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Pulmonology > SM 163 Introduction to DPLD > Flashcards

SM 163 Introduction to DPLD Flashcards

1
Q

What is DPLD and what is it’s other common name?

A

Diffuse Parenchymal Lung Disease aka Interstitial Lung Disease (ILD) = a group of diverse disorders

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2
Q

What part of the lung does DPLD effect?

A

DPLD effects the Alveolar wall, especially the interstitium, alveolar space, and small airways

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3
Q

Does DPLD effect large or small airways?

A

Small airways - also alveolar spaces

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4
Q

What type of cell are Type II Pneumocytes?

A

Cuboidal

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5
Q

What type of cell are Type I Pneumocytes?

A

Squamous

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6
Q

What is the most essential part of diagnosis of DLPD?

A

A careful history

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7
Q

What should the history in DLPD consider?

A

Focus the history on potential inhaled exposures, such as occupation, hobbies, pets, drugs, smoking/vaping

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8
Q

What diseases are associated with DLPD?

A

IPF (a form of UIP) and Sarcoidosis

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9
Q

What diseases are not associated with DLPD?

A

Hypersensitivity Pneumonitis and Rheumatoid Arthritis

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10
Q

Which lung cell types are most susceptible to injury?

A

Type I Pneumocytes

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11
Q

How does DLPD present in terms of symptoms?

A

Symptoms = slow-onset exertional dyspnea, non-productive cough

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12
Q

What physical findings suggest DLPD?

A

Fine crackles, digital clubbing, cyanosis, right heart failure

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13
Q

Can DLPD involve systemic illness?

A

Yes, things like Rheumatoid Arthritis can cause IPF

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14
Q

How can DLPD present on CXR?

A

May be normal 10% of the time, but also shows interstitial and alveolar patterns

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15
Q

What is the interstitial pattern of ILD?

A

Reticular and nodular findings on CXR at the base of the lung

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16
Q

What is the alveolar pattern of ILD?

A

Diffuse and patchy in the upper lung

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17
Q

What is HCRT?

A

High resolution computed tomagraphy

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18
Q

How does HCRT differ from CT?

A

More sensitive, can be diagnostic, can guide surgery

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19
Q

What HCRT finding is associated with Scleroderma?

A

Reticular opacities

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20
Q

What HCRT finding is associated with Sarcoidosis?

A

“Tree-in-Bud” opacities

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21
Q

What HCRT finding is associated with Hypersensitivity Pneumonitis?

A

Ground Glass opacities

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22
Q

What can reticular opacities suggest?

A

Scleroderma

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23
Q

What can “tree-in-bud” opacities suggest?

A

Sarcoidosis

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24
Q

What can Ground Glass Opacities suggest?

A

Hypersensitivity Pnumonitis

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25
Q

What is Traction Bronchiectasis?

A

The airways being ope ned pathologically due to continued inflammation

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26
Q

What can spirometry measure?

A

Minute ventilation

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27
Q

What can spirometry tell us?

A

Low FEFV1/FCR = obstructive lung disease

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28
Q

What does restrictive tell us?

A

High or Normal FEV1/FCR = restrictive lung disease

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29
Q

Is DLPD a restrictive or obstructive?

A

Restrictive, though mixed restrictive/obstructive is also possible

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30
Q

How does DLPD show up on spirometry?

A

Decreased lung volumes, normal/increased FEV1/FVC

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31
Q

How does DLPD show up on the pressure-volume curve?

A

Decreased compliance = flatter slope

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32
Q

How does DLPD effect DLCO?

A

Reduced DLCO

33
Q

How does DLPD effect exercise testing?

A

Subjects will be abnormal earlier than unaffected patients, with increased A-a gradient, decreased PaO2, and increased Vd/Vt

34
Q

How do DLPD get categorized?

A

DLPD: known vs unknown cause
DLPD known: exposure vs systemic disease
DLPD known exposure: occupational vs treatment
DLPD known systemic: granulomatous vs connective tissue disease

35
Q

What are occupational variants of DLPD?

A

Occupational variants of DLPD include Asbestosis and Silicosis, which occur due to exposure to an inhaled antigen

36
Q

What are treatment variants of DLPD?

A

Treatment variants of DLPD result from Radiation and Amiodarone, which occur due to exposure to the treatment for another disease as a side effect

37
Q

What are systemic variants of DLDP?

A

Systemic variants of DLPD result from Rheumatoid Arthritis and Scleroderma, which effect connective tissue through the entire body, including the Lungs

38
Q

What is the primary DLPD of unknown cause?

A

Idiopathic Interstitial Pneumonias, including IPF

39
Q

What is an IIP?

A

A class of DLPD, all of unknown cause

40
Q

What are the major IIP’s?

A

Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Nonspecific Interstitial Pneumonia (NSIP)
Acute Interstitial Pneumonia (AIP)
Cryptogenic Organizing Pneumonia (COP)

41
Q

What are the rare IIP’s?

A

Idiopathic Lymphocytic Interstitial Pneumonia (LIP)

Idiopathic Pleuroparenchymal Fibroelastosis

42
Q

What is the newest IIP?

A

IPAF, the autoimmune version

43
Q

What is the most common IIP?

A

IPF

44
Q

What types of DPLD can have both known and unknown causes?

A

Granulomatous lung disease, such as Sarcoidosis and Hypersensitivity Pneumonia

45
Q

What are potential infectious causes of DPLD?

A

TB, non-TB mycobacteria, Histo/blasto/coccidio fungal

46
Q

What are the potential non-infectious causes of DPLD?

A

Sarcoidosis, Beryliosis, and Hypersensitivity Pneumonitis

47
Q

What causes Berrylliosis?

A

Exposure to Berrylium, a metal, due to an occupation

48
Q

What are the immune effects of Sarcoidosis?

A

Increased Th1/Th17 responses at the site of disease

49
Q

What type of drug does Sarcoidosis respond to?

A

Steroids

50
Q

What needs to be excluded to diagnose Sarcoidosis and how?

A

Need to exclude infection with culture and stain, as well as excluding potential immune response

51
Q

What organ is mainly affected by Sarcoidosis?

A

The Lung, skin and eyes common too

52
Q

How does Sarcoidosis present on CXR?

A

Sarcoidosis results in nodes in the mid and upper lungs, which represent well organized granules

53
Q

What is a giant cell?

A

Fused macrophages, commonly found in granulomas

54
Q

What is Hypersensitivity Pneumonitis?

A

An immune-mediated lung disease that can be triggered by inhaled organic antigens

55
Q

Do innate or adaptive immune response mediate Hypersensitivity Pneumonitis?

A

Both Innate and adaptive immune responses play a role

56
Q

What type of antibody is involved in Hypersensitivity Pneumonitis?

A

IgG targeted towards the offending antigen

57
Q

Does Hypersensitivity Pneumonitis present acutely, subacutely, or chronically?

A

All of the above

58
Q

How does Hypersensitivty Pneumonitis present acutely?

A

Fever/chills/dyspnea within 4-8hrs of exposure

59
Q

How does Hypersensitivity Pneumonitis present subacutely?

A

Gradually over weeks

60
Q

How does Hypersensitivity Pneumonitis present chronically?

A

Insidious onset of dyspnea, cough, hypoxemia over months-years

61
Q

What is the most essential part of diagnosis for Hypersensitivity Pneumonitis?

A

Detailed exposure history

62
Q

What does Hypersensitivity Pneumonitis look like on HRCT?

A

Ground glass opacities if acute/subacute, traction bronchiectasis if chronic

63
Q

What tests can be used to diagnose Hypersensitivit Pneumonitis?

A

PFT’s, serum antibodies, BAL, lung biopsy

64
Q

What factors are essential for diagnosis DPLD?

A

History, serology, and HRCT pattern

65
Q

Why is bronchoscopic biopsy limited in DPLD?

A

Biopsy samples are too small for most diagnoses, except cancer, sarcoid, and infection

66
Q

What is the gold standard for lung biopsy?

A

Surgical lung biopsy

67
Q

How are pneumoconiosis treated?

A

Terminate the expousre

68
Q

How is IPF treated?

A

DO NOT USE CORTICOSTEROIDS

Pirfenidone and Nintedinib

69
Q

How are NSIP and IPAF treated?

A

TRAC = Tacrolimus, Rituximab, Azathioprine, Corticosteroids

70
Q

How does Rituximab work?

A

mAb against CD-20 on B-cells

71
Q

How is Sarcoidosis treated?

A

CAT = Corticosteroids, Azathioprine, TNF inhibitors

72
Q

What are infliximab and adalimumab?

A

TNF inhibitors

73
Q

How is Hypersensitivity Pneumonitis treated?

A

Termination of exposure if offending antigen is identified

Corticosteroids

74
Q

What is the interstitium?

A

A potential space between the alveolar epithelium and capillary endothelium

75
Q

What are the common symptoms of DLPD?

A

Dyspnea, cough, fatigue, and weight loss as the disease progresses

76
Q

What symptom is most specific to IPF?

A

Digital clubbing

77
Q

What disease is associated hilar and mediastinal lymphadenopathy?

A

Sarcoidosis

78
Q

Does the presence of antibodies indicate DLPD?

A

Not necessarily

79
Q

How do DLPD effect diffusion capacity?

A

Decreased diffusion capacity as seen on lower DLCO

Pulmonology (22 decks)

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