Miscellaneous Flashcards

1
Q

GOLD Stages

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
A

Bronchiectasis

Bronchiectasis is often divided into CF and non-CF bronchiectasis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Classification of Interstitial Lung Disease

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What ILD is associated with rheumatic disease?

A

UIP pattern in rheumatic disease portends the worst prognosis, with a 5-year mortality of > 50%. It is most commonly seen in rheumatoid arthritis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

“Crazy paving”

A

Pulmonary alveolar proteinosis (PAP)

PAP results from surfactant accumulation and alveolar macrophage dysfunction

Three types of PAP:

  1. Primary: disruption of GM-CSF signaling either by autoantibodies to GM-CSF or mutations of GSF2RA or CSF2RB (GM-CSF receptor subunits).
  2. Secondary: Underlying disease (hematological disease, in particular. myelodysplastic syndromes). GATA2 deficiency has AD inheritance with diverse presentations, including “MonoMAC” immune deficiency with the absence of circulating monocytes, NK cells, and B cells.
  3. Congenital: Mutations in genes involved in surfactant production (SFTPB, SFTPC, ABCA3, NKX2-1)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

ILD associated with primary immunodeficiency

A
  • ILD occurs much more frequently in CVID relative to XLA or X-linked and autosomal recessive hyper IgM
  • ILD occurs more frequently in monogenic immune dysregulation disorders associated with antibody deficiency (activated PI3 kinase syndrome, a gain of function of STAT3, and deficiency of CTLA-4 and LRBA)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

SERPINA1

A

alpha1 -antitrypsin deficiency

An imbalance between protease and antiprotease activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly