoid concomitant use
* Tz: avoid concominant use

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22
Q

DDIs of CFTR modulators

A

Lumacaftor/ Ivacaftor: STRONG cyp3a inducer

cotreatment w. cyp3a4 inhibitors (ex itraconazole),

if L/I added to a regimen w. storng inhibitor..
decrease lumacaftor/ivacaftor dose to 1 tab daily during first week, then rsume normal dose therafter

if string inhibitor added to L/I regimen.. no dose adjustment needed

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23
Q

Non Pharm Treatment of CF lung disease

A

high frequency chest wall oscillation (HFCWO) aka “vest” therapy

postural draining (PO)

Positive Expiratory pressure (PEP)

Oscillatory PEP-devices : flutter, Acapella, AerobikA and Corney

Exercise

Annual Influenza vaccination starting at age of 6 mo.

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24
Q

Topical Mucolytic/ Hydrating Agents

ex: Dornase Alfa

indication:

dose:

AE:

considerations:

A

ex:

indication: mucolytics

dose: 2.5 mg inhalation 1-2x daily

AE: hoursenss of voice, rash

considerations:
improves FEV and decrease acute pulmonary exacerbations.
well tolerated
chronic use recommended in mild and strongly recommended in mod-severe disease to improve lung function and increase qol
more commonly used in >/ 6y.o

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25
Q

Topical Mucolytic/ Hydrating Agents

ex: Hypertonic Saline

indication:

dose:

AE:

considerations:

A

ex:

indication: helps pull water into airway and derease thickness of scretion, making it easier

dose: 4mL inhalation BID

AE: bronchospasm, can be mitigated by albuterol

considerations:
limited impact on FVC OR FEV1, but does decreas rate of APE

well tolerated
chronic use recommended in mild-sev. disease to increase lung function and inc. qol
more commonlyused in adults >/6

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26
Q

broncodilator considerations

A

used in ~90% of pts

cf has asthma like component, makingthese beneficial

improve deposition of inhaled meds

SABAs such as albuterol commonly used

LABAs less comonly used such as salmeterol

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27
Q

anti-inflammtories: Azithromycin considerations

indication:
dose:
AE:

A

Azithromycin most commonly used antifinflammatory

most clear indication: in pts >/6 y.o chronically infected with P. Aeruginosa (and w.o) to improve lung function and reduce APE in mild-severe pts

dose:
10mg/kg PO MWF in pts. <25kg (off label dosing)
250mg PO MWF in pts. <40 kg
500 mg PO MWF in pts >40 kg

studies saww..
increase in FEV1 and fvc
increased ABW
decreased exacerbation
improv wol.
small studies saw abx resistance

ae: well tolerated. N,V wheezing

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28
Q

Considertions for inhaled abx

indication:
target organism:
dosing schedule

A

provide high conc directly to site of infection.
*targets bacterial colonization to decrease number of exacerbations

systemic absorbtion minimal

have on and off dosing (28 days on, 28 days off)to prevent adaptive resistance

target P. aeruginosa
indication: suppresive therapy chronic pulmonary infection due to p. aeruginosa in age >/6. recommended in milf and strongly recommended in mod-severe disease

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29
Q

inhaled ABX for CF

A
  1. Inhaled tobramycin
    tobramycin (TOBI) 300 mg inhaled BID-28 days ON and 18 OFF
    *nebulizer solution: administer over ~15 min

tobi Podhaler 112mg (4x28 mg caps) inhaled BID 28 days ON and 28 OFF
*DPI: administer over 2-7 min

AE: voice alteration, tinitis

  1. Inhaled Aztreonam
    75 mg inhaled TID 28 days ON and 28 OFF.
    *neb solution: administered over 2-3 min using altera nebulizer

AE: bronchospasm (can pretreat with SABA)

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30
Q

goal BMIs for CF pts

A

children: BMI> 50th percentile

adults:
MAle: BMI>23
Female: BMI>22

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31
Q

recommended diet for CF

A

MODERATE FAT, HIGH PROTEIN, AND HIGH CALORIES.

enteral feedings for children fallen off of the growth curve and asults not gaiing/maintaining weight

32
Q

Nutrition: Pancreatic Enzyme Replacement Therapy(PERT) dose

emperic doses
maintenance dose
considerations for dose asjustments

A

recommended dose:based on lipase component

empiric dose:
infants: 2000-5000 U/ 120 mL formula
children<4 y.o: 1000U/KG w/ meals and 1/2 dose w. snacks
Children>4 and adults: 400-500 U/kg w. mals and 1/2 dose w. snacks.
*mean dose=1800-1950 units/kg/meal

maintenance dose:
*titate dose based on abw, STOOL FAT CONTENT, AND GI SYMPTOMS

consider decreasing dose if
*pt on high doses w. good effect
*pt having AE

consider increasing if
*poor weight gain
*pt experiencing bloating, increased # of fatty stools

33
Q

PERT clinical pearls

A

brands and formulations not interchangeable

choose best dosage form: #after brand name is U/capsule. choose a formulation and round dose to nearest whole capsule

plan how it will be administered. (children<8 cant swallow tabs/caps well, so dose w. soft foods

34
Q

Nutrients: Supplementation

multivitamins:
vitamin D:
vitamin k:
minerals:

A

multivitamins supplement s contaiing fat soluble vitamins(ADEK)

age<12 mo: 1mL PO QD age 1-3: 2 mL PO qd
AGE4-10 Y.: 1 TAB po qd AGE >10 2 TAB po qd

VitD supplements for pts hacing low 25-oH vit. D levels
Age 1-11 years: Vitamin D3 – 1000 IU QD
 Age > 11 years: Vitamin D3 – 2000 IU QD
If level < 30 ng/ml after 3 months treatment…
o Age < 5 years: Vitamin D3 – 50,000 IU MWF X 1 month
o Age>5years:VitaminD3–50,000IUQDX1month
o May repeat course or refer if < 30 ng/ml after treatment course

Vit K supplemets during IV abx treatment.
*phytonadione (Vit.K( 5 mg PO twice weekly)

Minerals:
*Ferrous sulfate (iron),
*if poor weight gain-> zinc sulfate 1mg/kg/d divided bid

35
Q

VITAMIN supplementation considerations

A

kids <8y.omay not be able to swallow tabs/caps

may crush tabs and take them w. soft foods

36
Q

Acute Pulmonary Exacerbation

A

acute worsening of pullmoary symptoms

cough
increased sputum production
sob
chest pain
loss of appeitite
wightloss
lun function decline

37
Q

airway clearance during APE of CF

A

increase vest treatment
increase dornase alfa
increase hypertonic sale
increase bronchodilator

38
Q

abx trt of APE of CF considerations

A

abx selection:
*emperic abx based on population data in absense of culture data
*when culture is available , abx selection can be individualized based on historical culture data

abx dosing:
if pt has not been treated w. a given abx or if abx not monitored by tdm, dosig based on populaion pk data

if a pt trated w. a given abx tht required tdm, dosing can be individualized based on pt specific historical pk data. (this process helps get pts to goal quicker)
ex: if pt recieved tobramycin 10 mg and was titrated to 12 mg that pput them in the therepeutic range, next time they are admitted, they should be started on 12, assuming no change in clinical status or renal function

39
Q

most common clinical situations requiring abx treatment in CF (empiris)

A

cf exacerbation w. hx MSSA ( no PA)
*anti-staph PCN/CEPH

cf exacerbation w. hx of MSSA AND PA
*double PA coverage: aminoglycoside+cefepime

cf exacerbation w. hx of MRSA (no PA)
*vancomycin or linezolid

cf exacerbation w. hx of MRSA and PA
*vanco or Linezolid PLUS
*double PA coverage: aminoglycoside+beta lactam (eg. ceftazidime)

when C&S. therapy can individualized

40
Q

Less common clinical situations for abx treatment of APE

A

b cepacia and/or S. maltophilia
*combo therapy w. 2-3 drugs needed, guided by C&S

41
Q

Abx dosing strategy

A

Extended dosing:
*dose q24 hrs.
*takes advantage of conc dependent killing strategies and maximizing peak/MIC interval
*increases drug free interval, dec amount in kidneys(reduce toxicity)

41
Q

Abx dosing strategies

A

Extended Interval aminoglycioside dosing (EIAD):
*dose q24 hrs.
*takes advantage of conc dependent killing strategies and maximizing peak/MIC interval
*increases drug free interval, dec amount in kidneys(reduce toxicity). once daily dosing is preferable to TID dosing

Continous and extended/prolonged infusion beta lactam dosing
*beta lactama time dependent killing
*continous infusion-> maintains constant conc over course of regimen, dose individualized to target conc. above MIC
*extended infusion: conc graudlay increases until infuion stops. then decreases based on pts. pk parameters
*insufficient evidence to recommend continous (extended) infusion for beta lactams for APE trtment

42
Q

duration of abx treatent of APE

A

duration of 14-21 days or longer

if no improvement in 5-7 days, reculture and or ajust abx

therapy may be completed at home w. PICC or port, or step down PO

43
Q

monitoring efficacy and saftey for abx tretment in APE in CF

A

symptom persistence/ resolution

pulmonary function tests..FEV1 FVC

sputum culture and susceptibilities

BUN/ Scr

abx serum concentrations

44
Q

Aminoglycoside monitoring for efficacy and efficacy

A

monitor peak and trough amg conc

traidiotnsl dosing: 10-12 mcg/mL/goal trough <1-2

EIAD: goal peak: 22.5-27.5 mcg/mL/ Goal 18hr level<1 mcg/mL
goal AUC 80-100 mcg/mlh

serum conc drawn initially and then q3-7 days

45
Q

aminoglycose pk equations

A

Ke=ln(c1/c2)/deltaT

C=C0e^-kt

T1/2=0.693/ke

46
Q

monitoring efficacy and safety : vancomycin

A

time dependent abx. trough concentrations needed only

goal 10-20 mcg/mL(to achieve AUC/MIC>/400

serum conc should be drawn initially once pt reaches Css (18-30 hrs), then q3-7d after

47
Q

Unique issues for abx treatment acute cf exacerbations

A

multiple abx allergies: CHECK PT ALLERGIES BEFORE TRTMT

difficulty of penetration to site of infection due to thick mucus as well as mucoid layers on bacteria itself, especially on P. aeruginosa

more prone to AE of abx therpay, short term an dlong term

because reiceve multple abx therapies over lifetime, increase risk fo rmultidrug resistant organisms

abx pk:
*general rule. VD and CL INCREASEDDD (vs general population) of beta lactams and AMG in CF population

48
Q

lung mechanisms of injury

A

oxidant injury

immune complex mediated

interferance with matrix formation

interferance with lipid metabolism

49
Q

epidemiology of pulmonary toxicities

A

acute or chronic-> get good hx

ranges from reversible of toxicity to death

stopping med is most important step. corticosteroids can hellp, but not alot of good data

most present w. resp. symptoms and changes on xray

it is a dx og exclusion (Naranjo Scale)

lungs are capable of drug metabolism, co it can afect pulmonary vascular in a variety of ways

50
Q

naranjob scale

A

estimates the probability of an adverse event

important questions in scale
*did the adverse event appear after suspected drug was administered
*was the reACTION MORE SEVER WHEN THE DOSE WAS INCREASED OR LELSS SEVERE WHEN THE DOSE WAS DECREASEd

score>/5 there is an advers eevent

51
Q

risk factors for drug induced pulmonary diease

A

drug related
*dose or rate of administration
*treatment duration
*oxygen therapy
*radiation
cumulative dose (anything that can decrease antioxidants)

pt realted factors
*age (extremes of age)
*RA or preexisting lung disease
*impaired renal/hepatic function (review pkpd parameters)
genetics

52
Q

Types of drug induced interstitial lung diseases

A

pulmonary fibrosis

pneumonitis

organizing pneumonia

eosinophilic pneumonia

hypersensitivity pneumonia

noncardiac pulmonary edema

diffuse alveolar damage/ diffuse alveolar hemorrhage (DAD/DAH)

53
Q

drug induced insterstitial pneumonitis or fibrosis (DIP/F)

patho

onset

SS

Chronic

PE:

chest CT

A

patho:
usual or nonspecific interstitial pneumonia
*drug induced pulmonary fibrosis or idiopathic pulmonary fibrosis
*fibrosis can occur with many drugs and may be preceded by an acute pneumonitis
*fibrosis can lead to pulmonary HTN

onset: acute, subacute, chronic

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

Chronic: slowly progressing breathlessness, decreased physical activity

PE: crackles on expiration, clubbing

chest CT: fibrosis (decreased lung volumes, BL diffuse ground-glass opacities (honeycombing)

BAL: bronchoscopy alveolar lavage

54
Q

DIPF mechanism of toxicity

A

direct toxic effect or oxidative stress
v
causses damage to
v
*alveolar epithelial cells
*platelet activation, release of
inflammatory cells and mediators,causes fibroblast recruitment
*or endothelial cells
*increases permeability and vascular leak
*impair cell repair and cell death
V
overall increases inflammation and excess deposition of extracellular matrix
v
causes remodeling, honeycombing,and fibrosis

55
Q

causative agents of DIPF

antimicrobial
nitrofurzntoin

mechanism

presentation

incidence

A

mechanism: imbalance of oxidant/antioxidant

presentation:
*acute eosinophilic pneumonia
*pulmonary fibrosis (chronic):8. mo-16yrs

incidence: <10%, mortality: 8% (chronic)

56
Q

causative agents of DIPF

antirheumatic: Leflunomide

mechanism

presentation

incidence

A

causative agents of DIPF

mechanism–

presentation–

incidence: incidense-1.46-0.63%

57
Q

causative agents of DIPF

METHOTREXATE

mechanism

presentation

incidence

A

causative agents of DIPF

mechanism: hypersensitivity

presentation: onset days-years

incidence: incidence 0.43%
(low dose);5-10%

58
Q

causative agents of DIPF(ANTINEOPLASTICS)

BLEOMYCIN

mechanism

presentation

incidence

mortality

A

causative agents of DIPF(ANTINEOPLASTICS)

mechanism: cytokine, inflmmatory cells and free o2 radical induction

presentation: weeks to months;can progress to fibrosis (well known)

incidence: 6.8-21%

mortality: 48%

59
Q

causative agents of DIPF(ANTINEOPLASTICS)

BUSULFAN

mechanism

presentation

incidence

mortality

A

causative agents of DIPF(ANTINEOPLASTICS)

mechanism: direct alveolar injury

presentation: 4 years after monotherapy: months after high dose

incidence: 6%

mortality:25%

60
Q

causative agents of DIPF(ANTINEOPLASTICS)

CARMUSTINE

mechanism

presentation

incidence

mortality

A

causative agents of DIPF(ANTINEOPLASTICS)

mechanism–

presentation- months-years after initiation. can progress to fibrosis (years)

incidence: 1.5-20%

mortality: –

61
Q

causative agents of DIPF(ANTINEOPLASTICS)

CYCLOPHOSPHAMIDE

mechanism

presentation

incidence

mortality

A

causative agents of DIPF(ANTINEOPLASTICS)

mechanism: Direct alveolar injury

presentation: months of initiation (early), months-years (late)

incidence: 1%

mortality: 25%?

62
Q

causative agents of DIPF(ANTINEOPLASTICS)

GEMMCITABINE

mechanism

presentation

incidence

mortality

A

causative agents of DIPF(ANTINEOPLASTICS)

mechanism: endothelial dysfunction after cytokine release

presentation: –

incidence” 1.1-1.9% or up to 20%

mortality: 22%

63
Q

causative agents of DIPF

EGFRIs (epideral growth factor receptor inhibitors)

mechanism

presentation

incidence

mortality

A

causative agents of DIPF

mechanism: —

presentation: Acute-1 within 1 month

incidence:
*erlotinib/gefitinib: 1.2-1.6%
*mobocertinib/osimertinib: 3.3-4.3%
*cetuximab/panitumumab: 1%

mortality:
*erlotinib/gefitinib: 22.8%
*mobocertinib/osimertinib: 0.5-1.2%
*cetuximab/panitumumab:50%

64
Q

causative agents of DIPF

ICPI’s(immune check point inhibitors)

mechanism

presentation

incidence

mortality

A

causative agents of DIPF

mechanism–

presentation: onset. 3 months (but many be faster if used in combo)

incidence:
PD-10-10%
PD-L1:0-10%
CTLA4: 0.01%

mortality–

65
Q

causative agents of DIPF

mTORis

mechanism

presentation

incidence

mortality

A

causative agents of DIPF

mechanism DAD or hypersensitivity

presentation: varies 51 days-104 days:Daily regimens>weekly

incidence: <0.5%-42%

mortality:–

66
Q

causative agents of DIPF

Taxanes

mechanism

presentation

incidence

mortality

A

causative agents of DIPF

mechanism:hypersensitivity: Direct Toxicity or organ

presentation: during cycle 2 and within 18 days after last cycle

incidence: Paclitaxel: 0.7-12%

mortality–

67
Q

causative agents of DIPF

AMIODARONE

mechanism

presentation

Age:

incidence

mortality

A

causative agents of DIPF

mechanism:direct toxic effect

presentation: 4weeks-6 years
dose dpeendent: Yes; smaller doses_~200 mg) over years (>2 years) or larger doses shorter time frames;about 400 mg/day (>2 months)
Age: patients >60 years old, 3x increase in risk of toxicity each subsequent decade

incidence:1.2-8.8%

mortality: 3-37%

68
Q

DRUG induced interstitial lung disease treatment due to ICPis

Grade 1:
Grade2:
Grade3,4

A

Grade 1 pneumonitis
*consider holding med
*reassess in 1-2 weeks

Grade 2
*hold meds
*prednisone/methylprednisolone1-2 mg/kg/day
V
*treat until improvement to grade 1>Then taper over 4-6 weeks
*if no improvement 48-72 hrs>treat as grade 3

grade 3,4:
*permanenet D/C
*methylprednisone 1-2 mg/kg/day
*taper over 4-6 weeks
* if no improvenent in 48hrs, give infliximab, IVIG, or MMF

69
Q

Treatment of DIILD: mTORIs

Grade 1:
grade2:
grade3:
grade4:

A

grade 1: no recommendation

grade 2: dose reduce or hold med
*prednisone 0.75-1mg/kg/day>continue until grade 1

grade 3:*hold med
*prednisone 0.75-1mg/kg/day>continue until grade 1

Grade 4: permamntly D/C
*prednisone 0.75-1mg/kg/day>continue until grade 1

70
Q

other treatments of DIILD: select medications

A

bleomycin: 0.75 mg/kg/day for 4-6 weeks, then taper

Carmustine: Prednisone 60 mg po BID then 30 mg po daily then tapered by 10 mg po weekly then 5 mg po weekly

amiodarone: Prednisone 0..5-1mg/kg/day. continue for several months to up to 1 year

71
Q

Prevention of DIILD

A

baseline spirometry, DLCO, and chest xray in high risk agents

should occur q2weeks to q4mo.

amiodarone: Baseline then q3mo if clnically indicated
bleomycin: chest xray q1-2 weeks; and DLCO monthly

72
Q

Drug induced Pneumonias

Bronchiolitis obliterans organizing pneumonia (BOOP)

what is it:
SS:
causative agents:
treatment:

A

what is it: acute inflammatory respons ein lung

SS:nonproductive cough, dyspnea, BL crackles, occasional fever/rash, xray: BL patchy infiltrates

causative agents: minocycline/nitrofurantoin, bleomycin, amiodarone, sulfasalazine, carbamezapine, cocaine

treatment: D/C agent or add steroids

73
Q

Eosinophilic pneumonia

what is it:
SS:
causative agents:
treatment:

A

what is it: infiltration of pulmonary insterstitium w. eosinophils; drug or toxin mediated

SS:dry cough, dyspnea, echest pain, feevr;BL gorund glass opacities

causative agents: daptomycin, nitrofurantoin, minocycline,mesalamine, sulfasalazine (ALL)

treatment: Acute: treat w. steroids
chronic: not as common

74
Q

HYpersensitivity pneumonitis

what is it:
SS:
causative agents:
treatment:

A

what is it: immediate reaction is more common
SS: urticaria, angioedema, rhinitis, dyspnea Chestxray: localized or BL alveolar infiltrates

causative agents:NSAIDS, methotrexate

treatment:D/C drug. antihistamines and possible steroids

75
Q

Drug induced pulmonary edema (noncardiogenic)

what is it:
SS:
causative agents:
onset:
remits:
treatment:

A

what is it:

SS: cough, crepitation, cyanosis/hypoxemia, chest xray (acinar infiltrate and normal heart size

causative agents: narcotics, (iv heroin, morphine, methadone,meperidine, propoxyphene, naloxone, nalmefene, HCTZ
*dose dependent moderate to high dose narcotics

onset:minutes-2hrs

remits:24-48hrs (symptoms, 2-5days (xray), 10-12 weeks (PFTs)

treatment: naloxone, oxygen, ventilator support

76
Q

Drug induced Lupus

what is it:
SS:
causative agents:
onset:
remit:
dose dependent?
treatment:

A

what is it:

SS:feever, myalgias, rash, althralgias, arthritis, and serositis, pleuritic pain
chest xrazy: pleural effusion, diffuse intersitial pneumonitis, and alveolar infiltrates

causative agents: !!procainamide!!, hydralazine, isoniazid, or anti-TNF alpha

compared to spontaneous lupus
*F:m ratio is 1:1 for DI lupus
rare in DIL(common in regular lupus): discoid lesions, malar erythema, renal disease, and CNS disease, no formation or complement and immune complexes

onset: up to 3 years after initiation

remit: 6 weeks

dose dependent? : no

treatment: drug withdrawal

TXP: Pulm/ Tox. And Rheumatology (7 decks)

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