Rheumatology Advance Access originally published online on August 18, 2006
Rheumatology 2006 45(12):1455-1457; doi:10.1093/rheumatology/kel273
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
EDITORIALS |
Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis
Department of Rheumatology, University Hospital Zurich, Switzerland, 1Division of Rheumatology/ZAFES, J.W. Goethe University Frankfurt, 2German Rheumatism Research Centre, Berlin, 3Pediatric Rheumology Clinic, General Hospital Eilbek, Germany, 4Rheumatology Research Unit, 6th Avenue Specialist Centre Cotton Tree 4558, Sunshine Coast, Queensland, Australia, 5Centre for Rheumatology, Royal Free and University College, Medical School, London, UK, 6Service de Pneumologie et Reanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, Hopital Antoine-Beclere, Universite Paris-Sud, Clamart, France, 7Universita Degli Studie Terapia Medica I, Florence, Italy, 8University of Michigan Scleroderma Program, Ann Arbor, Michigan, 9Division of Pulmonary and Critical Care Medicine, University of California, San Diego School of Medicine, La Jolla and 10Division of Rheumatology, Department of Medicine, David Geffen School at UCLA, Los Angeles, CA, USA
Correspondence to: O. Dister, Department of Rheumatology, University Hospital Zurich, Switzerland. E-mail: Oliver.Distler@usz.ch
| The first 10% of the full text of this article appears below. |
SSc-pulmonary disease is a devastating condition
Pulmonary disease is a frequent complication of systemic sclerosis (SSc) and is at the same time challenging for the treating physician. Besides lung fibrosis, pulmonary arterial hypertension (PAH)—defined as a mean pulmonary artery pressure above 25 mmHg at rest or above 30 mmHg during exercise by right heart catheterization—can occur both in the limited and diffuse subset of SSc and leads to right cardiac overload with exercise intolerance, dyspnea and arrhythmia [1]. The natural history of PAH associated with SSc (APAH/SSc) is devastating. While 1-, 3- and 5-yr survival rates after diagnosis in the idiopathic form (IPAH) are 68, 48 and 34% (median life expectancy 2.8 yrs) in untreated patients, several studies have shown that the prognosis in APAH/SSc is even worse. Median survival in untreated patients is only 12 months, and the risk of death is nearly tripled [2
Recently some effective therapies for PAH have become available
Measures of response for PAH remain limited
Need for improved outcome measures in APAH/SSc
An approach to improving measures of response
Summary
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