restrictive lung disease/pulmonary hyperT Flashcards
ILD (interstitial lung disease)
aka restrictive lung disease: group of diseases characterized by diffuse lung injury and inflamm that leads to IRREVERSIBLE FIBROSIS and severely compromised gas exchange
ILD considered a “restrictive” lung disease… why ?
fibrosis and lung stiffening/noncompliance = difficult to inflate. This increases work of breathing b/c of dec. lung volume
Dx of ILD requires…
pathological dx by examination of lung tissue
& DETAILED HISTORY (enviornmental exposure)?
…to determine cause (i.e. sarcoidosis, pneumoconiosis, toxins, drugs, etc.)
most important for evidence of restrictive lung disease
PFTs : will show decreased ventilation and perfusion with much worse perfusion (high V/Q ratio)
PE for ILD
tachypnea, inspiratory “crackles”, no wheezing, clubbing (from chronic hypoxia)
PE add-ons if cause of ILD is sarcoidosis
skin lesions, gland enlargments, CN abnormalities, hepatomegaly
definitive Dx of ILD requires
biopsy
Txt for ILD
remove cause (if known), corticosteroids, O2, supportive therapy in end stages
idiopathic fibrosing interstitial pneumonia,
sarcoidosis &
pneumoconiosis are all types of …
ILDs
idiopathic fibrosing interstitial pneumonia is AKA …
idiopathic pulmonary fibrosis
txt for idiopathic pulmonary fibrosis
steroids (except if it is the UIP which needs lung transplant)
what is UIP? who does it effect?
“the bad one” - usual interstitial pneumonia
50yo +
UIP chest Xray and CT shows…
Xray: progressive fibrosis
CT: diffuse patchy fibrosis with honeycombing
(CT most useful)
overall UIP- 3 major characteristics
idiopathic, progressive fibrosis, nothing helps except transplant
sarcoidosis is …
well characterized systemic disease of uknown origin - granulomatous (inflammation) tissue in any organ system but mostly in lungs and lymphs
Dx of sarcoidosis: 4 Dx techniques and results for Dx
Chest X ray and CT: bilat hilar (roots) lymphadenopathy
lung Bx: noncaseating granulomas & serum ACE
PFTs: restrictive & obst. patterns
gallium scanning to highlight lesions
what helps the progressive dyspnea of sarcoidosis?
steroids (slows progression)
flares of sarcoidosis get progressively _____
worse
txt for sarcoidosis
none if mild, steroids to inflammation, transplant for definitive
pneumoconiosis
ILD: black lung disease
risk of developing pneumoconiosis depends on
how long pt had been around coal dust
rxn of lungs to granulomatous tissue from sarcoidosis
fibrosis
txt for pneumoconiosis
none
pulmonary hypertension: what is it and common cause?
high blood pressure of the vessels in the lungs. commonly caused by fibrosis or other lung disease
WHO new classification for pulmonary hypertension (2009) - medications are only recommended for those in group ____ but possibly harmful for those in group_____
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)
pathology for pulmonary hyperT
normally high blood flow and low pressure, low resistance
symptoms of pulmonary hyperT
fatigue, chest pain, syncope on exertion
CXR and EKG show what for pulmonary hyperT
right vent. hypertrophy
Dx for pulmonary hyperT
cardiac Echo
right heart cath
why the right heart cath for pulmonary hyperT?
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
txt for pulmonary hyperT
for those with PAH and vasoresponsiveness = CCBs
for nonresponders w/ PAH and others = epoprostenol
if secondary- txt underlying cause
lung transplant
atrial septostomy
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.
cor pulmonale
right sided HF most often caused by COPD …
right vent. hypertrophy and HF that results from pulmonary disease/hypoxia
labs for cor pulmonale show
inc RBCs (polycythemia) from hypoxic state, means more work for the heart
txt for cor pulmonale
txt like HF- txt pulmonary problem, O2, diuretics, fluid restriction
prognosis for cor pulmonale
bad (2-5years)
