Abstract
There is growing appreciation for the prevalence and impact of pulmonary hypertension in patients with sarcoidosis (SAPH). This chapter will focus on the pathophysiology of this complication and its association with patients’ outcomes, including functional ability and survival. When to suspect complicating SAPH will be addressed with a suggested work-up algorithm presented. The potential role and data supporting the use of pulmonary vasoactive agents in SAPH will be addressed. However, there is a paucity of data in this area and the need for further investigation in the form of multicenter, double-blind, randomized controlled studies will be underscored.
Keywords
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- 6MWT:
-
6-min walk
- BAL:
-
Bronchoalveolar lavage
- DLCO :
-
Diffusing capacity for carbon monoxide
- ET-1:
-
Endothelin 1
- ERA:
-
Endothelin receptor antagonist
- ILD:
-
Interstitial lung disease
- mPAP:
-
Mean pulmonary artery pressure
- NO:
-
Nitric oxide
- PAH:
-
Pulmonary arterial hypertension
- PCWP:
-
Pulmonary capillary wedge pressure
- PDE-5:
-
Phosphodiesterase 5
- PFTs:
-
Pulmonary function tests
- PH:
-
Pulmonary hypertension
- PVR:
-
Pulmonary vascular resistance
- RAP:
-
Right atrial pressure
- RHC:
-
Right heart catheterization
- RVSP:
-
Right ventricular systolic pressure
- SAPH:
-
Sarcoid-associated pulmonary hypertension
- TTE:
-
Transthoracic echocardiogram
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Shlobin, O.A., Nathan, S.D. (2014). Sarcoidosis-Associated Pulmonary Hypertension. In: Judson, M. (eds) Pulmonary Sarcoidosis. Respiratory Medicine, vol 17. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4614-8927-6_6
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