02a: Interstitial Lung Disease Flashcards

1
Q

T/F: Most ILDs are of unknown cause.

A

True

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2
Q

List four key clinical features of ILDs.

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

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

A

True

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4
Q

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

A

Decrease;

DLCO

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5
Q

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

A

Decrease;

Increased

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6
Q

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

A

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

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7
Q

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

A

X = UIP (usual interstitial pneumonia)

  1. Predominantly fibrosis along interlobular septa (esp subpleural and lower lobes)
  2. Temporal heterogeneity
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8
Q

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

A
X = fibroplastic foci
Y = normal lung or established fibrosis
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9
Q

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

A

False! Doesn’t respond to anti-inflammatory drugs

Result of “wound-healing” due to unidentified agent

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10
Q

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

A

True

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11
Q

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

A
  1. Responds to steroids

2. Much better prognosis

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12
Q

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

A

NSIP

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13
Q

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

A

False

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14
Q

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

A

False (not evenly distributed)

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15
Q

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

A
X = Organizing Pneumonia (OP)
Y = Cryptogenic Organizing Pneumonia (COP)
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16
Q

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

A

True

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17
Q

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

A

DIP (Desquamative Interstitial Pneumonia)

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

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18
Q

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

A

True

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19
Q

T/F: Fibrosis is prominent feature of COP.

A

False

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20
Q

DIP: Alveolar walls may be thickened due to:

A

WBCs (lymphocytes, plasma cells, eosinophils)

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21
Q

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

A

Hypersensitivity Pneumonitis

22
Q

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

A

HP (Hypersensitivity Pneumonitis)

23
Q

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

A

False - in alveoli (“allergic alveolitis”)

24
Q

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

A

X = DIP (Desquamative Interstitial Pneumonia)

Less

25
Q

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

A
  1. Smoker
  2. Insidious onset of cough, dyspnea
  3. Digital clubbing (50%)
26
Q

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

A

X = HP (hypersensitivity pneumonitis)

27
Q

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

A

X = pneumoconiosis

28
Q

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

A

Sarcoidosis

29
Q

Most prevalent chronic occupational disease in the world.

A

Silicosis (ILD)

30
Q

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

A
X = inhaled carbon pigment is engulfed by alveolar and interstitial macrophages (a pneumoconiosis)
Y = no
31
Q

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

A

Gradual (insidious);
Exertion;
Non-productive

32
Q

Orthopnea is:

A

Shortness of breath (dyspnea) while lying flat

33
Q

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

A

Low or normal (mild respiratory alkalosis);

Rises

34
Q

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

A

Predominantly RR (can’t increase TV)

35
Q

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

A

Nodulolinear Infiltrates (bilateral) with lower lobe, peripheral predominance

36
Q

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

A

True (but under 10%)

37
Q

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

A

Sarcoidosis, tumor, infectious granulomatous disease

38
Q

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

A
X = chronic eosinophilic pneumonia
Y = pulmonary edema
39
Q

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

A

X = CVD (Collagen Vascular Diseases)

Ex: Lupus, RA

40
Q

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.

A
X = Lupus or RA;
Y = IPF (low levels seen in 30-40% of patients)
41
Q

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

A
X = increase in linear markings (esp at bases)
Y = patchy peripheral process with honeycombing
Z = fibroblastic foci
42
Q

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

A

NSIP;

X = rheumatologic

43
Q

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

A

True

44
Q

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

A

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)

45
Q

New treatment options for IPF include:

A

Pirfenidone and Nintedanib.

46
Q

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

A

False - doesn’t respond to steroids!!

47
Q

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

A

False

48
Q

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

A

X = UIP/IPF

Oral Tyrosine Kinase Inhibitor that interferes with multiple receptors (FGF, PDGF, VEGF)

49
Q

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

A

X = UIP/IPF

We don’t know…

50
Q

How might unmonitored oxygen therapy accelerate disease progression of IPF?

A

Increased production of oxygen radicals

51
Q

“Ground glass” appearance on CT is caused by:

A

Alveolar inflammation

52
Q

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

A

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

Bronchiectasis