Interstitial Lung Diseases Flashcards

(75 cards)

1
Q

respiratory disease stress is common in…

A

(aka hyaline membrane disease)

preterm infants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Cause of RDS?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Infants with RDS usually present within…

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Signs of RDS

A

tachypnea, nasal flaring, expiratory grunting, retractions, cyanosis

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Clinical course of RDS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

CXAY of pt with RDS?

A

low lung volume

classic reticulogranular ground glass appearance with air bronchograms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

ABG in pt with RDS typically shows…

A

hypoxemia that responds to O2

as disease progresses, may see hyponatremia due to water retention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

In RDS diagnosis is…

A

clinical, must distinguish from other causes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How can we prevent RDS?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Management of RDS?

A

nasal CPAP

or Intubation + surfactant therapy + mechanical ventilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How is ARDS different from RDS?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A
  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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Main cause of NTM lung infections?

A

MAC (mycobacterium avium complex)

these organisms are acquired from the environment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Signs/sxs of MAC pulmonary disease?

A

looks like TB but less severe and often indolent

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

lung exam often norm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

MAC clinical presentation in those with underlying lung disease?

A

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

resembles TB clinically &radiographically but usu. less severe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MAC clinical presentation in those w/p lung disease

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How do you dx MAC pulmonary disease?

A

consistent CXR

positive sputum culture x 2

other dx have been excluded

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is bronchiectasis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Do you always need to tx MAC pulmonary disease?

A

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

If fibrocavitary disease > TREAT

if nodular bronchiectasis > depends on presentation/status of pt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A

depends on susceptibility

usually: Azithromycin + Rifampin + Ethambutol

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Interstitial lung disease aka…

A

pulmonary fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is interstitial lung disease?

A

includes variety of chronic lung disorders

nonmalignant/noninfectious but serious

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

How is the lung affected in ILD?

A
  • lung parenchyma is damaged
  • walls of the alveoli become inflamed
  • Scarring (fibrosis) begins in the interstitium and the lung becomes stiff
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Causes of ILD?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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