Interstitial Lung Diseases Flashcards

1
Q

Who gets Respiratory Distress syndrome (RDS)?

A

preterm infants (<37 wks)

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2
Q

Which syndrome is due to deficiency of surfactant in immature lung?

A

RDS

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3
Q

What is important to give a newborn w/ RDS?

A

-Give surfactant

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4
Q

How is RDS diagnosed?

A

_*Clinically_

  • CXR: reticulogranular ground glass appearance (interstitial pattern) w/ air bronchograms
  • must r/o other causes of resp. distress
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5
Q

How do you prevent RDS?

A

-prevent preterm birth

-give antenatal (before birth) steroids

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6
Q

How is RDS managed

A

CPAP

or

intubation + surfactant therapy + mechanical ventilation

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7
Q

The following are hallmarks of which dz?

Hypoxemia & bilateral radiographic opacities

A

Acute Respiratory Distress syndrome (ARDS)

(note- this is NOT the same as respiratory distress syndrome in newborns)

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8
Q

Which dz looks like TB but is less severe

(sxs include cough, chest discomfort, +/- hemoptysis, dyspnea, fatigue, malaise, weakness)

A

MAC pulmonary dz

(a non-TB mycobacteria infection AKA- NTM)

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9
Q

What is the name of each of the 2 major clinical presentations of MAC pulmonary dz?

  1. _______: pts w/ underlying lung dz
  2. ______: dz in pt w/o underlying lung dz
A

cavitary disease: pts w/ underlying lung dz

nodular bronchiectatic disease: dz in pt w/o underlying lung dz

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10
Q

primarily in white, middle-aged to elderly men (often alcoholics) w/ underlying COPD

A

MAC pulmonary dz in pt w/ underlying dz

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11
Q

Primarily nonsmoking women >50 who have underlying bronchiectasis

A

MAC pulmonary dz in pt w/o underlying lung dz

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12
Q

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

A

_1. *lung parenchyma is damaged_

_2. *nflammation of the alveolar walls_

_3. *Scarring (fibrosis) begins_

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13
Q
  • 2 most common sxs of ILD
  • 2 uncommon sxs of ILD
A
  • Progressive dyspnea on exertion
  • nonproductive cough

Uncommon: wheezing and CP

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14
Q

What is the most common CXR finding in interstitial lung dz

A

Reticular “netlike” opacities

**this will be on exam**

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15
Q

What is the prognosis of Interstitial lung dz (ILD) if honeycombing is seen on CXR?

A

poor prognosis

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16
Q

What do PFTs show in interstitial lung disease (ILD)

A

decreased TLC (<80%)

normal FEV1/FVC ratio (nml= 70-80%)

= restrictive

(also decreased DLCO and hypoxemia seen)

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17
Q

What is the gold standard for interstitial lung disease (ILD)?

A

Lung biopsy

(may be required for definitive diagnosis or to stage dz)

not always indicated

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18
Q

T/F: Interstitial Lung Disease (ILD) can lead to Cor pulmonale–> R heart failure?

A

True

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19
Q

What are the 4 known causes of interstitial lung diseases (ILDs)

A
  1. Occupational and environmental exposures (ex: asbestos)
  2. Drugs and poisons (ex: Chemo, radiation)
  3. Infections
  4. Idiopathic of assoc. w/ idiopathic diseases (ex: sarcoidosis)

“IDIO”

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20
Q

_______= any disease of the respiratory tract due to inhalation of dust particles

(ex: asbestosis or silicosis)

A

Pneumoconiosis

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21
Q

What condition?

  • presents 10-15 yrs after exposure
  • 40-75 y/o males
  • construction, pipefitter or shipyard worker
  • smoker
A

Asbestosis

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22
Q

What disease?

-Cancer assoc. w/ short- term (1-2 yrs) asbestos exposre

-not caused by smoking

-prognosis= 6-12 mo after presentation

A

Mesothelioma

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23
Q

What is the definitive dx of asbestosis but is not usually indicated

A

open-lung biopsy

24
Q

Which dz?

CXR- opacities in lower lungs, _pleural plaques*_

A

Asbestosis

25
What 4 things suggest diagnosis of asbestosis?
consistent history, physical findings, sxs and CXR
26
Asbestosis- what do PFTs look like?
decreased TLC FEV1/FVC ratio normal = restrictive (PFTs to follow dz progress)
27
T/F: there is no effective therapy for Asbestosis?
True. The goal is to stop progression
28
T/F: lung damage in Asbestosis is reversible
FALSE damage is IRREVERSIBLE
29
\_\_\_\_\_\_\_ + smoking= 59x more likely to get lung CA
30
What dz gives you an elevated risk for mesothelioma
Asbestosis | (b/c of asbestos exposure)
31
Which dz has **noncaseating granulomas**?
Sarcoidosis
32
**Which disease?** * multisystem inflammatory dz (unknown etiology) * 30-60 y/o African American female * noncaseating granulomas * Hilar adenopathy * elevated ACE
Sarcoidosis
33
**Which disease?** * asymptomatic to multisystem disease * (Fever, anorexia, arthralgias, DOE, cranial nerve palsies, visual disturbances, erythema nodusum, etc)
Sarcoidosis
34
Which disease shows the following on CXR depending on the stage? stage 0=normal 1- **hilar adenopathy** 2- **hilar adeopathy**+ diffuse infiltrates 3- only diffuse infiltrates 4- pulmonary fibrosis
Sarcoidosis
35
What do PFTs show in sarcoidosis?
Isolated decreased DLCO Restrictive pattern w/ advanced dz
36
**MC pattern of what in which disease?** Bilateral symmetric hilar and right paratracheal mediastinal adenopathy
MC pattern of **lymphadenopathy in sarcoidosis**
37
Labs for which dz show the following: * hypercalcemia * elevated ESR * Elevated serum protein (excess immunoglobulins) * **_elevated ACE_**
38
Diagnosis of Sarcoidosis usually requires what in most cases?
Biopsy: Fiberoptic bronchoscopy w/ transbronchial biopsy
39
How do you dx sarcoidosis (3 things)
1. compatible clinical and radiographic maifestations 2. exclusion of other diseases 3. Histopathologic- noncaseating granulomas
40
How is Sarcoidosis managed?
consult **pulmonologist** \>75% only need **NSAIDS** **Corticosteroids** if severe Monitor CXRs and sxs
41
Which dz? -immune mediated, systemic **vasculitis** **-Necrotizing granulomas** of the upper and lower resp tracts - **renal involvement** - **40-50y/o white male or female**
Granulomatosis w/ Polyangiitis
42
which disease can result in ESRD
Granulomatosis w/ Polyangiitis
43
Sxs include: -upper airway sxs MC * **purulent/bloody nasal discharge** * **oral/nasal ulcers**
Granulomatosis w/ polyangiitis
44
What do PFTs in Granulomatosis w/ Polyangiitis look like?
* **Restrictive & obstructive patterns found** * decreased DLCO
45
Are you more likely to see **hilar adenopathy** on CXR in Sarcoidosis or Granulomatosis w/ Polyangiitis?
**Sarcoidosis** (uncommon in Granulomatosis with Polyangiitis)
46
Which dz would you see **irregular and stellate-shaped** peripheral pulmonary arteries on CT?
Granulomatosis with Polyangiitis
47
Which dz has the following lab results? * **elevated ESR** * **elevated BUN/Cr**, **proteinuria** (if renal involvement) * **+ANCA** (Antineutrophil Cytoplasmic Antibodies), usually C-ANCA
Granulomatosis w/ Polyangiitis
48
Which dz has **histopathologic evidence of vasculitis and granulomatous inflammation?**
Granulomatosis w/ Polyangiitis
49
Management of which dz: 1. Refer to **Rheumatologist** * may tx w/ **immunosuppressant** (cyclophosphamide) and **glucocorticoid** what is prognosis?
**Granulomatosis w/ Polyangiitis** | (prognosis is 80% w/ cyclophosphamide)
50
Which is MC of the 7 idiopathic interstitial pneumonias?
Idiopathic Pulmonary Fibrosis
51
Which dz? * inflammation & fibrosis of lung parenchyma * **no specific findings-** dx after excluding other causes of ILD * **60 y/o male** * **smoker**
Idiopathic Pulmonary Fibrosis
52
which dz? CXR: Bilateral diffuse **_reticular or reticulonodular infiltrates_**, periphery & bases
Idiopathic pulmonary fibrosis
53
Which dz has insidious onset: **_exertional dyspnea_** and **nonproductive cough**
Idiopathic Pulmonary Fibrosis
54
What does idopathic pulmonary fibrosis show on HRCT?
reticular opacities
55
Histopathology of which dz? predominantly **fibrosis** and **scant numbers of inflammatory cells**
**Idiopathic Pulmonary Fibrosis** (this is how you distinguish from interstitial pneumonia)
56
What is the management for Idiopathic Pulmonary Fibrosis?
1. Consult p**ulmonologist** (no tx) 2. Eval for **lung transplant** (MC indication for lung transplant)
57
Possible tx of which dz? **Azithromycin (macrolide) + Ethambutol + Rifampin** usually **x15-18mo** (until sputum cultures consecutively neg for at least 12 months)
**MAC pulmonary disease** (not all patients need tx at time of dx- hard to tolerate, may decide to monitor closely instead)