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Pulmonology > Intro to DPLD > Flashcards

Intro to DPLD Flashcards

1
Q

What are the four main classifications of ILD?

A

1) diseases of known cause or association
2) idiopathic interstitial pneumonias
3) granulomatous diseases
4) miscellaneous interstitial diseases

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2
Q

Interstitial lung diseases (ILDs) also go by what name?

A

diffuse parenchymal lung diseases

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3
Q

What is the defining feature of ILD’s histologically speaking?

A

Damage to the alveolar wall and interstitium

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4
Q

What is pneumoconiosis?

A

DPLD of known causes from occupational exposure to inorganic dusts

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5
Q

What is hypersensitivity pneumonitis?

A

DPLD of known causes from an immunologic response to inhaled organic dusts

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6
Q

DPLDs caused by reactions to chemicals, drugs, radiation and toxic gases or associated with connective tissue diseas fall under what category of DPLD?

A

known cause

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7
Q

Idiopathic pulmonary fibrosis (IPF) and

Idiopathic nonspecific interstitial pneumonia (NSIP) are what category of idiopathic interstitial pneumonias (IIPs)?

A

chronic fibrosing

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8
Q

What are the 2 smoking related IIPs

A

Respiratory bronchiolitis-interstitial lung disease (RB-ILD)

Desquamative interstitial pneumonia (DIP)

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9
Q

What are the 2 acute/subacute IIPs?

A

Cryptogenic organizing pneumonia (COP)

Acute interstitial pneumonia (AIP)

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10
Q

What is the most common class of idiopathic interstitial pneumonias?

A

chronic fibrosing IIPs

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11
Q

Sarcoidosis and hypersensitivity pneumonitis are what kind of DPLD?

A

granulomatous

hypersensitivity is also a DPLD of known cause

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12
Q

What is the most frequent presenting symptom of DPLD?

A

dyspnea

followed by non productive cough

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13
Q

What is the most common finding on pulmonary exam in DPLD?

A

inspiratory crackles (especially IPF)

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14
Q

Most ILD’s have what pattern on Xray?

A

reticular or reticulonodular, typically at the bases

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15
Q

What CXR finding is common in sarcoidosis?

A

hilar lymphadenopathy

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16
Q

What imaging technique is used in diagnosing DPLD?

A

high resolution CT

17
Q

What are characteristic IPF patterns in HRCT?

A

lower lobe-predominant peripheral reticular opacities, traction bronchiectasis and honeycombing

18
Q

ground glass opacities on HRCT are common in what form of DPLD?

A

NSIP

19
Q

upper- or mid-lung-zone-predominant peribronchovascular nodules with hilar and mediastinal lymphadenopathy are common HRCT findings in what form of DPLD?

A

sarcoidosis

20
Q

What kind of testing is useful in diagnosing connective tissue disease associated ILD?

A

auto-antibody serology

21
Q

What elevated enzyme is associated with sarcoidosis?

A

ACE

not sensitive or specific

22
Q

What kind of defect is found in PFT’s for ILDS?

A

restrictive with reduced diffusion capacity

An obstructive defect or mixed restrictive/obstructive defect may also be seen in sarcoidosis or hypersensitivity pneumonitis

23
Q

When is bronchoscopy with trasnbronchial biopsy and bronchoalveolar lavage useful in diagnosing ILD?

A

good for sarcoidosis or lymphangitic carcinoma

24
Q

Video-assisted thoracoscopic surgery (VATS) is used in what process?

A

surgical lung biopsy for diagnosis of many ILDs

25
Q

Other than removing exposure, what is used in hypersensitivity pneumonitis?

A

Corticosteroids and other anti-inflammatory agents are often used; they are typically beneficial in sarcoidosis and hypersensitivity pneumonitis, and sometimes in connective tissue disease-associated ILD

26
Q

Pirfenidone and nintedanib are both used in what disease? (anti-fibrotics)

A

IPF

27
Q

What ILD does not generally respond to steroids?

A

IPF

28
Q

What is the most common organ involved in sarcoidosis?

A

lungs

29
Q

What disease commonly presents with dyspnea and cough; fatigue, fever, night sweats, weight loss and specific symptoms referable to involvement of other organs, as well as hilar/mediastinal lymphadenopathy and reticulonodular opacities, usually in a mid- and upper-lung-zone-predominant distribution and a restrictive defect?

A

sarcoidosis

30
Q

If sarcoidosis does not remit wihin 2-5 years and granulomatous inflammation persists, what is the long term effect?

A

fibrosis and impaired function

31
Q

bird proteins (“bird fancier’s disease), thermophylic actinomycetes in moldy hay (“farmer’s lung”), and Cladosporium or Mycobacterial species in hot tubs (“hot tub lung”) are all examples of what?

A

organic antigens that trigger HP

32
Q

What immune responses play a role in HP?

A

T cell and humeral

33
Q

Acute dyspnea, often accompanied by fever, chills and malaise, within hours after handling birds, hay, or being in a hot tub . This is followed by resolution of symptoms within one to a few days.. What disease does this desribe?

A

hP

34
Q

Is chronic HP often preceded by acute symtpoms?

A

no

35
Q

HRCT findings include centrilobular ground-glass nodular opacities, often with an upper-lobe predominant distribution, and signs of air trapping. Chest imaging show reticular opacities, traction bronchiectasis and honeycombing indicative of fibrosis. On pulmonary function testing, restrictive, obstructive and mixed defects are found, as are decreases in diffusing capacity. Bronchoscopy with bronchoalveolar lavage reveals a cell profile with increased lymphocytes. Lung biopsy findings include airway and alveolar inflammation with large numbers of lymphocytes and poorly formed non-caseating granulomas peribronchially. What disease does this describe?

A

chronic HP

36
Q

What is the difference between HP granulomas and sarcoidosis?

A

The loose granulomas in HP contrast with the tightly packed, organized epithelioid granulomas seen in sarcoidosis.

37
Q

What is the most important part of treating HP?

A

antigen avoidance

can also use corticosteroids and immunosuppression in progressive disease

38
Q

Which pneumocytes are most susceptible to injury?

A

type I

Pulmonology (35 decks)

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