Cordova and D'Alonzo reported phenotypes underlying pulmonary hypertension in patients with sarcoidosis. The authors are from Temple University in Philadelphia.
Patient selection: sarcoidosis and pulmonary hypertension
Confirmation of pulmonary hypertension involves right heart catheterization.
Phenotype
Pathology
Prognosis
Feature
1A
granulomatous vasculitis
good
steroid responsive
1B
vasculopathy
good
not steroid responsive
2
interstitial fibrosis
poor
lung transplant
3
cardiac sarcoidosis
good
CHF management
4
veno-occlusive disease
poor
lung transplant
Pulmonary function testing:
(1) Types 1A, 3 and 4: normal or mild/moderate restrictive pattern
(2) Type 1B: FVC > 50%
(3) Type 2: severe restriction with FVC < 50%
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