02a: Interstitial Lung Disease

Question 1

T/F: Most ILDs are of unknown cause.

Answer 1

True

Question 2

List four key clinical features of ILDs.

Answer 2

1. Dyspnea
2. Tachycardia
3. Cyanosis
4. Crackles

Question 3

T/F: Pulmonary HT and CHF are common clinical consequences of ILDs.

Answer 3

True

Question 4

Often the first pulm function test to be abnormal in ILDs is (increase/decrease/not change) in (X).

Answer 4

Decrease;

DLCO

Question 5

You would expect lung compliance to (increase/decrease/not change) in ILDs since elastic recoil is (increased/decreased/not changed).

Answer 5

Decrease;

Increased

Question 6

Regardless of cause, the earliest common manifestation of most ILDs is:

Answer 6

Alveolitis (accumulation of inflammatory/immune effector cells within alveolar walls/spaces)

Question 7

IPF (Idiopathic Pulm Fibrosis) will show (X) histology pattern. Briefly describe what you would see.

Answer 7

X = UIP (usual interstitial pneumonia)

1. Predominantly fibrosis along interlobular septa (esp subpleural and lower lobes)
2. Temporal heterogeneity

Question 8

Temporal heterogeneity: areas of ongoing fibrosis, aka (X), can be seen near areas of (Y).

Answer 8

X = fibroplastic foci
Y = normal lung or established fibrosis

Question 9

T/F: Fibrosis/tissue damage in IPF (Idiopathic Pulm Fibrosis) is a result of inflammation.

Answer 9

False! Doesn’t respond to anti-inflammatory drugs

Result of “wound-healing” due to unidentified agent

Question 10

T/F: IPF can eventually lead to bronchiectasis, metaplasia, and smooth muscle hyperplasia.

Answer 10

True

Question 11

NSIP (Non-specific Interstitial Pneumonia) clinical course differs from UIP in which way(s)?

Answer 11

1. Responds to steroids

2. Much better prognosis

Question 12

(NSIP/UIP) tends to occur in younger patients.

Answer 12

NSIP

Question 13

T/F: “Ground glass” opacifications/honeycombing is seen in UIP, but not NSIP.

Answer 13

False

Question 14

T/F: NSIP will present histologically similar to infectious pneumonia.

Answer 14

False (not evenly distributed)

Question 15

The least specific pattern of lung injury is (X). When no etiology is identified the term (Y) is applied.

Answer 15

X = Organizing Pneumonia (OP)
Y = Cryptogenic Organizing Pneumonia (COP)

Question 16

T/F: Cryptogenic Organizing Pneumonia (COP) and Desquamative Interstitial Pneumonia (DIP) respond super well to steroid therapy.

Answer 16

True

Question 17

(UIP/NSIP/COP/DIP) has a clear association with smoking and is characterized by large collections of (X) cells in airspaces.

Answer 17

DIP (Desquamative Interstitial Pneumonia)

X = (“smoker’s”) macrophages (filled with brown pigment)

Question 18

T/F: DIP is minimally (if at all) associated with fibrosis.

Answer 18

True

Question 19

T/F: Fibrosis is prominent feature of COP.

Answer 19

False

Question 20

DIP: Alveolar walls may be thickened due to:

Answer 20

WBCs (lymphocytes, plasma cells, eosinophils)

Question 21

An immunologically-mediated predominantly interstitial lung disease caused by
prolonged exposure to inhaled organic dusts and antigens.

Answer 21

Hypersensitivity Pneumonitis

Question 22

“Farmer’s lung” and “air conditioner lung” are examples of which type of pulm interstitial diseases?

Answer 22

HP (Hypersensitivity Pneumonitis)

Question 23

T/F: Reactions of HP (Hypersensitivity Pneumonitis) occur in interstitium, esp lobar septa.

Answer 23

False - in alveoli (“allergic alveolitis”)

Question 24

Respiratory Bronchiolitis (RB) ILD is similar to (X) disease, but with (more/less) pronounced symptoms.

Answer 24

X = DIP (Desquamative Interstitial Pneumonia)

Less

Question 25

List some clinical symptoms/patient history that would make you suspicious for DIP (Desquamative Interstitial Pneumonia).

Answer 25

1. Smoker 2. Insidious onset of cough, dyspnea 3. Digital clubbing (50%)

Question 26

Presence of circulating immune complexes and small non-caseating granulomas is characteristic of (X) interstitial lung disease.

Answer 26

X = HP (hypersensitivity pneumonitis)

Question 27

(X) diseases are lung reactions to inhaled mineral dusts (coal dust, silica, asbestos, beryllium, iron oxides).

Answer 27

X = pneumoconiosis

Question 28

Non-caseating granulomas and multi-nucleated giant cells in lung make you suspicious of (UIP/NSIP/DIP/Sarcoidosis).

Answer 28

Sarcoidosis

Question 29

Most prevalent chronic occupational disease in the world.

Answer 29

Silicosis (ILD)

Question 30

Anthracosis refers to disease in which (X) occurs. At its mildest, it presents with (Y) symptoms.

Answer 30

``` X = inhaled carbon pigment is engulfed by alveolar and interstitial macrophages (a pneumoconiosis) Y = no ```

Question 31

Patients with UIP typically present with (sudden/gradual) increase dyspnea during (exertion/rest/sleep). This is accompanied by (productive/non-productive) cough.

Answer 31

Gradual (insidious); Exertion; Non-productive

Question 32

Orthopnea is:

Answer 32

Shortness of breath (dyspnea) while lying flat

Question 33

ILDs: PCO2 is typically (high/normal/low), except for advanced disease, when it (rises/drops) relative to normal.

Answer 33

Low or normal (mild respiratory alkalosis); | Rises

Question 34

Unlike normal individuals, patients with ILDs increase Alv ventilation during exercise by increasing:

Answer 34

Predominantly RR (can't increase TV)

Question 35

The majority of ILD patients will have which findings on CXR at initial evaluation.

Answer 35

Nodulolinear Infiltrates (bilateral) with lower lobe, peripheral predominance

Question 36

T/F: Some patients with ILDs may present with completely normal CXR.

Answer 36

True (but under 10%)

Question 37

Prominent hilar and mediastinal adenopathy on CXR are common in which disease states?

Answer 37

Sarcoidosis, tumor, infectious granulomatous disease

Question 38

Peripheral infiltrates with central sparing is seen in (X) disease. The opposite is seen in (Y) disease.

Answer 38

``` X = chronic eosinophilic pneumonia Y = pulmonary edema ```

Question 39

ILDs: Serological studies primarily help in the diagnosis of (X) diseases as the cause for pulm fibrosis.

Answer 39

X = CVD (Collagen Vascular Diseases) Ex: Lupus, RA

Question 40

ILDs: while elevated levels of anti-nuclear Ab or Rheumatoid factor may be suggestive of (X) diseases, low levels of these is suggestive of (Y) disease.

Answer 40

``` X = Lupus or RA; Y = IPF (low levels seen in 30-40% of patients) ```

Question 41

IPF: CXR shows (X), CT shows (Y), and biopsy shows (Z).

Answer 41

``` X = increase in linear markings (esp at bases) Y = patchy peripheral process with honeycombing Z = fibroblastic foci ```

Question 42

(UIP/NSIP/HP/DIP) is typically associated with (X) conditions such as scleroderma.

Answer 42

NSIP; | X = rheumatologic

Question 43

T/F: Both UIP and DIP patterns can be found (simultaneously) in different areas of the same lung.

Answer 43

True

Question 44

T/F: Diagnosis of UIP can be done by VAT (Video-Assisted Thoroscopy) or transbronchial biopsy.

Answer 44

False - VAT is procedure of choice (biopsy is ok too) Bronchoscopy/transbronchial biopsy not possible in UIP due to heterogeneous pattern (samples too small, sampling error too great)

Question 45

New treatment options for IPF include:

Answer 45

Pirfenidone and Nintedanib.

Question 46

T/F: Management of IPF includes long-term treatment with corticosteroids.

Answer 46

False - doesn't respond to steroids!!

Question 47

T/F: Antioxidants have proven to be beneficial for IPF treatment.

Answer 47

False

Question 48

Nintedanib has been FDA approved for treatment of (X). What's its mechanism of action?

Answer 48

X = UIP/IPF Oral Tyrosine Kinase Inhibitor that interferes with multiple receptors (FGF, PDGF, VEGF)

Question 49

Pirfenidone has been FDA approved for treatment of (X). What's its mechanism of action?

Answer 49

X = UIP/IPF We don't know...

Question 50

How might unmonitored oxygen therapy accelerate disease progression of IPF?

Answer 50

Increased production of oxygen radicals

Question 51

"Ground glass" appearance on CT is caused by:

Answer 51

Alveolar inflammation

Question 52

Signet ring sign on CT is (X) and indicative of which disease complication?

Answer 52

X= dilated airway (diameter larger than plum a it travels with) Bronchiectasis