restrictive lung disease/pulmonary hyperT

Question 1

ILD (interstitial lung disease)

Answer 1

aka restrictive lung disease: group of diseases characterized by diffuse lung injury and inflamm that leads to IRREVERSIBLE FIBROSIS and severely compromised gas exchange

Question 2

ILD considered a “restrictive” lung disease… why ?

Answer 2

fibrosis and lung stiffening/noncompliance = difficult to inflate. This increases work of breathing b/c of dec. lung volume

Question 3

Dx of ILD requires…

Answer 3

pathological dx by examination of lung tissue
& DETAILED HISTORY (enviornmental exposure)?
…to determine cause (i.e. sarcoidosis, pneumoconiosis, toxins, drugs, etc.)

Question 4

most important for evidence of restrictive lung disease

Answer 4

PFTs : will show decreased ventilation and perfusion with much worse perfusion (high V/Q ratio)

Question 5

PE for ILD

Answer 5

tachypnea, inspiratory “crackles”, no wheezing, clubbing (from chronic hypoxia)

Question 6

PE add-ons if cause of ILD is sarcoidosis

Answer 6

skin lesions, gland enlargments, CN abnormalities, hepatomegaly

Question 7

definitive Dx of ILD requires

Answer 7

biopsy

Question 8

Txt for ILD

Answer 8

remove cause (if known), corticosteroids, O2, supportive therapy in end stages

Question 9

idiopathic fibrosing interstitial pneumonia,
sarcoidosis &
pneumoconiosis are all types of …

Answer 9

ILDs

Question 10

idiopathic fibrosing interstitial pneumonia is AKA …

Answer 10

idiopathic pulmonary fibrosis

Question 11

txt for idiopathic pulmonary fibrosis

Answer 11

steroids (except if it is the UIP which needs lung transplant)

Question 12

what is UIP? who does it effect?

Answer 12

“the bad one” - usual interstitial pneumonia

50yo +

Question 13

UIP chest Xray and CT shows…

Answer 13

Xray: progressive fibrosis
CT: diffuse patchy fibrosis with honeycombing
(CT most useful)

Question 14

overall UIP- 3 major characteristics

Answer 14

idiopathic, progressive fibrosis, nothing helps except transplant

Question 15

sarcoidosis is …

Answer 15

well characterized systemic disease of uknown origin - granulomatous (inflammation) tissue in any organ system but mostly in lungs and lymphs

Question 16

Dx of sarcoidosis: 4 Dx techniques and results for Dx

Answer 16

Chest X ray and CT: bilat hilar (roots) lymphadenopathy
lung Bx: noncaseating granulomas & serum ACE
PFTs: restrictive & obst. patterns
gallium scanning to highlight lesions

Question 17

what helps the progressive dyspnea of sarcoidosis?

Answer 17

steroids (slows progression)

Question 18

flares of sarcoidosis get progressively _____

Answer 18

worse

Question 19

txt for sarcoidosis

Answer 19

none if mild, steroids to inflammation, transplant for definitive

Question 20

pneumoconiosis

Answer 20

ILD: black lung disease

Question 21

risk of developing pneumoconiosis depends on

Answer 21

how long pt had been around coal dust

Question 22

rxn of lungs to granulomatous tissue from sarcoidosis

Answer 22

fibrosis

Question 23

txt for pneumoconiosis

Answer 23

none

Question 24

pulmonary hypertension: what is it and common cause?

Answer 24

high blood pressure of the vessels in the lungs. commonly caused by fibrosis or other lung disease

Question 25

WHO new classification for pulmonary hypertension (2009) - medications are only recommended for those in group ____ but possibly harmful for those in group_____

Answer 25

``` group 1 (cause is something wrong with lungs) meds possible harmful to pts in group 2 and 3 (something wrong with heart, other causes and lungs secondarily) ```

Question 26

pathology for pulmonary hyperT

Answer 26

normally high blood flow and low pressure, low resistance

Question 27

symptoms of pulmonary hyperT

Answer 27

fatigue, chest pain, syncope on exertion

Question 28

CXR and EKG show what for pulmonary hyperT

Answer 28

right vent. hypertrophy

Question 29

Dx for pulmonary hyperT

Answer 29

cardiac Echo | right heart cath

Question 30

why the right heart cath for pulmonary hyperT?

Answer 30

measures pressures directly for vasoreactive testing - PA pressure before and after txt with vasoDs , those with PAH (pulm. arterial hyperT) will response with 10mmHg drop. - responders benefit from CCB s

Question 31

txt for pulmonary hyperT

Answer 31

for those with PAH and vasoresponsiveness = CCBs for nonresponders w/ PAH and others = epoprostenol if secondary- txt underlying cause lung transplant

Question 32

atrial septostomy

Answer 32

artificial hole between left and right atria: makes right side of heart not have to work as hard to push blood but dec. excercise tolerance.

Question 33

cor pulmonale

Answer 33

right sided HF most often caused by COPD ... | right vent. hypertrophy and HF that results from pulmonary disease/hypoxia

Question 34

labs for cor pulmonale show

Answer 34

inc RBCs (polycythemia) from hypoxic state, means more work for the heart

Question 35

txt for cor pulmonale

Answer 35

txt like HF- txt pulmonary problem, O2, diuretics, fluid restriction

Question 36

prognosis for cor pulmonale

Answer 36

bad (2-5years)