Interstitial Lung Diseases Flashcards

1
Q

What population if respiratory distress syndrome common in? Why?

A

preterm infants (<37 weeks gestation)

due to deficiency of surfactant in immature lung

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2
Q

What are signs of respiratory distress syndrome? When does it present?

A
  • tachypnea
  • nasal flaring
  • expiratory grunting
  • intercostal, subcostal retractions
  • cyanosis

presents within mins to hrs after birth- progressively worsens over 1st 48 hrs of life

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3
Q

What are physical exam findings you would see in a pt w/ respiratory distress syndrome?

A
  • decreased breath sounds
  • pale w/ decreased peripheral pulses
  • urine output in 1st 48 hrs often low
  • peripheral edema common
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4
Q

What is the classic finding on CXR of a baby w/ respiratory distress syndrome?

A

Classic reticulogranular ground glass appearance w/ air bronchograms

(+ low lung volume seen)

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5
Q

How do you dx respiratory distress syndrome?

A

Diagnosis is clinical!

must distinguish RDS from other causes of respiratory distress

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6
Q

How can you prevent respiratory distress syndrome?

A
  • prevent preterm birth when possible

- Antenatal (before birth) steroids (given to all women 23-34 wks gestation at risk of preterm delivery in next 7 days)

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7
Q

How do you treat RDS?

A

CPAP
intubate
administer surfactant therapy

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8
Q

Non-TB mycobacteria infections are responsible for what? (what do they cause the most)

A

chronic lung infections (account for ~90% of pt encounters due to NTM)

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9
Q

Is MAC able to be transmitted from human-to-human or animal-to-human, like TB?

A

No

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10
Q

What are signs and sx of MAC?

A

looks like TB but less severe, often indolent

  • fatigue, malaise, weakness
  • cough-productive or dry
  • dyspnea, chest discomfort
  • fever and weight loss less common that with TB

Lung exam often nl

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11
Q

What are the 2 major clinical presentations of MAC? (how do their CXRs look)

A
  1. In those w/ underlying lung dz
    - primarily white, middle-aged or elderly men, often alcoholics and/or smokers w/ underlying COPD
    - disease resembles TB clinically and radiographically by usu. less severe
  2. In those w/o underlying lung disease
    - primarily nonsmoking women over age 50 who have interstitial pattern of CXR
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12
Q

What is required for clinical dx of MAC pulmonary dz?

A
  1. pulmonary symptoms, nodular or cavitary opacities on CXR, or high-resolution computed tomography scan that shows multifocal bronchiectasis w/ multiple small nodules

AND
2. Appropriate exclusion of other diagnoses

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13
Q

Do all pts with MAC pulmonary disease need tx?

A

No
anti-mycobacterial tx is prolonged, drugs can be difficult to tolerate, long-term response rates modest- may decide to monitor closely

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14
Q

What abx are usually given for MAC pulmonary dz? How long is it given for?

A

Azithromycin + Rifampin + Ethambutol

Continued until sputum cultures are consecutively negative for at least 12 months (so usually total 15-18 months)

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15
Q

Another name for Interstitial lung disease

A

pulmonary fibrosis

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16
Q

What are the 3 ways the lung is affected in interstitial lung dz?

A
  • lung parenchyma is damaged (by known or idiopathic cause)
  • walls of the alveoli become inflamed (bronchioles or capillaries may be involved)
  • scarring (fibrosis) begin in the interstitial (tissue between the alveoli and lung becomes stiff
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17
Q

Interstitial lung dz etiology/associations

A
  • Diseases (sarcoidosis, connective tissue dz, infections)
  • Exposures (asbestos, coal dust, aluminum)
  • Drugs (nitrofurantoin- Macrobid, amiodarone, statins, chemo meds)
  • Idiopathic (idiopathic pulmonary fibrosis, sarcoidosis)
  • Infections
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18
Q

If your pt has interstitial lung dz, what would their complaints be?

A

progressive dyspnea on exertion and nonproductive cough

wheezing and chest pain are uncommon!!

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19
Q

PE findings for a pt w/ interstitial lung dz

A
  • crackles
  • inspiratory squeaks
  • cor pulmonale
  • cyanosis
  • digital clubbing
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20
Q

What are 3 things that could be seen on a CXR of a pt with interstitial lung dz?

A
  • ground glass appearance often early finding (hazy opacity, assoc. w/ inflammation)
  • reticular “netlike” (MOST COMMON), nodular, or mixed pattern of opacities (BILATERAL)
  • honeycombing (small cystic spaces) indicates poor prognosis
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21
Q

What defect pattern do most interstitial lung disease show on pulmonary function tests?
How are TLC, FEV1, FVC, and FEV1/FVC ratio changed?

A

Restrictive defect

  • decreased: total lung capacity
  • decreased flow rates (FEV1 and FVC) but changes proportional to diminished lung volumes, so FEV1/FVC ratio is nl or increased
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22
Q

What is FVC?

A

Forced vital capacity= amount of air forcefully exhaled after max inspiration

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23
Q

In Interstitial lung dz, what happens to DLCO? what is it/what does it mean

A

REDUCED diffusing capacity of lung for carbon monoxide (DLCO)- common in interstitial lung dz

-diffusing capacity= ability of gas to cross from air to interstitial to blood

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24
Q

What is the gold standard for dx of Interstitial lung dz? When would it be performed?

A

Lung biopsy

  • provide specific dx
  • assess disease activity
  • exclude neoplastic or infectious process
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25
Q

Complications of Interstitial lung dz

A
  • pulmonary htn -> cor pulmonale -> R heart failure
  • PTX
  • elevated CA risk
  • Progressive respiratory insufficiency
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26
Q

What is Pneumoconiosis?

A

any dz of the respiratory tract due to inhalation of dust particles (asbestosis, silicosis, coal worker’s dz, berylliosis)

27
Q

Asbestos and relation to Asbestosis.

  • how long of exposure?
  • more in males or females?
  • used extensively until when?
A

10-15 years of exposure

Males > females (due to work hx)

until 1960s

28
Q

What is Mesothelioma and how does it differ from Asbestosis?

A

A form of CA almost always a/w asbestos exposure

-exposure 1-2 yrs
vs asbestosis which is 10-15 yrs
-poor prognosis

29
Q

What sign/sx would a pt show if they have Asbestosis?

A

-no specific sign/sx

  • insidious onset
  • dyspnea, reduced exercise tolerance
  • dry cough
  • inspiratory crackles
  • sometimes digital clubbing
30
Q

On CXR, what finding would you see in a pt with Asbestosis?

A
  • Opacities in lower lungs
  • Thickened pleura
  • Pleural plaques
31
Q

What pattern do PFTs show in Asbestosis?

A

Restrictive pattern

  • decreased TLC
  • FEV1/FVC ratio nl
32
Q

What do you do for a pt with Asbestosis? (management)

What is the goal?

A
  • consult pulmonology
  • smoking cessation
  • eliminate exposure
  • no effective therapy

Goal: eliminate progression of sx, reduce risk of other lung disorders

33
Q

“Al Asbestosis”

A

male
60ish
construction worker
smoker- making things worse

pleural plaques on CXR

34
Q

Prognosis of damage in Asbestosis

What will smoking do?

A

lung damage is irreversible

smoking increases risk of lung CA 59x

35
Q

What is sarcoidosis? what is the cause?

A

Multisystem inflammatory disease

unknown etiology

36
Q

What is characteristic of Sarcoidosis?

A

Noncaseating granulomas- predominantly in the lungs (noncaseating= no necrosis)

37
Q

Who is most at risk for Sarcoidosis?

A
  • 10x greater in African American
  • peak in 20-40 yr olds
  • slight female predominance
38
Q

Of the pt’s who have sarcoidosis, how many resolve/how many develop chronic dz?

A

2/3 resolve spontaneously

1/3 develop chronic dz

39
Q

What complaints are associated with sarcoidosis?

A
  • 45% have systemic complaints (fever, anorexia, arthralgias)
  • Pulmonary- dyspnea on exertion, cough, CP
  • Extrapulmonary (arthritis, cranial nerve palsies, visual disturbances, erythema nodusum)
40
Q

What are the different stages of Sarcoidosis seen on CXR?

A

Stage 0= normal

Stage 1= hilar adenopathy

Stage 2= hilar adenopathy + diffuse infiltrates

Stage 3= only diffuse parenchymal infiltrates

Stage 4= pulmonary fibrosis

41
Q

What would you see on PFTs in a pt with Sarcoidosis?

A
  • Isolated decreased DLCO

- Restrictive pattern w/ advanced dz

42
Q

What is the most common pattern of lymphadenopathy in Sarcoidosis on CXR?

A

Bilateral symmetric hilar and R paratracheal mediastinal adenopathy

43
Q

What 4 lab studies would be elevated in a pt with Sarcoidosis?

A
  • Hypercalcemia
  • increased ESR
  • increased protein (excess immunoglobulins)
  • increased serum ACE in 60%
44
Q

Dx of Sarcoidosis requires what in most cases?

A

Biopsy

45
Q

Sally Sarcoidosis

A

Female
African American
30ish
non-smoker

  • hilar adenopathy
  • She’s and ACE
46
Q

Sarcoidosis management:
What do you do initially?
For non severe dz?
For severe?

A
  • Consult Pulmonologist
  • NSAIDs (75% require only symptomatic tx)
  • Corticosteroids= mainstay of therapy for severe dz
47
Q

What is Granulomatosis w/ Polyangitis (aka Wegener’s Granulomatosis)?
How is it characterized?

A

immune-mediated, systemic vasculitis

characterized by necrotizing granulomas of the upper and lower respiratory tracts

48
Q

Besides the lungs, what other organ sx is usually involved with Granulomatosis w/ Polyangitis?

A

75% have renal involvement/ glomerulonephitis

49
Q

Who has highest prevalence of Granulomatosis w/ Polyangitis?

A

40-50s
White
Men= women

50
Q

What symptoms are most common in pts with Granulomatosis w/ Polyangitis?

A

Upper airway sx!= most common

  • rhinorrhea, purulent/bloody nasal discharge
  • oral/ nasal ulcers

Lower airway sx:
-dyspnea, cough, pleuritic pain, hemoptysis

51
Q

What would you expect in PFTs of a pt with Granulomatosis w/ Polyangitis?

A
  • Restrictive and obstructive patterns found
  • Decreased DLCO
  • Decreased lung volumes when diffuse interstitial involvement present
52
Q

What would you see on CXR of a pt with Granulomatosis w/ Polyangitis?

A

CXR: usu. abnormal but highly variable

  • opacities
  • nodules which can cavitate
53
Q

In Granulomatosis w/ Polyangitis, what sign is seen on CT?

what else?

A

“Vasculitis sign”= irregular and stellate-shaped peripheral pulmonary arteries

Also, blood vessels leading to nodules and cavities (feeding vessels)

54
Q

What lab test would be positive that is more specific for Granulomatosis w/ Polyangitis?

A

+ ANCA (Antineutrophil cytoplasmic antibodies)

55
Q

“Gary Granulomatosis w/ Polyangitis”

A

Male or female
40-50
Upper airway sx (chest the nose!)
Goes to AA (+ ANCA antibodies)

  • Vasculitis sign
  • renal involvement
56
Q

What is done for management of pts with Granulomatosis w/ Polyangitis?

A

Consult Rheumatology

-May tx w/ immunosuppressant (methotrexate or cyclophosphamide) and glucocorticoid

57
Q

What is another name for Idiopathic Pulmonary Fibrosis?

Is it common?

A

Unusual interstitial pneumonia

Most common of the 4 idiopathic interstitial pneumonias

58
Q

What is Idiopathic Pulmonary Fibrosis characterized by?

Most at risk?

A

inflammation and fibrosis of lung parenchyma

~60 yrs
Slight male predominance
70% smokers
rare familial cases

59
Q

How do you diagnose Idiopathic Pulmonary Fibrosis? Are there specific findings?

A

No specific findings

Diagnosis of exclusion

60
Q

How does Idiopathic Pulmonary Fibrosis present symptomatically (most common sx/sudden or progressive)?

PE?

A

Insidious onset

  • exertional dyspnea= most common!
  • nonproductive cough

PE:
inspiratory crackles
digital clubbing (20-50%)

61
Q

In Idiopathic Pulmonary Fibrosis, what would you see on CXR?

A

Bilateral diffuse reticular or reticulonodular infiltrates, periphery and bases

62
Q

Besides CXR, what other studies could you do to help dx? What would they show

A

HRCT (patchy infiltrates, reticular opacities, honeycombing- poor prognosis)

PFTs- restrictive pattern

63
Q

“Ivan IPF”

A

male
60ish
a common man
smoker

64
Q

What is the most common indication for lung transplant?

A

Idiopathic Pulmonary Fibrosis