Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients



Status:Completed
Conditions:High Blood Pressure (Hypertension), Skin and Soft Tissue Infections, Neurology, Dermatology
Therapuetic Areas:Cardiology / Vascular Diseases, Dermatology / Plastic Surgery, Neurology
Healthy:No
Age Range:18 - 75
Updated:5/4/2016
Start Date:February 2005

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The Natural History and Outcome of Patients With Scleroderma at High Risk for or With Early Pulmonary Hypertension

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. Global Inclusion Criteria

- Eligible patients must meet all of the following inclusion criteria:

- Patient ≥ 18 years with a clinical diagnosis of SSc (ACR criteria or the LeRoy
criteria for limited or diffuse scleroderma

2. Specific Inclusion Criteria

- Diagnosis of "pre" pulmonary arterial hypertension defined as:

- Echocardiogram with a resting sPAP of ≥ 40mmHg Or

- Pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLco
ratio >1.6. or

- Right heart catheterization which shows or a mean PA pressure > 30mmHg with
exercise (with a mPAP < 25mmHg at rest)

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.

- Diagnosis of definite pulmonary hypertension Patients with pulmonary hypertension
with a right heart catheterization showing a mean PA pressure > 25mmHg, diagnosed in
the past 6 months.

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

- Diagnosis and treatment of pulmonary hypertension for > 6 months

- Patients with known severe interstitial fibrosis, pulmonary thrombotic disease, heart
failure, cardiomyopathy,history of coronary artery disease or other cardio-pulmonary
problems which could cause pulmonary hypertension are not eligible for the
'pre'-pulmonary hypertension but do qualify for the definite pulmonary hypertension
group if they have a right heart catheterization showing a mean PAH >25mmHg.
We found this trial at
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8701 W Watertown Plank Rd
Milwaukee, Wisconsin
(414) 955-8296
Medical College of Wisconsin The Medical College (MCW) of Wisconsin is a major national research...
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800 Washington St
Boston, Massachusetts 02111
(617) 636-5000
Tufts Medical Center Tufts Medical Center is an internationally-respected academic medical center – a teaching...
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5801 South Ellis Avenue
Chicago, Illinois 60637
 773.702.1234
University of Chicago One of the world's premier academic and research institutions, the University of...
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Minneapolis, Minnesota 55455
(612) 625-5000
Univ of Minnesota With a flagship campus in the heart of the Twin Cities, and...
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4200 Fifth Ave
Pittsburgh, Pennsylvania 15260
(412) 624-4141
University of Pittsburgh The University of Pittsburgh is a state-related research university, founded as the...
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201 Presidents Circle
Salt Lake City, Utah 84108
801) 581-7200
University of Utah Research is a major component in the life of the U benefiting...
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Albany, New York 12206
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Ann Arbor, Michigan 48109
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Baltimore, Maryland 21224
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Boston, Massachusetts 02118
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171 Ashley Avenue
Charleston, South Carolina 29425
843-792-1414
Medical University of South Carolina The Medical University of South Carolina (MUSC) has grown from...
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303 East Superior Street
Chicago, Illinois 60611
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757 Westwood Plaza
Los Angeles, California 90024
(310) 825-9111
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Minneapolis, Minnesota 55414
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New Brunswick, New Jersey 08901
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New Hyde Park, New York 11040
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New York, New York 10021
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535 E 70th St
New York, New York 10021
(212) 606-1000
Hospital for Special Surgery Founded in 1863, Hospital for Special Surgery is the nation
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3451 Walnut St
Philadelphia, Pennsylvania 19104
1 (215) 898-5000
Univ of Pennsylvania Penn has a long and proud tradition of intellectual rigor and pursuit...
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450 Serra Mall
Stanford, California 94305
(650) 723-2300
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3800 Reservoir Rd NW
Washington, District of Columbia 20007
(202) 687-7695
Georgetown University Medical Center Georgetown University Medical Center is committed to excellence in research, education...
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