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Flashcards in Interstitial Lung Diseases Deck (57)
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1
Q

Who gets Respiratory Distress syndrome (RDS)?

A

preterm infants (<37 wks)

2
Q

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

A

RDS

3
Q

What is important to give a newborn w/ RDS?

A

-Give surfactant

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
5
Q

How do you prevent RDS?

A

-prevent preterm birth

-give antenatal (before birth) steroids

6
Q

How is RDS managed

A

CPAP

or

intubation + surfactant therapy + mechanical ventilation

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)

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)

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

10
Q

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

A

MAC pulmonary dz in pt w/ underlying dz

11
Q

Primarily nonsmoking women >50 who have underlying bronchiectasis

A

MAC pulmonary dz in pt w/o underlying lung dz

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_

13
Q

Clinical presentation of which disease?

A

Progressive dyspnea on exertion and nonproductive cough

(wheezing and CP uncommon)

14
Q

What is the most common CXR finding in interstitial lung dz

A

Reticular “netlike” opacities

**this will be on exam**

15
Q

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

A

poor prognosis

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)

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

18
Q

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

A

True

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”

20
Q

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

(ex: asbestosis or silicosis)

A

Pneumoconiosis

21
Q

What condition?

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

Asbestosis

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

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
Q

What 4 things suggest diagnosis of asbestosis?

A

consistent history, physical findings, sxs and CXR

26
Q

Asbestosis- what do PFTs look like?

A

decreased TLC
FEV1/FVC ratio normal

= restrictive

(PFTs to follow dz progress)

27
Q

T/F: there is no effective therapy for Asbestosis?

A

True. The goal is to stop progression

28
Q

T/F: lung damage in Asbestosis is reversible

A

FALSE

damage is IRREVERSIBLE

29
Q

_______ + smoking= 59x more likely to get lung CA

A
30
Q

What dz gives you an elevated risk for mesothelioma

A

Asbestosis

(b/c of asbestos exposure)

31
Q

Which dz has noncaseating granulomas?

A

Sarcoidosis

32
Q

Which disease?

  • multisystem inflammatory dz (unknown etiology)
  • 30-60 y/o African American female
  • noncaseating granulomas
  • Hilar adenopathy
  • elevated ACE
A

Sarcoidosis

33
Q

Which dz?

  • asymptomatic to multisystem dz
  • (F, anorexia, arthralgias, dyspnea on exertion, cranial nerve palsies, erythema nodusum, etc)
  • can have spont. resolution, 1/3 develop chronic dz
A

Sarcoidosis

34
Q

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

A

Sarcoidosis

35
Q

What do PFTs show in sarcoidosis?

A

Isolated decreased DLCO

Restrictive pattern w/ advanced dz

36
Q

MC pattern of what in which disease?

Bilateral symmetric hilar and right paratracheal mediastinal adenopathy

A

MC pattern of lymphadenopathy in sarcoidosis

37
Q

Labs for which dz show the following:

  • hypercalcemia
  • elevated ESR
  • Elevated serum protein (excess immunoglobulins)
  • elevated ACE
A
38
Q

Diagnosis of Sarcoidosis usually requires what in most cases?

A

Biopsy:

Fiberoptic bronchoscopy w/ transbronchial biopsy

39
Q

How do you dx sarcoidosis (3 things)

A
  1. compatible clinical and radiographic maifestations
  2. exclusion of other diseases
  3. Histopathologic- noncaseating granulomas
40
Q

How is Sarcoidosis managed?

A

consult pulmonologist

>75% only need NSAIDS

Corticosteroids if severe

Monitor CXRs and sxs

41
Q

Which dz?

-immune mediated, systemic vasculitis

-Necrotizing granulomas of the upper and lower resp tracts

  • renal involvement
  • 40-50y/o white male or female
A

Granulomatosis w/ Polyangiitis

42
Q

which disease can result in ESRD

A

Granulomatosis w/ Polyangiitis

43
Q

Sxs include:

-upper airway sxs MC

  • purulent/bloody nasal discharge
  • oral/nasal ulcers
A

Granulomatosis w/ polyangiitis

44
Q

What do PFTs in Granulomatosis w/ Polyangiitis look like?

A
  • Restrictive & obstructive patterns found
  • decreased DLCO
45
Q

Are you more likely to see hilar adenopathy on CXR in Sarcoidosis or Granulomatosis w/ Polyangiitis?

A

Sarcoidosis

(uncommon in Granulomatosis with Polyangiitis)

46
Q

Which dz would you see irregular and stellate-shaped peripheral pulmonary arteries on CT?

A

Granulomatosis with Polyangiitis

47
Q

Which dz has the following lab results?

  • elevated ESR
  • elevated BUN/Cr, proteinuria (if renal involvement)
  • +ANCA (Antineutrophil Cytoplasmic Antibodies), usually C-ANCA
A

Granulomatosis w/ Polyangiitis

48
Q

Which dz has histopathologic evidence of vasculitis and granulomatous inflammation?

A

Granulomatosis w/ Polyangiitis

49
Q

Management of which dz:

  1. Refer to Rheumatologist
    • may tx w/ immunosuppressant (cyclophosphamide) and glucocorticoid

what is prognosis?

A

Granulomatosis w/ Polyangiitis

(prognosis is 80% w/ cyclophosphamide)

50
Q

Which is MC of the 7 idiopathic interstitial pneumonias?

A

Idiopathic Pulmonary Fibrosis

51
Q

Which dz?

  • inflammation & fibrosis of lung parenchyma
  • no specific findings- dx after excluding other causes of ILD
  • 60 y/o male
  • smoker
A

Idiopathic Pulmonary Fibrosis

52
Q

which dz?

CXR: Bilateral diffuse reticular or reticulonodular infiltrates, periphery & bases

A

Idiopathic pulmonary fibrosis

53
Q

Which dz has insidious onset: exertional dyspnea and nonproductive cough

A

Idiopathic Pulmonary Fibrosis

54
Q

What does idopathic pulmonary fibrosis show on HRCT?

A

reticular opacities

55
Q

Histopathology of which dz?

predominantly fibrosis and scant numbers of inflammatory cells

A

Idiopathic Pulmonary Fibrosis

(this is how you distinguish from interstitial pneumonia)

56
Q

What is the management for Idiopathic Pulmonary Fibrosis?

A
  1. Consult pulmonologist (no tx)
  2. Eval for lung transplant (MC indication for lung transplant)
57
Q

Possible tx of which dz?

Azithromycin (macrolide) + Ethambutol + Rifampin

usually x15-18mo (until sputum cultures consecutively neg for at least 12 months)

A

MAC pulmonary disease

(not all patients need tx at time of dx- hard to tolerate, may decide to monitor closely instead)