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Pulmonary Arterial Hypertension in Patients with Sarcoidosis: The Pulsar Single Center Experience

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Respiratory Regulation - Clinical Advances

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 755))

Abstract

Sarcoidosis is a systemic granulomatous disease with unknown etiology. Lungs and lymph nodes are commonly affected. Also, cases of pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are described. However, the exact prevalence of PAH in patients with sarcoidosis is unclear. A 111 patients with proven sarcoidosis were recruited from January 2010 to October 2010. All patients were studied prospectively by transthoracic echocardiography (TTE) for the presence of PH. In assumed PH, a right heart catheterization (RHC) followed if there were no other reasons for PH. In 23 of the 111 patients (21%) PH was assumed in TTE. Three patients presented with severe mitral insufficiency III° and IV°, in eight patients PH was supposed to be caused by chronic heart failure or relevant diastolic dysfunction > II°, two patients declined undergoing RHC. Of the ten patients investigated with RHC, four showed a precapillary pulmonary arterial hypertension and in one patient a postcapillary hypertension was diagnosed. All four patients with precapillary PH had a radiologic stage III and IV. In three of the four patients a significantly reduced transfer factor for carbon monoxide (TLCO) <50% was found. All patients with precapillary PH had a chronic course of sarcoidosis lasting ≥13 years. This is the first study which prospectively investigated a large cohort of patients with sarcoidosis for the prevalence of PH and PAH. The prevalence of precapillary PH was found to be at least 3.6% (4/111) and therefore exceeds the prevalence of PAH in the normal population by far. A chronic and progressive lung involvement due to sarcoidosis seems to be the most evident risk factor for developing a sarcoidosis PH.

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Acknowledgements

We thank Simone Krämer (Medical Clinic II, University Hospital Bonn) for her excellent technical support. We wish to thank all patients who have participated in this study. This work was supported by an unrestricted grant from Pfizer.

Conflicts of interest: The authors declare no conflicts of interest in relation to this article.

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Authors and Affiliations

  1. Medical Clinic II, Division of Pneumology, University Hospital of Bonn, Sigmund-Freud-Str. 25, 53105, Bonn, Germany

    Stefan Pabst, Christoph Hammerstingl, Natalie Grau, Jens Kreuz, Uwe R. Juergens, Georg Nickenig & Dirk Skowasch

  2. Department of Pneumology, Evangelische Lungenklinik, Lindenberger Weg 27, Haus 205, 13125, Berlin, Germany

    Christian Grohe

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  1. Stefan Pabst
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Correspondence to Stefan Pabst .

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  1. Medical Research Center, Respiratory Research, Polish Academy of Sciences, Pawinskiego 5, Warsaw, 02-106, Poland

    Mieczyslaw Pokorski

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Pabst, S. et al. (2013). Pulmonary Arterial Hypertension in Patients with Sarcoidosis: The Pulsar Single Center Experience. In: Pokorski, M. (eds) Respiratory Regulation - Clinical Advances. Advances in Experimental Medicine and Biology, vol 755. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4546-9_38

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