Interstitial Lung Diseases Flashcards Preview

Foundations II- Pulmonary > Interstitial Lung Diseases > Flashcards

Flashcards in Interstitial Lung Diseases Deck (75):
1

respiratory disease stress is common in...

(aka hyaline membrane disease)

preterm infants

2

Cause of RDS?

deficiency of surfactant in immature lung --> makes gas exchange difficult

3

Infants with RDS usually present within...

minutes to hrs after birth, worsens over 1st 48 hrs of life

4

Signs of RDS

tachypnea, nasal flaring, expiratory grunting, retractions, cyanosis

decreased BS, pale, decreased peripheral pulses, low UOP, peripheral edema

5

Clinical course of RDS

uncomp RDS usually progressd 48-72 hrs & improves as endogenous surfactant production is increased

exogenous surfactant dramatically improves pulm func. -> sxs resolve -> shorter course

CPAP also improves clinical course

6

CXAY of pt with RDS?

low lung volume

classic reticulogranular ground glass appearance with air bronchograms

7

ABG in pt with RDS typically shows...

hypoxemia that responds to O2

as disease progresses, may see hyponatremia due to water retention

8

In RDS diagnosis is...

clinical, must distinguish from other causes

9

How can we prevent RDS?

prevent pre-term birth when possible

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

10

Management of RDS?

nasal CPAP

or Intubation + surfactant therapy + mechanical ventilation

11

How is ARDS different from RDS?

ARDS is acute respiratory distress syndrome in adults, is an acute, diffuse, inflammatory lung injury that leads to:

-decreased pulmonary vascular permeability

-increased lung weight and loss of aerated tissue-assoc. w/ high mortality rate


caused by direct or indirect injury to the lung

12

What are the 4 clinical syndromes that can be caused by Non-TB mycobacteria infections (NTM)?

1. pulmonary disease (esp. in older pts with cystic fibrosis

2. superficial lymphadenitis

3. Disseminated disease (in severely immunocompromised)

4. skin and soft tissue infection

13

Main cause of NTM lung infections?

MAC (mycobacterium avium complex)

these organisms are acquired from the environment

14

Signs/sxs of MAC pulmonary disease?

looks like TB but less severe and often indolent

-fatigue, malaise, weakness, cough, dyspnea, chest discomfort

lung exam often norm

15

MAC clinical presentation in those with underlying lung disease?

-white, middle aged or elderly men often alcoholics and/or smokers with underlying COPD

resembles TB clinically &radiographically but usu. less severe

16

MAC clinical presentation in those w/p lung disease

non smoking women >50 y/p who have interstitial pattern on CXR

17

How do you dx MAC pulmonary disease?

consistent CXR

positive sputum culture x 2

other dx have been excluded

18

What is bronchiectasis?

chronic condition where walls of the bronchi are thickened from inflammation and infection

19

Do you always need to tx MAC pulmonary disease?

NO, anti-myobacterial drugs can be dif. to tolerate

If fibrocavitary disease > TREAT

if nodular bronchiectasis > depends on presentation/status of pt

20

What abx should you give to a pt with MAC pulmonary disease?

depends on susceptibility

usually: Azithromycin + Rifampin + Ethambutol

for usually 15-18 months (tx until cultures -)

21

Interstitial lung disease aka...

pulmonary fibrosis

22

What is interstitial lung disease?

includes variety of chronic lung disorders

nonmalignant/noninfectious but serious

23

How is the lung affected in ILD?

-lung parenchyma is damaged

-walls of the alveoli become inflamed

-Scarring (fibrosis) begins in the interstitium and the lung becomes stiff

24

Causes of ILD?

diseases (sarcoidosis, neurofibromatosis), exposures (asbestos), drugs (Macrobid), Idiopathic

25

Clinical presentation of ILD?

progressive dyspnea on exertion and non productive cough

(usually NO wheezing of CP)

26

PE in ot with ILD might reveal?

crackles, inspiratory squeals, cor pulmonale

advanced disease: cyanosis, digital clubbing

extrapulmonary: ass. w/ other disease ie. erythema nodusum in sarcoidosis

27

ILD CXR?

ground-glass appearance often early finding-hazy opacity assoc. with inflammation

reticular "netlike" most common

honeycombing --> poor prognosis

28

What other diagnostic tests can you use to dx ILD?

HRCT

PFTs
- will see restrictive defect: decreased TLC, decreased FEV1 and PVC but normal FEV1/FVC ratio

ABG
-may be norm or may show hypoxemia or respiratory alkalosis

Bronchoalveolar Lavage
-minor extension of bronchoscopy, allows for cellular analysis

Lung Biopsy **

29

What is a normal FEV1/FVC ratio?

70-80%

30

If a patient has a + bronchodilator challenge this indicates...

obstructive disease (like asthma)

not restrictive disease (like ILD)

31

What is meant by V/Q mismatch?

there will be some areas of the lungs which are better perfused than ventilated and some areas that are better ventilated than perfused

32

What is diffusing capacity?

the ability of has to cross from air > interstitium > lung

may have reduced diffusing capacity of lung for carbon monoxide

33

Indications for lung biopsy?

atypical or progressive sxs

Age <50 y/o

fever, weight loss, hemoptysis

ILD sxs with norm or atypical CXR

predict prognosis before initiating therapies with serious side effects, etc.

34

Types of lung biopsies?

fiberoptic bronchoscopy with transbronchial lung biopsy- less invasive but less info

thoracoscopy

open lung biopsy

35

Complications of ILD?

pulmonary HTN, cor pulmonale, pneumothorax, elevated CA risk, progressive respiratory insufficiency

36

What are some known causes of ILD?

inorganic dust (asbestos), organic dust (bacteria), gases, fumes

chemo meds, abx (Macrobid), radiation tx

Infections

37

What is pneumoconiosis?

any disease of the respiratory tract due to inhalation of dust particles
-asbests, silicosis, coal worker's disease, berylliosis

males> females due to work hx

38

What are some occupations associated with risk of asbestosis?

construction, auto mechanics, pipe fitters, plumbers, welders, janitors, shipyard workers

39

Is mesothelioma asbestosis?

NO, it is associated with asbestos but isn't asbestosis

It is a form of CA almost always associated with asbestos exposure
-exposure can be short term
-CA develops in mesothelium
-not caused by smoking
-poor prognosis (med. survical 6-12 mo after presentation)

40

Asbestosis PE?

no specific signs/sxs

insidious onset-dyspnea, reduced exercise tolerance. usually dry cough

inspiratory crackes

41

Asbestosis dx studies?

CXR- opacities in lower lungs, thickened pleura, pleural plaques

Definitive: open lung biopsy

PFTs - restrictive pattern

histopathology shows fibrosis and asbestos bodies

42

management of asbestosis?

consult pulm-may need long term O2

smoking cessation, eliminate exposure

No effective therapy/drugs not effective

Vaccinate: flue, pneumovax

43

Classic pt with Asbestosis?

"Al asbestosis"

male

~60 y/o

construction worker

smoker

*pleural plaques

44

Course of asbestosis?

lung damage is irreversible

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

elevated risk for mesothelioma

45

What is sarcoidosis?

multi-system inflammatory disease of unknown etiology

non-caseating granulomas-predominantly in the lungs

46

What is a granuloma?

a small inflammatory nodule of macrophages-produced in response to injury/infection, boy's attempt to "wall off" something

47

besides the lungs, sarcoidosis can also affect...

heart, liver, spleen, joints, skin and bones

48

Sarcoidosis is most common in...

10x greater in African Americans

20-40 y/o

females

49

Sxs of sarcoidosis?

asymptomatic- multi-system disease
-fever, anorexia, arthralgias, DOE, dry cough, CP
-extrapulmonary: arthritis, CN palsies, erythema nodusum


pts can have spontaneous resolution, 1/3 develop chronic disease

50

CXR stages in sarcoidosis?

1: hilar adenopathy

2: hilar adenopathy + diffuse infiltrates

3: only diffuse parenchymal infiltrates

4: pulmonary fibrosis

51

PFTs in sarcoidosis?

isolated decreased in DLCO

restrictive pattern with advanced disease (decreased TLC, norm FEV/FEV1)

52

What labs would you check if you are concerned for sarcoidosis?

calcium- hypercalcemia

ESR

serum protein - excess immunoglobulins

serum ACE- elevated in 60%

53

In most causes dx of sarcoidosis requires...

biopsy- fiberoptic bronchoscopy with transbronchial biopsy

54

Classic pt with sarcoidosis?

"Sally sarcoidosis"

female, African American, 30ish, non smoker

hilar adenopathy

she's an ACE

55

How do you manage sarcoidosis?

-consult pulm

-most pts resolve spontaneously- give NSAIDS for sxs

-if severe disease --> corticosteroids (Prednisone)

-monitor: CXRS, ACE level

56

What is granulomatosis with polyangiitis?

aka Wegener's granulomatosis

immune mediated systemic vasculitis

characterized by necrotizing granulomas of the upper and lower respiratory tracts

rare

57

75% of pts with granulomatosis with polyangiitis have...

renal involvement/glomerulonephritis

sig. morbidity-> can results in ESRD

58

Sxs of granulomatosis with polyangiitis

upper airway: most common- oral/nasal ulcers, rhinorrhea, purulent/bloody nasal DC

lower airway: dyspnea, cough, pleuritis pain, hemoptysis

59

Pulmonary func. in pt with granulomatosis with polyangiitis?

restrictive and obstructive patterns found

DLCO

60

CXR in pt with granulomatosis with polyangiitis? CT?

highly variable -opacities, nodules which may cavitate

blood vessels leading to nodules & cavities, irregular and stellate-shaped peripheral pulmonary aa. "vasculitis sign"

61

Hilar adenopathy should prompt concern for...

sarcoidosis or infection (TB or fungal pna)

62

Additional dx tests for granulomatosis with polyangiitis

Labs:
-ESR
-leukocytosis, thrombocytosis, normocytic anemia
-BUN/Cr, proteinuria if renal involvement
- + ANCA (antineutrophil cytoplasmic abs)


tissue biopsy

63

classic pt with Granulomatosis with Polyangiitis?

"Gary granulomatosis with polyangiitis"

male or female

40-50ish

upper airway sxs- in the nose

Goes to AA (+ANCA Abs)

*vasculitis
*renal involvement

64

Management for granulomatosis with polyangiitis?

consult rheumatology

may tx with immunosuppressant (methotrexate or cyclophosphamide) &glucocorticoid

65

Prognosis for granulomatosis with polyangiitis

with cyclophosphamide- 80% 8 yr survival rates

66

What is idiopathic pulmonary fibrosis?

aka usual interstitial pna

most common of the 7 idiopathic interstitial pnas

inflammation & fibrosis of the lung and parenchyma

67

Dx of idiopathic pulmonary fibrosis is made..

after excluding other causes of ILD

68

IPF presentation?

insidious onset

-DOE, nonproductive cough

69

IPF PE?

inspiratory crackles

digital clubbing

70

IPF CXR?

bilateral diffuse reticular or reticulonodular infiltrates in periphery and bases

71

Additional dx tests for IPF?

HRCT- reticular opacities, honeycombing indicates poor prognosis

PFTs- restrictive pattern

lung bx

72

Histopathology of IPF?

predominantly fibrosis and scant numbers of inflammatory cells: usual interstitial pneumonitis

73

Classic pt with IPF

Ivan IPF

male
60ish
a common man (common)
smoker

74

Management of IPF?

consult pulm

eval for lung transplant- most common indication for lung transplant

smoking cessation

75

prognosis of IPF?

20-40% 5 yr survival rate at time of dx