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ABIM Pulmonary > Diffuse Interstitial Lung Dz > Flashcards

Diffuse Interstitial Lung Dz Flashcards

1
Q

UIP Histology & HRCT Findings

A

Histology: Fibroblasts, peripheral patchy fibrosis, honeycombing, +/- inflammation
HRCT: Subpleural/basal predominance, reticular changes, traction bronchiectasis, +/- honeycombing

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2
Q

NSIP Histology & HRCT Findings

A

Histology: Homogenous, chronic interstitial inflammation/fibrosis
HRCT: Homogenous, GGOs, reticular, +/- subpleural sparing

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3
Q

OP Histology & HRCT Findings

A

Histology: Patchy, granulation tissue plugs
HRCT: Patchy consolidation / GGOs, Atoll sign (reverse halo sign)

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4
Q

RBILD / DIP Histology & HRCT Findings

A

Histology: Pigmented (smoker’s) Macrophages, bronchiolocentric or diffuse
HRCT: Vague nodules, patchy to diffuse GGOs

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5
Q

DAD Histology & HRCT Findings

A

Histology: hyaline membranes, granulation tissue, organizing fibroproliferative
HRCT: Diffuse consolidation / GGOs

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6
Q

LIP Histology & HRCT Findings

A

Histology: Lymphoid, plasma cells

HRCT Findigns: Patchy GOs, nodules, cysts

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7
Q

PPFE Histology & HRCT Findings

A

Histology: Pleural / subpleural fibroelastosis, septal elastosis
HRCT: Upper / subpleural fibrosis, pneumomediastinum, pneumothorax

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8
Q

Five smoking-related IIPs

A
  • RB-ILD
  • DIP
  • PLCH (Pulm Langerhan’s Cell Histiocytosis)
  • AEP (Acute Eosinophilic Pneumonia)
  • CPFE (Combined Pulm Fibrosis & Emphysema)
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9
Q

Two anti-fibrotic therapies for IPF

A

Nintedanib (Ofev)

Pirfenidone (Esbriet)

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10
Q

Age rage for IPF

A

50-80 years

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11
Q

IIPs which are steroid responsive

A
  • RBILD
  • DIP
  • COP (recurs)
  • AIP
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12
Q

Most connective tissue diseases cause ____ (form of IIP), except Sjogren’s, which causes ______.

A
  1. NSIP

2. LIP

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13
Q

Antisynthetase Syndrome antibody

A

Anti-Jo-1

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14
Q

Score for IPF Mortality

A

GAP Score

  • Gender
  • Age
  • PFTs (FVC, DLCO)
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15
Q

Typical UIP HRCT Pattern

A
  • Subpleural/basal predominance, heterogenous
  • Reticular abnormalities
  • Honeycombing +/- traction bronchiectasis
  • Absence of GGOs, extensive consolidation, mosaic attenuation, nodules, cysts
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16
Q

Typical UIP Histology Pattern

A
  • Subpleural or paraseptal predominance
  • Patchy involvement
  • Dense fibrosis / architectual distortion +/- honeycombing
  • Fibroblast foci
  • Absence of other features (granulomas)
17
Q

Age for eligibility of clinical Dx of IPF

A

60yo

18
Q

Acute IIPs

A
  • OP / COP

- DAD / AIP

19
Q

PFT abnormality in CPFE (Combined Pulmonary Fibrosis & Emphysema)

A
  • Low DLCO

- Preserved spirometry & lung volumes

20
Q

Term for when IIP is first manifestation of CTDz

A

IPAF (Interstitial Pneumonia w/ AI Features)

21
Q

Types of granulomas in Sarcoidosis

A

Noncaseating, epitheloid cell granulomas w/o other cause

22
Q

Epidemiology of Sarcoidosis

  • Gender?
  • Race?
  • Nationality
A
  • Women
  • African Americans
  • Scandinavians
23
Q

Screen for extrapulmonay sarcoid with what testing?

A

EKG definitely. Consider TTE.

24
Q

Staging of Sarcoid 0-4

A 
0 = no lung involvement
1 = bilateral hilar lymphadenopathy w/o parenchymal involvement
2 = bilateral hilar lymphadenopathy w/ parenchymal involvement
3 = parenchymal involvement w/o hilar lymphadenopathy
4 = progressive fibrosis +/- cavitary lesion or cysts
25
Q

Mortality from sarcoid due to what 3 things?

A
  • Progressive pulmonary fibrosis
  • Aspergilloma bleed
  • Cardiac involvement
26
Q

GLILD (Granulomatous Lymphocytic ILD) in what characteristic patient population

A

Severe immunosuppression, usually CVID

27
Q

GLILD (Granulomatous Lymphocytic ILD) histologic features?

A

non-necrotizing granulomas, LIP< follicular bronchiolitis

28
Q

GLILD (Granulomatous Lymphocytic ILD) treatment?

A

steroids, immunosuppression, IVIG

29
Q

Treatment for cough in Sarcoidosis?

A

ICS

30
Q

Indications for treatment in sarcoidosis?

A
  • Pulmonary stage II-III w/ moderate-severe PFT changes

- Extra-pulmonary disease (cardiac, neurologic, occular not responding to topical therapy, hypercalcemia)

31
Q

Treatment for sarcoidosis?

A

Prednisone, with 6-12month taper

2nd line is immunosuppressives, usually methotrexate

ABIM Pulmonary (37 decks)

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