Restrictive Lung Dz Flashcards
Normal FVC
80% of predicted
Normal FEV1
80% of predicted
Normal FEV1/FVC
80% of predicted
Extrathoracic airway obstruction is worse during…
inspiration because Patm is greater than Ptr so particles move inward toward trachea, worsening the obstruction
Intrathoracic airway obstruction is worse duirng…
exhalation because pleural pressure is greater than tracheal pressure
Low ratio, low DLCO
low ratio= obstructive. Low DLCO= emphysema
Low ratio, normal DLCO
Bronchitis
Low ratio, high DLCO
low ratio- obstructive. HIgh DLCO- asthma
Low VC, low DLCO
restrictive, interstitial fibrosis
Categories of ILD
ILD of known cause, Idopathic interstitial pneumonia, granulamatous ILD (sarcoidosis), and other forms of ILD
What abnormal breath sounds would you hear in IPF?
Bilateral, High pitched, fine late inspiratory crackles in lung bases
HRCT of IPF
Diffuse fibrosis in lung bases and periphery , honeycombing, cyst formation, UIP
UIP
Usual interstitial pneumonia- progressive scarring of both lungs
Is IPF extraparenchymal?
No, it is limited to the lung
Cause of IPF
Unknown
Risk factors for IPF
Smoking, family history of IPF, antidepressants, chronic aspiration, infectious agents, environmental factors
If you suspect someone of IPF but signs and symptoms are suggestive of systemic disorder, what to do?
Suggest alternate diagnosis, IPF limited to lung
Describe IPF’s patients cough
Would be nonproductive, dry
Is the onset of IPF rapid or slow? When does patient present?
Insidious- GRADUAL onset of nonproductive cough and dyspnea. patient presents late in course of disease. Usually will present with dyspnea which is progressive and present for more than 6 months before presentation
PE of IPF
Fine, high pitched late inspiratory crackles in lung bases, cyanosis, pulmonary HTN, cor pulmonale
Angle in clubbing of fingers
More than 180 degrees
You suspect your patient of IPF. He has a fever. What does this tell you?
Fever suggestive of systemic disease process,IPF is limited to the lung. If fever, might be connective tissue disease like rheumatoid arthiritis
Associated symptoms with IPF
Weight loss, malaise, fatigue
What is nice about CXR of patient with IPF?
Almost all will have abnormal CXR! Makes diagnosis a bit easier- bilateral basilar interstitial infiltrates, decreased lung volume (in sync with restrictive lung dz), honeycombing, subpleural cysts, traction bronchiectasis (stretchy walls). CT shows reticular, patchy abnormalities and GROUND GLASS opacities
DDx with IPF
Asbestos and CTD (RA or scleodema), esp if fever associated
ADvantages of using CT for IPF
Increases level of diagnostic confidence for IPF, helps to narrow DDx based on CT pattern, allows earlier diagnosis of IPF, allows evaluation of extent of associated emphysema
Ratio of FEV1/FVC in IPF
Normal or increased
VC, TLC, RV, and DLCO in IPF
Reduced VC, TLC, and RV because of fibrosis causing stiffning of lungs- can’t get as much air in and out. DLCO reduced- fibrosis getting in the way of proper gas exchange from alveoli to capillaries
Oxygen levels in IPF
ABG show normal or hypoxemic with respiratory alkalosis. Marked O2desaturation seen with exercise.
What research tool has been useful in identifying content of pulmonary airways?
Bronchoalveolar lavage- helpful in detecting scarring of lungs from fibrosis
Problem with bronchoalveolar lavage?
Not good for diagnosing IPF, because neutrophilia associated with underlying fibrotic process is in lots of lung conditions!
Definitive diagnosis of IPF may require…
Lung biopsy
Histopathological pattern that identifies IPF is
UIP- usual interstitial pneumonia
What procedure is recommended in patients with suspected IPF and without contraindications to surgery?
Surgical lung bipsy- Open thoracotomy or VATS, TBB (transbronchial lung biopsy)
In absence of surgical lung biopsy, what can be done to increase likelihood of correct CLINICAL diagnosis of IPF?
Presence of all the major criteria and at least 3 out of four minor criteria
Major criteria for IPF
Cancel out other DDx! 1. Exclusion of other known causes of ILD like drug toxicities, environmental exposures. 2. TBB or BAL showing no features to suggest alternate diagnosis. 3. PFT with evidence of restriction- reduced VC, increased ratio, impaired gas exchange shown by decreased DLCO or increased AaPO2. 4. HRCT- bibasilar reticular abnormalities with minimal ground glass opacities
Minor criteria for IPF
Age more than 50, insidious onset of otherwise unexplained dyspnea on exertion, duration of illness for more than 3 months at least, and bibasilar, inspiratory crackles heard on auscultation
IPF Tx
Focused on suppressing inflammatory component of IPF. Supportive care + immunosuppressive agents, antifibrotic agents, and antioxidant agents. Or lung transplantation.
Antifibrotic meds
Pirfenidone, bosentan
Antioxidant agents
N-acetylcysteine
What is the preferred tx option for IPF
Lung transplantaion- bilateral. 5 year survival of 40-50%. preferred because only a minority of patients respond to therapy, and spontaneous remissions do NOT occur
Supportive care tx of IPF
Supplemental oxygen, smoking cessation, pulmonary rehabilitation, vaccinations, treat co-morbid conditions, opioids for dyspnea
Most frequent cause of death in IPF
Respiratory failure. 5 year survival 20-30%
New therapies for IPF
Immunomodulators- TNF alpha-antagonist effective in IPF animal models
Cause of sarcoidosis
Unknown
What is sarcoidosis characterized by?
Non-caseating granulomas
How do sarcoidosis patients present?
90% pulmonary- some asymptomatic, others symptomatic with dyspnea, cough, and crackles. 30% have extrapulmonary manifestations, like hypercalcemia, cardiac dysfunctions
PFT in sarcoidosis
Normal or restrictive pattern, decreased DLCO, possible obstructive component
Stage I sarcoidosis
Bilateral hilar adenopathy
Stage 2 sarcoidosis
Bilateral hilar adenopathy with reticular opacities
Stage 3 sarcoidosis
Reticular opacities without hilar adenopathy
Stage 4 sarcoidosis
reticular opacities with evidence of volume loss- upper lung SCARRING with traction bronchiectasis, cyst formation
Nodular sarcoidosis
Multiple, bilateral lung NODULES with minimal hilar adenopathy
Why is there a decrease in FVC in ILD
Fibrosis leads to scarring leads to lung volume loss
What would CT scan of sarcoidosis show?
UPPER lung zones affected. Hilar and mediastinal lymphadenopathy, ground glass opacification, bronchial wall thickening, traction bronchiectasis
Diagnosis of sarcoidosis
TBB or VATS to confirm diagnosis histologically, BAL, CXR findings, pulm and extrapulmonary clinical findings
When to treat sarcoidosis?
If asymptomatic- watchful observation. If pulm symptoms, PFT, or CXR symptoms getting worse 2. severe ocular, neurologic, or cardiac symtoms, 3. severe hypercalcemia. 4. symptomatic stage 2, 3, or 4 pulm disease TREAT
Sarcoidosis Tx
glucocorticoids (prednisone 6-12 months). inhaled, if pulmonary. Other immunosuppressives- methotrexate, azathioprince, TNF alpha antagonist (Infliximab), or organ transplantation
What is infliximab
Immunomodular- TNF alpha antagonist used as new promising therapy for IPF, and as option to treat sarcoidosis
Sarcoidosis prognosis
May have spontaneous remission in stage 1-3. (chances more likely when earlier).
Indicators for poor prognosis
Age after 40, symptomatic, multisystem involvement, african descent, increasing pulmonary infiltrates
What is a complication of advanced COPD and a possible complication of IPF?
pulmonary HTN!
When to suspect pulmonary HTN?
When VC less than 50% of predicted or DLCO less than 45% of predicted.
Definition of PH
mean pulmonary artery pressure greater than 25mmHg
Primary cause of PH
Increased pulmonary vascular resistance caused by pulm vasoconstriction (from hypoxia), abnormal cell proliferation, or blood clots in vessels
Levels of endothelin, NO, and prostacyclin in PH
increased endothelin (causes vasoconstriction), decreased NO (causes vasodilation), and decreased prostacyclin levels
What severe condition can PH lead to?
Right heart failure- RV hypertrophies- trying to push blood to lungs, but pulmonary artery pressure is so high. RV dilates, leading to decreased cardiac output
Difference between right and left sided heart failure
Right sides CHF- dyspnea not improved when sitting up. Left sided CHF- dyspnea IS improved by sitting up
Patient presents with exertional dyspnea, tachypnea, fatigue, chest pain, RUQ abdominal pain, hoarseness. Clubbing and cor pulmonale. increased intensity of pulmonic component of the second heart sound. Systolic ejection murmur. Diagnosis?
PH
Cor pulmonale
Impaired function of the RV due to PH caused by diseases affecting the LUNGS.
Most common cause of cor pulmonale
copd
Can cor pulmonale be caused by left sided heart failure or Congenital heart disease?
NO!
XCR of PH
main pulmonary artery segment and central, hilar pulmonary arteries are dilated. Enlargement of right and left ventricle, and right atrium.
PH TX
Treat underlying cause, oxygen if hypoxemic, salt and fluid restriction, exercise program, anticoag if PE. Advanced therapy- meds for vasodilation and anti-proliferation, atrial septostomy, transplantation
Vasodilatin and anti-proliferation meds for PH
Prostacyclin analogues (EPOPROSTENOL), endothelin receptor antagonists, phophodiesterase 5 inhibitors, calcium channel blockers
What is atrial septostomy
creates a right to left shunt
PH prognosis
3 year survival without epoprestenol is 50%
