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Sarcoidosis-Associated Pulmonary Hypertension

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Pulmonary Sarcoidosis

Part of the book series: Respiratory Medicine ((RM,volume 17))

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.

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Abbreviations

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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Authors and Affiliations

  1. Advanced Lung Disease and Transplant Program, Department of Medicine, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA, 22024, USA

    Oksana A. Shlobin M.D. & Steven D. Nathan M.D.

Authors
  1. Oksana A. Shlobin M.D.
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  2. Steven D. Nathan M.D.
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Corresponding author

Correspondence to Steven D. Nathan M.D. .

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Editors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, New York, USA

    Marc A. Judson

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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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  • DOI: https://doi.org/10.1007/978-1-4614-8927-6_6

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