Restrictive and Inflammatory Lung Disease

Question 1

Restrictive physiology is defined by ____

TLC

FRC (TGV)

FEV1 and FVC

Answer 1

reduced lung volumes

TLC < 80% predicted

FRC < 80% predicted

FEV1 and FVC symm reduced

Question 2

Restrictive physiology may be caused by either (2)

Answer 2

1) incr in lung elastic recoil (stiffness)
2) chest wall or pleural disease

Question 3

For PV curve of lung, ___ does not affect the slope of this curve

Answer 3

Chest wall changes

Question 4

Relationship between compliance and elastance

Answer 4

Inverse

Compliance (dV/dP)

Elastance (dP/dv)

Question 5

Compare obstructive vs. restrictive in terms of compliance

Answer 5

Obstructive = incr compliance (incr volume at given pressure) = hard to exhale

Restrictive = decr compliance (decr volume at given pressure) = hard to inhale

Question 6

What is work of breathing?

Answer 6

Combined elastic + resistive work

Ptot = Pel + Pr

Pr = resistive work against airflow

Pel = work against elastic recoil

Question 7

When would you have to work harder to expand your lungs (2)

Answer 7

1) incr lung elastic recoil
2) decr chest wall compliance

Question 8

3 mechanisms that decr lung compliance (restrictive disease)

Answer 8

1) incr thickness of lung interstitium= inflammation or scarring may expand interstitium
2) incr lung water
3) incr alveolar surface tension

Question 9

Mechanism of incr thickness of lung interstitium in restrictive disease

example of disease

Answer 9

chronic interstitial lung disease

1) incr deposition of elastic/connective tissue
2) fibroblasts produce XS collagen/elastin in alveoli
3) incr interstitial matrix (incr elastic recoil and stiffens lung)

Question 10

Mechanism of incr lung water

example of disease

Answer 10

Congestive heart failure, cardiogenic or noncardiogenic pulm edema (incr lung water)

1) fluid leaves capillary and fills interstitium (incr elasticity)
2) fluid fills alveoli, disrupt surfactant, incr surface tension
3) collapses alveoli, decr compliance

Question 11

Define respiratory distress syndrome in pediatric vs. adult

Answer 11

pediatric = immature lungs –> inadequate surfactant production

adults = ARDS = injury of type 2 alveolar cells –> noncardiogenic pulm edema that dilutes surfactant

Question 12

how does restrictive lung disease affect TLC, FRC and RV

how does it affect PV curve

how does it affect FEV1/FVC ratio

Answer 12

Decr all

Shift down PV curve

normal or incr FEV1/FVC

Question 13

how do you measure static compliance in mechanically ventilated patients?

Answer 13

measure pressure to inflate lung to certain volume

Question 14

How does restrictive disease affect gas exchange

Answer 14

Decr DLCO/gas exchange

1) decr alveolar surface area, decr lung volumes
2) incr diffusion distance, exacerbated with exercise
3) V/Q mismatch

Question 15

Describe traction bronchiectasisi and effect on Flow rate vs. volume curve

Answer 15

Supranormal airflow for given lung volume due to traction applied from adjacent parenchyma to keep airways dilated

Question 16

How does pulmonary edema incr elastic recoil?

Answer 16

1) interstitial pulm edema = incr mass and stiffens interstitium
2) alveolar pulmonary edema - deplete surfactant, incr mass and stiffness of interstitium
3) surfactant depletion - incr alveolar surface tension - alveolar collapse (atelectasis)
4) alveolar filling process (pneumonia, alveolar hemorrhage)

Question 17

What should you be aware of when determining DLCO with VA?

Answer 17

DLCO corrected for VA may miss interstitial lung disease because many interstitial diseases decr both VA and DLCO

Question 18

Causes of increased lung water that decreases lung compliance

Answer 18

1) ARDS
2) pneumonia
3) cardiogenic pulm edema

Question 19

Diseases that affect chest wall compliance

Answer 19

1) Burns- thick eschar
2) obesity- incr soft tissue mass, decr compliance
3) kyphoscoliosis- deformity of spine
4) ankylosing spondylitis - ossification of liagments in spine
5) respiratory muscle weakness- paralysis of muscles
6) pleural fibrosis/thickening- restrict expansion of lung
7) pleural effusion = fluid in pleural cavity limit lung expansion

Question 20

For mild reduction in lung volumes, what does DLCO and VA look like?

Answer 20

DLCO decreases but DLCO is corrected for alveolar volume (VA)

Question 21

How does FRC change in people with muscular weakness?

Answer 21

FRC is normal (low TLC and incr RV)

Question 22

how can you distinguish between chest wall and muscular disease from restrictive lung diseases (fibrosis/edema)?

Answer 22

chest wall and muscular disease = slope of P-V is normal and DLCO/VA is normal and decr lung volume (TLC) so shifted down but same shape

restrictive disease = slope is decreased and DLCO/VA does not correct

Question 24

compare gas exchange in chest wall diseases vs. restrictive lung diseases

Answer 24

chest wall diseases = no gas exchange problem (DLCO decr but DLCO/VA is normal)

restrictive lung disease = loss of alveolar surface area, (both DLCO and DLCO/VA is decreased)

Question 25

What are examples of acute restrictive proceses?

Answer 25

1) pulm edema
2) ARDS
3) Pneumonia
4) Pleural effusion

Question 26

What are examples of chronic restrictive proceses?

Answer 26

1) ILD
2) Pleural fibrosis/plaques
3) Pleural Effusion

Question 27

Describe mixed obstructive and restrictive disease

Types of patients who have mixed obstructive and restrictive

Answer 27

Decreased lung volumes AND dcr FEV1/FVC < 0.70

•coal miner who smokes

construction worker with interstitial lung disease from asbestos or silica exposure who also has a1 antitrypsin deficiency
•obese person with alpha 1 anti-trypsin deficiency

obese person with asthma

• Combined pulmonary fibrosis and emphysema
• Fibrotic hypersensitivity pneumonitis
• Sarcoidosis

Question 28

how does mixed disease appear on PFT’s?

Answer 28

1) decr TLC or FRC (restrictive)
2) decr FEV1/FVC (obstructive)
3) decr DLCO

Question 29

Define interstitial lung disease (diffuse parenchymal lung disease)

classified by etiology and pattern

Answer 29

combined inflammation + scarring

classified by etiology and pattern

Question 30

Causes of ILD can be grouped into 5 groups

Answer 30

1) Autoimmune
2) Exposure to inorganic dusts (asbestos/silica)
3) Exposure to organic molecules –> hypersensitivity pneumonitis
4) Drug effects
5) idiopathic

Question 31

Breakdown of Interstitial lung diseases

Answer 31

1) ILD due to known cause
2) idiopathic interstitial pneumonia
a) IPF
b) not IPF
- DIP, AIP, NSIP, COP, LIP
3) granulomatous (sarcoidosis)

Question 32

Most common symptoms of ILD

Rarely, this disease is ____

Answer 32

1) insidious onset of dyspnea on exertion
2) nonproductive cough

rarely, sudden onset unless acute hypersensitivity pneumonitis, acute interstitial pneumonitis, acute eosinophilic pneumonia

Question 33

How do PFTs look for ILD

Answer 33

restrictive pattern (normal or incr FEV1/FVC, decr TLC and TGV) with decr DLCO

Question 34

Different causes of ILD

Answer 34

Question 35

For prognosis of ILD, what disease is most significantly causing decr survival?

Answer 35

UIP

Question 36

Physical exam in ILD

Answer 36

1) Auscultation = end inspiratory crackles
2) Digital clubbing with advanced
3) Joint and Skin exam (Gottron’s papules, RA, mechanic’s hands)

Question 37

Treatment optiosn for ILD

Answer 37

1) Remove offending exposure
2) immunosuppressive (corticosteroids and steroid sparing drugs)
3) O2
4) transplantation

Question 38

More specifically, what is treatment for IPF?

Answer 38

Nintedanib, pirfenidone

Question 39

Idiopathic Pulmonary Fibrosis

1) Define
2) Etiology
3) Age of patients
4) Associated with?
5) Symptoms
6) Physical exam

Answer 39

1) scarring lung disease with pattern of UIP
2) idiopathic
3) 60 + y/o
4) tobacco use
5) Cough, DOE, fatigue
6) basilar predominant “velcro-crackles”, digital clubbing

Question 40

Idiopathic Pulmonary Fibrosis

Imaging findings

Pathology findings

Treatment

Answer 40

Imaging:

1) PAUCITY OF GROUND GLASS INFILTRATE
2) peripheral/basilar predominant reticulation
3) traction bronchiectasisi
4) honeycombing

Pathology

1) spatially and temporally heterogeneous fibrosis with fibroblast foci
2) juxtaposition of fiibrotic and normal lung

Treatment

Pirfenidone and nintedanib

Question 41

NSIP

1) similar presentation to ___
2) cause
3) patient population
4) prognosis
5) treatment

Answer 41

1) ILD with cough and DOE
2) idiopathic, collagen vascular disease (RA, sjogren’s, scleroderma, myositis), fibrotic HP, drug
3) younger female
4) better tha UIP/IPF and collagen vascular is better than idiopathic
5) corticosteroids and steroid sparing

Question 42

NSIP

Radiographic

Pathology

Answer 42

Radiographic

1) basilar predominant reticular abnormality
2) volume loss
3) traction bronchiectasis
4) frequent ground glass opacity
5) rare honeycombing

Pathology

1) temporally HOMOGENOUS fibrosis with varying degrees of inflamm and fibrosis

Question 43

Smoking-related ILD’s

Answer 43

1) respiratory bronchiolitis- ILD
2) Desquamative interstitial pneumonia (DIP)
3) Pulmonary Langerhans cell histiocytosis

Question 44

Pulmonary Langerhans Cell histiocytosis

1) patient population
2) radiology,
3) PFTs
4) other manifestations
5) treatment

Answer 44

1) young smokers
2) cysts and nodules; upper lobe predominant; pneumothorax
3) mixed PFTs (airway + nodule);
4) 15% extrapulm diagnosis = bone lesions, pituitary involvement
5) smoking cessation

Question 45

Organizing pneumonia = BOOP

1) severity of presentation
2) cause
3) type of pneumonia
4) radiology
5) pathology
6) treatment

Answer 45

1) subacute to chronic
2) idiopathic (COP) or secondary to CVD, drugs, HP, aspiration
3) noninfectious pneumonia
4) ground glass and migratory consolidation
5) organizing pneumonia; plugs of granulation tissue and fibrosis distal to bronchioles
6) steroids 6-12 mo

Question 46

Acute eosinophilic pneumonia

1) mimics ___
2) diagnosis how?
3) treatment
4) type of pneumonia

Answer 46

1) ARDS
2) diagnosed by BAL showing eosinophils
3) steroids
4) noninfectious = eosinophil in alveoli and interstitium

Question 47

Chronic eosinophilic pneumonia

1) type of onset
2) assoc with ___
3) radiology
4) diagnosed how?
5) treatment

Answer 47

1) subacute (eosinophils in blood stream unlike acute)
2) asthma
3) peripheral eosinophilia “photographic negative of pulm edema”
4) BAL
5) steroids

Question 48

Lymphangioleiomyomatosis (LAM)

1) type of lung disease
2) patient population
3) mutaiton
4) mechanism
5) common co-morbidities
6) PFTs
7) treatment

Answer 48

1) cystic
2) young women 20-30
3) mutation in tuberous sclerosis gene 1 or 2
4) peribronchovascular prolif of smooth muscle
5) pneumothorax, chylothorax renal tumors (angiomyolipomas)
6) obstructive pattern
7) mTOR inhibitors (sirolimus)

Question 49

Sarcoidosis

1) describe
2) path
3) commonly affects
4) common co-morbidities
5) PFTs
6) symptoms
7) patient population
8) radiology
9) treatment

Answer 49

1) systemic granulomatous disorder unknown etiology
2) noncaseating granulomas
3) entire body
4) mediastinal and bilateral hilar lymphadenopathy, abnormal CXR, fibrosis, airway involve
5) normal in stage 1

airflow limitation and decr FEV1/FVC ratio with bronchodilator response

with infiltrative disease, restrictive pattern with decr DLCO

can have mixed

6) cough, chest pain, wheezing, fever, chills, fatigue, wheezing
7) before age 40 in northern Euro descent
8) Lambda sign, interstitial infiltrates and nodules
9) no treatment necessary but can use steroids/cytotoxic

Question 50

how is diagnosis of sarcoidosis made?

Answer 50

clinical finding and granulomas

Question 51

Radiographic stages of Sarcoid

Answer 51

Stage 1: Bilateral hilar LAD without infiltration
Stage 2: Bilateral hilar LAD with infiltration

Stage 3: Infiltration without LAD

Stage 4: Fibrotic disease

Question 52

Lofgren’s syndrome characterized by (3 things)

How do you diagnosis?

PFTs?

Answer 52

Inflammatory arthritis

Erythema nodosum

Bilateral hilar lymphadenopathy

___

Diagnosis by finding well-formed granulomas via bronchoscopy, mediastinoscopy, surgical lung biopsy

____

PFT normal in stage 1