Less Common Pulmonary Disease Flashcards Preview

ABIM Pulmonary > Less Common Pulmonary Disease > Flashcards

Flashcards in Less Common Pulmonary Disease Deck (33)
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1
Q

Ddx of diffuse cystic lung disease (5)?

A
  • LAM
  • PLCH
  • Birt-Hogg-Dube
  • LIP
  • Amyloid
2
Q

PFTs in LAM?

A

obstructive with decreased DLCO

3
Q

LAM associated with what other disease?

A

Tuberos Sclerosis

4
Q

LAM biomarkers?

A

VEGF-D, HMB-45

5
Q

Treatment of LAM?

A

Sirolimus

-O2, bronchodilators, transplant

6
Q

Biomarkers for PLCH?

A

CD1a

7
Q

Biopsy findings of PLCH?

A

Bronchiolocentric, destrutive lesions containing Langerhan’s cells

8
Q

BAL findings in PLCH?

A

> 5% CD1a

9
Q

Treatment of PLCH?

A
  • Stop smoking
  • Cladribine
  • Vemurafenib (B-raf inhibitor, if BRAF(+))
  • No role for transplant; disease recurs
10
Q

Gene with mutation in Birt-Hogg-DUbe?

A

folliculin

11
Q

Treatment of follciular bronchitis?

A
  • Erythromycin would be buzz word.

- In reality, treat underlying disease, bronchodilators, steroids. Good prognosis.

12
Q

Antibodies in Pulmonary Alveolar Proteinosis?

A

Anti-GMCSF

13
Q

Biomarkers for Pulmonary Alveolar Proteinosis?

A

Increased:

  • LDH
  • Surfactant A & D
  • KL-6 (mucin-like glycoprotein)
14
Q

PFTs in Pulmonary Alveolar Proteinosis?

A

restrictive, decreased DLCO

15
Q

CT findings in Pulmonary Alveolar Proteinosis?

A

Crazy Paving

16
Q

Dx of Pulmonary Alveolar Proteinosis?

A

Cloudy/milky fluid in BAL, PAS(+)

17
Q

Treatment of Pulmonary Alveolar Proteinosis?

A
  • Observe mild disease
  • Whole lung lavage in ToC
  • Inhaled or SQ GM-CSR
  • Rituxin/statin
  • No role for transplant. Disease recurs.
18
Q

Cause of death in Pulmonary Alveolar Proteinosis?

A
  • Respiratory failure

- Opportunistic disease w/ Nocardia or NTM

19
Q

CT findings in Pulmonary Alveolar Microlithiasis?

A

“Sandstorm”

“Black Pleural Line”

20
Q

Genetic mutation in Pulmonary Alveolar Microlithiasis?

A

SLC34A2, encoding type IIb Na-Phosphate transporter

21
Q

Treatment in Pulmonary Alveolar Microlithiasis?

A

Transplant. No medicine works.

22
Q

Buzzword: “Foamy histiocytes, CD1a (-)”?

A

Erdheim-Chester Disease

23
Q

Treatment for Erdheim-Chester Disease?

A

Vemurafenib (BRAF-inhibitor), IFN, Chemo

24
Q

Diagnosis of pulmonary amyloid?

A

Congo-red (+) on biopsy

25
Q

Treatment of IgG4-related disease?

A

steroids. Responds well.

26
Q

Pulmonary manifestations of IBD-related disease?

A
  • Large & small airway involvement
  • Sublgottic stenosis
  • Entero-pulmonary fistulas
  • ILDs: OP, NSIP, granulomas
  • Serositis (pleuritis, pericarditis)
27
Q

Time of onset of radiation pneumonitis?

A

4-12 weeks later

28
Q

Time of onset of radiation fibrosis?

A

6-12 months later

29
Q

Radiation-induced organizing pneumonia now classified under what other disease?

A

BOOP

30
Q

CT of BOOP?

A

Mosiac pattern with patchy areas of trapping on expiratory films, scattered bronchiectasis

31
Q

Treatment of boop?

A

Azithro, steroids, ICS/LABA

32
Q

Laterality of bronchopulmonary sequestration?

A

Left more common than right

33
Q

Intra-lobar or extra-lobar bronchopulmonary sequestration seen in adults? Has or does not have its own pleura?

A

Extra-lobar. DOES have its own pleura.