Open Badges Commentary

Systemic sclerosis-associated pulmonary hypertension: why disease-specific composite endpoints are needed

Christopher P Denton1*, Jerome Avouac2, Frank Behrens3, Daniel E Furst4, Ivan Foeldvari5, Marc Humbert6, Doerte Huscher7, Otylia Kowal-Bielecka8, Marco Matucci-Cerinic9, Peter Nash10, Christian F Opitz11, David Pittrow12, Lewis J Rubin13, James R Seibold14 and Oliver Distler15

Author Affiliations

1 Centre for Rheumatology, Royal Free Campus, University College Medical School, London, NW3 2PF, UK

2 Rheumatology A Department, RDU, Paris, 75006, France

3 German Rheumatism Research Centre, 10117 Berlin, Germany

4 Division of Rheumatology, Department of Medicine, David Geffen School at UCLA, Los Angeles, CA 90095, USA

5 Pediatric Rheumatology Clinic, General Hospital Eilbek, 22081 Germany

6 Service de Pneumologie et Reanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, Hopital Antoine-Beclere, Université Paris-Sud, Clamart, 92140, France

7 Division of Rheumatology/ZAFES, JW Goethe University, 60325 Frankfurt, Germany

8 Department of Rheumatology and Internal Medicine, Medical University of Bialystok, 15-089, Poland

9 Department of Medicine, Division of Rheumatology, Denothe Center, University of Florence, 50134, Italy

10 Rheumatology Research Unit, Cotton Tree, Sunshine Coast, Queensland, 4558, Australia

11 Klinik für Innere Medizin, DRK-Kliniken Berlin Köpenick, 12559 Berlin, Germany

12 Institute for Clinical Pharmacology, Medical Faculty, Technical University, 01187 Dresden, Germany

13 Division of Pulmonary and Critical Care Medicine, University of California, San Diego School of Medicine, La Jolla, CA 92093-0348 USA

14 Scleroderma Research Consultants, CT 06001, USA

15 Department of Rheumatology, University Hospital Zurich, CH-8091, Switzerland

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Arthritis Research & Therapy 2011, 13:114  doi:10.1186/ar3346

Published: 20 June 2011


Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc). In clinical trials PAH-SSc has been grouped with other forms, including idiopathic PAH. The primary endpoint for most pivotal studies was improvement in exercise capacity. However, composite clinical endpoints that better reflect long-term outcome may be more meaningful. We discuss potential endpoints and consider why the same measures may not be appropriate for both idiopathic PAH and PAH-SSc due to inherent differences in clinical outcome and management strategies of these two forms of PAH. Failure to take this into account may compromise progress in managing PAH in SSc.