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Respiratory > ILD > Flashcards

ILD Flashcards

0
Q

Name the 7 idiopathic interstitial pneumonias

A

Idiopathic pulmonary fibrosis, non-specific interstital pneumonia, acute interstital pneumonia, cryptogenic organising pneumonia, desquamative interstital pneumonia, respiratory bronchiolitis associated ILD, lymphocytic interstitial pneumonitis

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1
Q

Name then two idiopathic interstital pneumonias particularly associated with smoking

A

DIP (desquamative intersitial pneumonia) and respiratory bronchiolitis associated ILD.

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2
Q

Name 3 pulmonary haemorrhage syndromes

A

Granulomatosis with polyangiitis (formerly Wegners)
Goodpastures (anti-glomerular basement membrane)
Idiopathic pulmonary haemosideroisis

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3
Q

What are 3 typical characteristics of pulmonary lymphangioleiomyomatosis?

A

Affects pre-menopausal women
Present with recurrent pneumothorax
Chylous effusions

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4
Q

Name 5 connective tissue disorders associated with ILD

A 
Progressive systemic sclerosis
Rheumatoid arthritis
Systemic lupus erythrmatosis
Polymyositis/dermatomyositis
Sjogrens
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5
Q

What are 3 extra-pulmonary signs or conditions associated with pulmonary langerhans cell histiocytosis?

A

Diabetes insipidus (Central)
Lyric bone lesions
Exophthalmos

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6
Q

What is the other name for acute interstital pneumonia?

A

Hamman-rich syndrome

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8
Q

What are the characteristic radiological findings in idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonia pattern

  • predominantly basal changes
  • sub pleural reticular opacities
  • honeycombing
  • traction bronchiectasis
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9
Q

What ILDs are associated with basal changes?

A

IPF, asbestosis, NSIP

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10
Q

What ILDs are associated with upper lobe changes?

A

Sarcoid

Hypersensitivity pneumonitis

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11
Q

What findings on PFTs for ILD?

A

Restrictive

  • reduced TLC, FRC, RV
  • reduced FEV1 and FVC - ratio preserved
  • DLCO decreased
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12
Q

What is the most common idiopathic interstital pneumonia?

A

IPF

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13
Q

Who typically gets IPF?

A

males
often ex smokers
age greater then 60 years

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14
Q

What is the classic finding of IPF on imaging?

A

Honeycombing

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15
Q

What is the pathological pattern of IPF?

A

Usual interstital pneumonia - UIP

  • fibroblastic foci
  • lymphoplasmacytic infiltrate
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16
Q

What are some new therapies for IPF?

A

Nintedanib - multiple tyrosine kinase receptor blocker, slows rate of disease progression and increases time to first exacerbation
Pirfenidone - antifibrotic agent, slows rate of decline and reduces mortality

17
Q

When to consider lung transplant for IPF?

A

When DLCO less then 40%

- IPF do best of all disease transplanted

18
Q

What is the imaging features of NSIP?

A

basillar subpleural ground glass opacities

*No honeycombing

19
Q

What is NSIP often associated with?

A

Connective tissue disease related lung disease

20
Q

What is AIP?

A

acute onset, similar to ARDs, high mortality, often requires mechanical ventilation
pathological pattern is diffuse alveolar damage (DAD)
Often have hyaline membrane (which is also present in ARDS)

21
Q

What is COP?

A

Cryptogenic organizing pneumonia (COP)

  • usually follows an infection
  • basilar subpleural ground glass opacities and consolidation
  • good prognosis, 2/3 respond to steroids
22
Q

What is DIP?

A

Desquamative interstital pneumonia
typical in smokers
diffuse ground glass opacity, basal and peripheral
lots of reactive macrophages in intraalvelor spaces
good prognosis with smoking cessation

23
Q

What is RB-ILD?

A

Respiratory bronchiolitis associated ILD

  • centrilobular, micronodular disase
  • occurs in smokers, resolves with cessation of smoking
24
Q

What is pulmonary alveolar proteinosis?

A

most are aquired
autoimmune process with igG antibody against GM-CSF which causes dysfunctional alveolar macrophages
leads to decreased surfactant clearence and occumulation of acid schiff positive proteinaceous material

25
Q

Treatment of PAP?

A

whole lung lavage

26
Q

What is the most common connective tissue disease leading to ILD?

A 
Scleroderma - occurs in half
RA typically in men, 20%
SLE - uncommon to get progressive ILD
Poly/dermatomyositis - 10%
Sjogrens - get lymphocytic interstital pneumonia
27
Q

Name some exposure related ILD?

A

Amiodarone - typically occurs in first year

Methotrexate - uncommon

Respiratory (12 decks)

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