Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
The purpose of this study is to determine the timeline of progression from pre-pulmonary hypertension to diagnosable pulmonary hypertension based on right heart catheterization. Moreover, to determine the timeline for progression from diagnosable pulmonary hypertension to clinical worsening of disease as defined as death, hospitalization, or worsening of PHT symptoms.
Pulmonary Arterial Hypertension
|Study Design:||Observational Model: Case-Only
Time Perspective: Prospective
|Official Title:||The Natural History and Outcome of Patients With Scleroderma at High Risk for or With Early Pulmonary Hypertension|
|Study Start Date:||February 2005|
Systemic sclerosis (SSc) is a rare, often fatal idiopathic disease, which has no effective therapy. One of the most major complications of systematic sclerosis is pulmonary hypertension (PHT), which is now the cause of all scleroderma related deaths. New therapeutic advances have improved short-term management of pulmonary hypertension in scleroderma, but long-term outcomes are unknown. With this in mind, Dr. Steen has developed Pulmonary Hypertension Assessment Registry of Scleroderma (PHAROS), a preventive, multi-center, web based observational study that looks at the natural history and outcome of scleroderma patients who are at high risk or have early pulmonary hypertension. Patients entered into the registry will be followed in prospective fashion noting the clinical course of disease by both scheduled and event driven follow up. A thorough baseline history will be collected to determine key prognostic and correlative factors for both disease prevalence and progression. Yearly follow up consisting of questionnaires, pulmonary function tests, echocardiogram, 6 minute walk tests and predefined patient characteristics will also be conducted to further understand and note the progression of scleroderma related PAH. Event driven follow up will occur to record findings and record specific predetermined events in the clinical course of disease.
|Contact: Shelley Moorefirstname.lastname@example.org|
|Contact: Molly Lurie-Marinoemail@example.com|
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|Principal Investigator:||Virginia D. Steen, MD||Georgetown University|