Less Common Pulmonary Disease

Question 1

Ddx of diffuse cystic lung disease (5)?

Answer 1

• LAM
• PLCH
• Birt-Hogg-Dube
• LIP
• Amyloid

Question 2

PFTs in LAM?

Answer 2

obstructive with decreased DLCO

Question 3

LAM associated with what other disease?

Answer 3

Tuberos Sclerosis

Question 4

LAM biomarkers?

Answer 4

VEGF-D, HMB-45

Question 5

Treatment of LAM?

Answer 5

Sirolimus

-O2, bronchodilators, transplant

Question 6

Biomarkers for PLCH?

Answer 6

CD1a

Question 7

Biopsy findings of PLCH?

Answer 7

Bronchiolocentric, destrutive lesions containing Langerhan’s cells

Question 8

BAL findings in PLCH?

Answer 8

> 5% CD1a

Question 9

Treatment of PLCH?

Answer 9

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

Question 10

Gene with mutation in Birt-Hogg-DUbe?

Answer 10

folliculin

Question 11

Treatment of follciular bronchitis?

Answer 11

• Erythromycin would be buzz word.

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

Question 12

Antibodies in Pulmonary Alveolar Proteinosis?

Answer 12

Anti-GMCSF

Question 13

Biomarkers for Pulmonary Alveolar Proteinosis?

Answer 13

Increased:

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

Question 14

PFTs in Pulmonary Alveolar Proteinosis?

Answer 14

restrictive, decreased DLCO

Question 15

CT findings in Pulmonary Alveolar Proteinosis?

Answer 15

Crazy Paving

Question 16

Dx of Pulmonary Alveolar Proteinosis?

Answer 16

Cloudy/milky fluid in BAL, PAS(+)

Question 17

Treatment of Pulmonary Alveolar Proteinosis?

Answer 17

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

Question 18

Cause of death in Pulmonary Alveolar Proteinosis?

Answer 18

• Respiratory failure

- Opportunistic disease w/ Nocardia or NTM

Question 19

CT findings in Pulmonary Alveolar Microlithiasis?

Answer 19

“Sandstorm”

“Black Pleural Line”

Question 20

Genetic mutation in Pulmonary Alveolar Microlithiasis?

Answer 20

SLC34A2, encoding type IIb Na-Phosphate transporter

Question 21

Treatment in Pulmonary Alveolar Microlithiasis?

Answer 21

Transplant. No medicine works.

Question 22

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

Answer 22

Erdheim-Chester Disease

Question 23

Treatment for Erdheim-Chester Disease?

Answer 23

Vemurafenib (BRAF-inhibitor), IFN, Chemo

Question 24

Diagnosis of pulmonary amyloid?

Answer 24

Congo-red (+) on biopsy

Question 25

Treatment of IgG4-related disease?

Answer 25

steroids. Responds well.

Question 26

Pulmonary manifestations of IBD-related disease?

Answer 26

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

Question 27

Time of onset of radiation pneumonitis?

Answer 27

4-12 weeks later

Question 28

Time of onset of radiation fibrosis?

Answer 28

6-12 months later

Question 29

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

Answer 29

BOOP

Question 30

CT of BOOP?

Answer 30

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

Question 31

Treatment of boop?

Answer 31

Azithro, steroids, ICS/LABA

Question 32

Laterality of bronchopulmonary sequestration?

Answer 32

Left more common than right

Question 33

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

Answer 33

Extra-lobar. DOES have its own pleura.