Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
- Conditions
- Pulmonary Arterial HypertensionSclerodermaSystemic SclerosisPulmonary Hypertension
- Registration Number
- NCT00377949
- Lead Sponsor
- Georgetown University
- Brief Summary
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.
- Detailed Description
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.
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- All
- Target Recruitment
- 602
Not provided
Not provided
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method Pulmonary Hypertension Progression 10 years The primary objective of the study is to determine the timeline of progression from pre-pulmonary hypertension to diagnosable pulmonary hypertension based on right heart catheterization
- Secondary Outcome Measures
Name Time Method
Trial Locations
- Locations (25)
University of Massachussetts Memorial Medical Center
🇺🇸Worcester, Massachusetts, United States
University of Medicine and Dentistry of New Jersey
🇺🇸New Brunswick, New Jersey, United States
Northwestern University
🇺🇸Chicago, Illinois, United States
University of Chicago
🇺🇸Chicago, Illinois, United States
UCLA Medical Center
🇺🇸Los Angeles, California, United States
John Hopkins University Medical Center
🇺🇸Baltimore, Maryland, United States
Stanford University
🇺🇸Stanford, California, United States
Boston University Medical School
🇺🇸Boston, Massachusetts, United States
National Jewish Medical and Research Center
🇺🇸Denver, Colorado, United States
Georgetown University Medical Center
🇺🇸Washington, District of Columbia, United States
Louisiana State University Health Science Center
🇺🇸New Orleans, Louisiana, United States
Tufts Medical Center
🇺🇸Boston, Massachusetts, United States
University of Michigan-Scleroderma Program
🇺🇸Ann Arbor, Michigan, United States
Hennepin County Medical Center
🇺🇸Minneapolis, Minnesota, United States
Center for Rheumatology
🇺🇸Albany, New York, United States
University of Minnesota
🇺🇸Minneapolis, Minnesota, United States
North Shore Long Island Jewish Medical Center
🇺🇸New Hyde Park, New York, United States
University of Pennsylvania
🇺🇸Philadelphia, Pennsylvania, United States
University of Pittsburgh
🇺🇸Pittsburgh, Pennsylvania, United States
Hospital for Special Surgery
🇺🇸New York, New York, United States
Cornell University
🇺🇸New York, New York, United States
Medical University of South Carolina
🇺🇸Charleston, South Carolina, United States
The University of Texas Health Science Center
🇺🇸Houston, Texas, United States
University of Utah
🇺🇸Salt Lake City, Utah, United States
The Medical College of Wisconsin
🇺🇸Milwaukee, Wisconsin, United States