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Clin Med I- PULMONARY > Restrictive Lung Dz > Flashcards

Restrictive Lung Dz Flashcards

1
Q

Normal FVC

A

80% of predicted

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2
Q

Normal FEV1

A

80% of predicted

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3
Q

Normal FEV1/FVC

A

80% of predicted

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4
Q

Extrathoracic airway obstruction is worse during…

A

inspiration because Patm is greater than Ptr so particles move inward toward trachea, worsening the obstruction

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5
Q

Intrathoracic airway obstruction is worse duirng…

A

exhalation because pleural pressure is greater than tracheal pressure

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6
Q

Low ratio, low DLCO

A

low ratio= obstructive. Low DLCO= emphysema

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7
Q

Low ratio, normal DLCO

A

Bronchitis

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8
Q

Low ratio, high DLCO

A

low ratio- obstructive. HIgh DLCO- asthma

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9
Q

Low VC, low DLCO

A

restrictive, interstitial fibrosis

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10
Q

Categories of ILD

A

ILD of known cause, Idopathic interstitial pneumonia, granulamatous ILD (sarcoidosis), and other forms of ILD

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11
Q

What abnormal breath sounds would you hear in IPF?

A

Bilateral, High pitched, fine late inspiratory crackles in lung bases

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12
Q

HRCT of IPF

A

Diffuse fibrosis in lung bases and periphery , honeycombing, cyst formation, UIP

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13
Q

UIP

A

Usual interstitial pneumonia- progressive scarring of both lungs

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14
Q

Is IPF extraparenchymal?

A

No, it is limited to the lung

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15
Q

Cause of IPF

A

Unknown

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16
Q

Risk factors for IPF

A

Smoking, family history of IPF, antidepressants, chronic aspiration, infectious agents, environmental factors

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17
Q

If you suspect someone of IPF but signs and symptoms are suggestive of systemic disorder, what to do?

A

Suggest alternate diagnosis, IPF limited to lung

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18
Q

Describe IPF’s patients cough

A

Would be nonproductive, dry

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19
Q

Is the onset of IPF rapid or slow? When does patient present?

A

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

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20
Q

PE of IPF

A

Fine, high pitched late inspiratory crackles in lung bases, cyanosis, pulmonary HTN, cor pulmonale

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21
Q

Angle in clubbing of fingers

A

More than 180 degrees

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22
Q

You suspect your patient of IPF. He has a fever. What does this tell you?

A

Fever suggestive of systemic disease process,IPF is limited to the lung. If fever, might be connective tissue disease like rheumatoid arthiritis

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23
Q

Associated symptoms with IPF

A

Weight loss, malaise, fatigue

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24
Q

What is nice about CXR of patient with IPF?

A

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

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25
Q

DDx with IPF

A

Asbestos and CTD (RA or scleodema), esp if fever associated

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26
Q

ADvantages of using CT for IPF

A

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

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27
Q

Ratio of FEV1/FVC in IPF

A

Normal or increased

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28
Q

VC, TLC, RV, and DLCO in IPF

A

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

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29
Q

Oxygen levels in IPF

A

ABG show normal or hypoxemic with respiratory alkalosis. Marked O2desaturation seen with exercise.

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30
Q

What research tool has been useful in identifying content of pulmonary airways?

A

Bronchoalveolar lavage- helpful in detecting scarring of lungs from fibrosis

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31
Q

Problem with bronchoalveolar lavage?

A

Not good for diagnosing IPF, because neutrophilia associated with underlying fibrotic process is in lots of lung conditions!

32
Q

Definitive diagnosis of IPF may require…

A

Lung biopsy

33
Q

Histopathological pattern that identifies IPF is

A

UIP- usual interstitial pneumonia

34
Q

What procedure is recommended in patients with suspected IPF and without contraindications to surgery?

A

Surgical lung bipsy- Open thoracotomy or VATS, TBB (transbronchial lung biopsy)

35
Q

In absence of surgical lung biopsy, what can be done to increase likelihood of correct CLINICAL diagnosis of IPF?

A

Presence of all the major criteria and at least 3 out of four minor criteria

36
Q

Major criteria for IPF

A

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

37
Q

Minor criteria for IPF

A

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

38
Q

IPF Tx

A

Focused on suppressing inflammatory component of IPF. Supportive care + immunosuppressive agents, antifibrotic agents, and antioxidant agents. Or lung transplantation.

39
Q

Antifibrotic meds

A

Pirfenidone, bosentan

40
Q

Antioxidant agents

A

N-acetylcysteine

41
Q

What is the preferred tx option for IPF

A

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

42
Q

Supportive care tx of IPF

A

Supplemental oxygen, smoking cessation, pulmonary rehabilitation, vaccinations, treat co-morbid conditions, opioids for dyspnea

43
Q

Most frequent cause of death in IPF

A

Respiratory failure. 5 year survival 20-30%

44
Q

New therapies for IPF

A

Immunomodulators- TNF alpha-antagonist effective in IPF animal models

45
Q

Cause of sarcoidosis

A

Unknown

46
Q

What is sarcoidosis characterized by?

A

Non-caseating granulomas

47
Q

How do sarcoidosis patients present?

A

90% pulmonary- some asymptomatic, others symptomatic with dyspnea, cough, and crackles. 30% have extrapulmonary manifestations, like hypercalcemia, cardiac dysfunctions

48
Q

PFT in sarcoidosis

A

Normal or restrictive pattern, decreased DLCO, possible obstructive component

49
Q

Stage I sarcoidosis

A

Bilateral hilar adenopathy

50
Q

Stage 2 sarcoidosis

A

Bilateral hilar adenopathy with reticular opacities

51
Q

Stage 3 sarcoidosis

A

Reticular opacities without hilar adenopathy

52
Q

Stage 4 sarcoidosis

A

reticular opacities with evidence of volume loss- upper lung SCARRING with traction bronchiectasis, cyst formation

53
Q

Nodular sarcoidosis

A

Multiple, bilateral lung NODULES with minimal hilar adenopathy

54
Q

Why is there a decrease in FVC in ILD

A

Fibrosis leads to scarring leads to lung volume loss

55
Q

What would CT scan of sarcoidosis show?

A

UPPER lung zones affected. Hilar and mediastinal lymphadenopathy, ground glass opacification, bronchial wall thickening, traction bronchiectasis

56
Q

Diagnosis of sarcoidosis

A

TBB or VATS to confirm diagnosis histologically, BAL, CXR findings, pulm and extrapulmonary clinical findings

57
Q

When to treat sarcoidosis?

A

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

58
Q

Sarcoidosis Tx

A

glucocorticoids (prednisone 6-12 months). inhaled, if pulmonary. Other immunosuppressives- methotrexate, azathioprince, TNF alpha antagonist (Infliximab), or organ transplantation

59
Q

What is infliximab

A

Immunomodular- TNF alpha antagonist used as new promising therapy for IPF, and as option to treat sarcoidosis

60
Q

Sarcoidosis prognosis

A

May have spontaneous remission in stage 1-3. (chances more likely when earlier).

61
Q

Indicators for poor prognosis

A

Age after 40, symptomatic, multisystem involvement, african descent, increasing pulmonary infiltrates

62
Q

What is a complication of advanced COPD and a possible complication of IPF?

A

pulmonary HTN!

63
Q

When to suspect pulmonary HTN?

A

When VC less than 50% of predicted or DLCO less than 45% of predicted.

64
Q

Definition of PH

A

mean pulmonary artery pressure greater than 25mmHg

65
Q

Primary cause of PH

A

Increased pulmonary vascular resistance caused by pulm vasoconstriction (from hypoxia), abnormal cell proliferation, or blood clots in vessels

66
Q

Levels of endothelin, NO, and prostacyclin in PH

A

increased endothelin (causes vasoconstriction), decreased NO (causes vasodilation), and decreased prostacyclin levels

67
Q

What severe condition can PH lead to?

A

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

68
Q

Difference between right and left sided heart failure

A

Right sides CHF- dyspnea not improved when sitting up. Left sided CHF- dyspnea IS improved by sitting up

69
Q

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?

A

PH

70
Q

Cor pulmonale

A

Impaired function of the RV due to PH caused by diseases affecting the LUNGS.

71
Q

Most common cause of cor pulmonale

A

copd

72
Q

Can cor pulmonale be caused by left sided heart failure or Congenital heart disease?

A

NO!

73
Q

XCR of PH

A

main pulmonary artery segment and central, hilar pulmonary arteries are dilated. Enlargement of right and left ventricle, and right atrium.

74
Q

PH TX

A

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

75
Q

Vasodilatin and anti-proliferation meds for PH

A

Prostacyclin analogues (EPOPROSTENOL), endothelin receptor antagonists, phophodiesterase 5 inhibitors, calcium channel blockers

76
Q

What is atrial septostomy

A

creates a right to left shunt

77
Q

PH prognosis

A

3 year survival without epoprestenol is 50%

Clin Med I- PULMONARY (7 decks)

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