Less Common Pulmonary Disease Flashcards
Ddx of diffuse cystic lung disease (5)?
- LAM
- PLCH
- Birt-Hogg-Dube
- LIP
- Amyloid
PFTs in LAM?
obstructive with decreased DLCO
LAM associated with what other disease?
Tuberos Sclerosis
LAM biomarkers?
VEGF-D, HMB-45
Treatment of LAM?
Sirolimus
-O2, bronchodilators, transplant
Biomarkers for PLCH?
CD1a
Biopsy findings of PLCH?
Bronchiolocentric, destrutive lesions containing Langerhan’s cells
BAL findings in PLCH?
> 5% CD1a
Treatment of PLCH?
- Stop smoking
- Cladribine
- Vemurafenib (B-raf inhibitor, if BRAF(+))
- No role for transplant; disease recurs
Gene with mutation in Birt-Hogg-DUbe?
folliculin
Treatment of follciular bronchitis?
- Erythromycin would be buzz word.
- In reality, treat underlying disease, bronchodilators, steroids. Good prognosis.
Antibodies in Pulmonary Alveolar Proteinosis?
Anti-GMCSF
Biomarkers for Pulmonary Alveolar Proteinosis?
Increased:
- LDH
- Surfactant A & D
- KL-6 (mucin-like glycoprotein)
PFTs in Pulmonary Alveolar Proteinosis?
restrictive, decreased DLCO
CT findings in Pulmonary Alveolar Proteinosis?
Crazy Paving
Dx of Pulmonary Alveolar Proteinosis?
Cloudy/milky fluid in BAL, PAS(+)
Treatment of Pulmonary Alveolar Proteinosis?
- Observe mild disease
- Whole lung lavage in ToC
- Inhaled or SQ GM-CSR
- Rituxin/statin
- No role for transplant. Disease recurs.
Cause of death in Pulmonary Alveolar Proteinosis?
- Respiratory failure
- Opportunistic disease w/ Nocardia or NTM
CT findings in Pulmonary Alveolar Microlithiasis?
“Sandstorm”
“Black Pleural Line”
Genetic mutation in Pulmonary Alveolar Microlithiasis?
SLC34A2, encoding type IIb Na-Phosphate transporter
Treatment in Pulmonary Alveolar Microlithiasis?
Transplant. No medicine works.
Buzzword: “Foamy histiocytes, CD1a (-)”?
Erdheim-Chester Disease
Treatment for Erdheim-Chester Disease?
Vemurafenib (BRAF-inhibitor), IFN, Chemo
Diagnosis of pulmonary amyloid?
Congo-red (+) on biopsy
Treatment of IgG4-related disease?
steroids. Responds well.
Pulmonary manifestations of IBD-related disease?
- Large & small airway involvement
- Sublgottic stenosis
- Entero-pulmonary fistulas
- ILDs: OP, NSIP, granulomas
- Serositis (pleuritis, pericarditis)
Time of onset of radiation pneumonitis?
4-12 weeks later
Time of onset of radiation fibrosis?
6-12 months later
Radiation-induced organizing pneumonia now classified under what other disease?
BOOP
CT of BOOP?
Mosiac pattern with patchy areas of trapping on expiratory films, scattered bronchiectasis
Treatment of boop?
Azithro, steroids, ICS/LABA
Laterality of bronchopulmonary sequestration?
Left more common than right
Intra-lobar or extra-lobar bronchopulmonary sequestration seen in adults? Has or does not have its own pleura?
Extra-lobar. DOES have its own pleura.
