An observational study in Systemic Sclerosis, Scleroderma and Pulmonary Hypertension, sponsored by Georgetown University. Completed at 25 sites in United States. Open to participants aged 18 Years to 75 Years. Per ClinicalTrials.gov, last updated 2016-05-03.
Sponsored by Georgetown University · Observational
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.
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.
1,105 studies on the registry are indexed under Hypertension, Pulmonary; 234 are open to participants now.
This study's enrollment of 602 is above the median of 116 across 386 observational studies indexed under Hypertension, Pulmonary.
Browse Hypertension, Pulmonary studies →Georgetown University is the lead sponsor of 286 studies on the registry; 42 are open to participants now.
Of its 28 completed or terminated interventional studies of FDA-regulated products, 20 (71%) have results posted.
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Primary care, rheumatology and pulmonary hypertension clinics.
Global Inclusion Criteria
Specific Inclusion Criteria
Patients entered as a 'pre'-pulmonary arterial hypertension who then undergo right heart catheterization and are found to have pulmonary arterial hypertension, pulmonary venous hypertension or diastolic dysfunction or pulmonary hypertension secondary to interstitial lung disease will be followed as a definite PH patient and classified into the appropriate category.
Classification of PH Group 1 PAH - Patients with mPAP ≥ 25mmHg with a wedge \< 15mmHg Group 2 PVH - Patients who have a mean PA pressure ≥ 25mmHg with a wedge pressure which is > 15 mmHg Group 3 PH-ILD Patients who have a mean PA pressure ≥ 25mmHg (on right heart catheterization) who have moderate to severe interstitial fibrosis on HRCT scan with a FVC and TLC \< 65% predicted
b. Exclusion Criteria
Pulmonary Hypertension Progression
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
Time frame: 10 years
This study is completed, as verified in May 2016. You cannot join it, but the record below documents what was studied.
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