Pulmonary Fibrosis Flashcards Preview

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Flashcards in Pulmonary Fibrosis Deck (19):
1

Shared ILD features

Diffuse, bilateral pulmonary involvement (patchy?)

Distortion or destruction of alvoeli associated with inflamm cells

2

IPF key things

Older adults, limited to lungs, cannot explain why

3

Risk factors of IPF

Age
Cigarettes
Environmental
Fam history
GE rflux
Viruses (EBV other herpesviruses)

4

Major dx criteria

Exlusion of others

Abnormal pulm function studies show evidence of restriction and impaired gas exchange

Compatible HRCT

Transbronchial lung biopsy excluding other d

5

Minor dx

Age over 50

Insidious onset of unexplained dyspnea

Sx over 3 mos

Bibasilar inspiratory crakcles

Need all 4 major plus 3 minor to dx

6

Clinical evals of pts with dyspnea

Spirometry, lung volumes, diff capacity, exercise study...measure O2 sat with and without exercise

CXR and HRCT

Labratory data

7

Pulm function studies

LOw VC and TLC

FEV/FVC >75%

Reduced compliance

<60% predicted diffusion capacity

INcreased alv-arterial O2 difference (determined by alveolar air equation)...or resting or exercise hypoxemia

If coexisting with emphysema, could alter these

8

Gross pathology

Cobblestone lung

9

Lab studies to do

CBC, sed rate, liver, urinalysis

10

HS pnuemonitis hx

Think bird cages

11

Pathogenesis

Patchy, progressive epithelial injury and disruption of alveolar basement membranes

Ineffect reapir

Alvolear fibrosis associated with fibroblastic foci at the lung periphery

Keloid formation

12

Molecular endotyping

INjured epithelial cells basically recruit more fibroblasts

13

Fibroblastic focus

MUST be seen in order to call IPF

Start at the periphery and move inward

14

Once dx made

Immunize, O2, talk about end of life planning

15

What happens to pts?

Rapid progression

Slow progression

Stable

Most will have an acute worsening (exacerbation)...suddenly decline and die

16

Acute exacerbation

Deterioration characterized by new, widespread alveolar abnormality

WIll probably have hypoexmia or worsened hypoxemia

PDx - previous or concurring dx of IPF

Unexplained worsening or development of dyspnea within 30 days

HRCT with bilateral GGO or consolidation with background of reticular or honeycomb

Exlude other things

17

Comps and influences in prognosis

Pullm HTN

Pulm fibrosis/emphysema overlap

SLeep abnea

Venous TE

Lung cancer

HEart dz

18

Pirfenidone

Nintedanib

Don't need to know MOA

Lessen rates of decline in VC...best is started early

19

Approach to dx

If hx, PE, and physiologic, compatible, HRCT may be confirmatory

If quesiton about infection or sarcoidosis...transbronchial biopsy may help

If think other idiopathic lung dz, then surgical lung biopsy

Lung transplant?

Discuss newly available drugs