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Editorials |

Sarcoidosis-Associated Pulmonary Hypertension: One Size Does Not Fit All

Gustavo A. Heresi, MD; Raed A. Dweik, MD, FCCP
Author and Funding Information

Correspondence to: Raed A. Dweik, MD, FCCP, Director, Pulmonary Vascular Program, Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, 9500 Euclid Ave, A90, Cleveland, OH 44122; e-mail: dweikr@ccf.org

Dr. Heresi is Associate Staff, Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic.

Dr. Dweik is Director, Pulmonary Vascular Program, Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic.


The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;135(6):1410-1412. doi:10.1378/chest.08-3040
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Extract

Sarcoidosis is a multisystem disease of unknown etiology that is characterized pathologically by noncaseating granulomatous inflammation with a particular predilection for the lungs. Its clinical course is highly variable, with a sizable proportion of patients experiencing spontaneous regression, while in others chronic disease develops.1

Pulmonary hypertension (PH) is a hemodynamic state defined as a mean pulmonary artery pressure of > 25 mm Hg at rest or > 30 mm Hg with exercise. PH is seen in a variety of clinical settings, notably left-sided valvular and myocardial disease, chronic lung disease with hypoxemia, and chronic pulmonary embolism (Table 1).2 PH with a pulmonary capillary wedge pressure of < 15 mm Hg is called pulmonary arterial hypertension (PAH), and is seen in association with connective tissue disorders, congenital heart disease, and HIV infection, among other conditions. When no clear etiology can be identified in patients with PAH, the disease is classified as idiopathic PAH (IPAH).2,3 While IPAH is less common than other forms of PH, it is the best studied mainly because it is a well-defined subset of the disease.3,4 Sarcoidosis-associated PH is one of the harder to define PH subsets and falls into the “miscellaneous” category in the current classification, highlighting its heterogeneity and our lack of complete understanding of this condition.

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