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Clin Med- P > restrictive lung disease/pulmonary hyperT > Flashcards

restrictive lung disease/pulmonary hyperT Flashcards

1
Q

ILD (interstitial lung disease)

A

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

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2
Q

ILD considered a “restrictive” lung disease… why ?

A

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

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3
Q

Dx of ILD requires…

A

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

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4
Q

most important for evidence of restrictive lung disease

A

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

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5
Q

PE for ILD

A

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

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6
Q

PE add-ons if cause of ILD is sarcoidosis

A

skin lesions, gland enlargments, CN abnormalities, hepatomegaly

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7
Q

definitive Dx of ILD requires

A

biopsy

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8
Q

Txt for ILD

A

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

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9
Q

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

A

ILDs

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10
Q

idiopathic fibrosing interstitial pneumonia is AKA …

A

idiopathic pulmonary fibrosis

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11
Q

txt for idiopathic pulmonary fibrosis

A

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

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12
Q

what is UIP? who does it effect?

A

“the bad one” - usual interstitial pneumonia

50yo +

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13
Q

UIP chest Xray and CT shows…

A

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

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14
Q

overall UIP- 3 major characteristics

A

idiopathic, progressive fibrosis, nothing helps except transplant

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15
Q

sarcoidosis is …

A

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

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16
Q

Dx of sarcoidosis: 4 Dx techniques and results for Dx

A

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

17
Q

what helps the progressive dyspnea of sarcoidosis?

A

steroids (slows progression)

18
Q

flares of sarcoidosis get progressively _____

A

worse

19
Q

txt for sarcoidosis

A

none if mild, steroids to inflammation, transplant for definitive

20
Q

pneumoconiosis

A

ILD: black lung disease

21
Q

risk of developing pneumoconiosis depends on

A

how long pt had been around coal dust

22
Q

rxn of lungs to granulomatous tissue from sarcoidosis

A

fibrosis

23
Q

txt for pneumoconiosis

A

none

24
Q

pulmonary hypertension: what is it and common cause?

A

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

25
Q

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

A 
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)
26
Q

pathology for pulmonary hyperT

A

normally high blood flow and low pressure, low resistance

27
Q

symptoms of pulmonary hyperT

A

fatigue, chest pain, syncope on exertion

28
Q

CXR and EKG show what for pulmonary hyperT

A

right vent. hypertrophy

29
Q

Dx for pulmonary hyperT

A

cardiac Echo

right heart cath

30
Q

why the right heart cath for pulmonary hyperT?

A

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
31
Q

txt for pulmonary hyperT

A

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

32
Q

atrial septostomy

A

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.

33
Q

cor pulmonale

A

right sided HF most often caused by COPD …

right vent. hypertrophy and HF that results from pulmonary disease/hypoxia

34
Q

labs for cor pulmonale show

A

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

35
Q

txt for cor pulmonale

A

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

36
Q

prognosis for cor pulmonale

A

bad (2-5years)

Clin Med- P (124 decks)

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