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Systemic SclerosisNCT00377949

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

An observational study of Systemic Sclerosis and Scleroderma, run by Georgetown University at 25 sites in United States.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial 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… Read more about this study

Who is it for?
Ages 18 to 75 years
How many people
602 participants
Where
25 sitesUnited States
Study dates
Feb 2005to Jan 2016
Phase
Observational
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 to 75 years
  • All sexes
  • No healthy volunteers
  • Adult, Older adult

Study population: Primary care, rheumatology and pulmonary hypertension clinics.

  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
  1. 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

  1. 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.

Trial locations and contacts

25 sites in 1 country.

25 sites in 1 country

United States25 sites

  • Center for Rheumatology

    Albany, New York, 12206

  • University of Michigan-Scleroderma Program

    Ann Arbor, Michigan, 48109

  • John Hopkins University Medical Center

    Baltimore, Maryland, 21224

  • Tufts Medical Center

    Boston, Massachusetts, 02111

  • Boston University Medical School

    Boston, Massachusetts, 02118

  • Medical University of South Carolina

    Charleston, South Carolina, 29425

  • Northwestern University

    Chicago, Illinois, 60611

  • University of Chicago

    Chicago, Illinois, 60637

  • National Jewish Medical and Research Center

    Denver, Colorado, 80206

  • The University of Texas Health Science Center

    Houston, Texas, 77030

  • UCLA Medical Center

    Los Angeles, California, 90095

  • The Medical College of Wisconsin

    Milwaukee, Wisconsin, 53226

  • Hennepin County Medical Center

    Minneapolis, Minnesota, 55415

  • University of Minnesota

    Minneapolis, Minnesota, 55455

  • University of Medicine and Dentistry of New Jersey

    New Brunswick, New Jersey, 08903

  • North Shore Long Island Jewish Medical Center

    New Hyde Park, New York, 11040

  • Louisiana State University Health Science Center

    New Orleans, Louisiana, 70112

  • Hospital for Special Surgery

    New York, New York, 10021

  • Cornell University

    New York, New York, 10065

  • University of Pennsylvania

    Philadelphia, Pennsylvania, 19104

  • University of Pittsburgh

    Pittsburgh, Pennsylvania, 15261

  • University of Utah

    Salt Lake City, Utah, 84132

  • Stanford University

    Stanford, California, 94305

  • Georgetown University Medical Center

    Washington D.C., District of Columbia, 20007

  • University of Massachussetts Memorial Medical Center

    Worcester, Massachusetts, 01605

Other trials in United States

About this study

Official title: 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.

Read the 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.

Keywords
Systemic Sclerosis, Scleroderma, Pulmonary Hypertension, PHAROS, PHROS, Exercise Echocardiogram, Pulmonary Functional Tests, Six minute walk tests, 6 minute walk tests, Right heart catheterization
Medical subject headings
Scleroderma, Systemic, Scleroderma, Diffuse, Hypertension, Pulmonary, Pulmonary Arterial Hypertension

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. 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

    Measured: 10 years

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted19 Sep 2006 (estimated)
  2. Study startFeb 2005
  3. Primary completionJan 2016

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Observational
Observational model
Case only
Time perspective
Prospective
Enrolment
602 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT00377949
Sponsor's protocol ID
IRB # 04-227
Status last verified
May 2016
First submitted
18 Sep 2006
Last updated
3 May 2016
Results posted
No
Data monitoring committee
No

Publications

  • Young RH, Mark GJ. Pulmonary vascular changes in scleroderma. Am J Med. 1978 Jun;64(6):998-1004. doi: 10.1016/0002-9343(78)90455-2.

    PubMed 148843

  • Salerni R, Rodnan GP, Leon DF, Shaver JA. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). Ann Intern Med. 1977 Apr;86(4):394-9. doi: 10.7326/0003-4819-86-4-394.

    PubMed 848800

  • Stupi AM, Steen VD, Owens GR, Barnes EL, Rodnan GP, Medsger TA Jr. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum. 1986 Apr;29(4):515-24. doi: 10.1002/art.1780290409.

    PubMed 3707629

  • Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, Koerner SK, Langleben D, Keller CA, Murali S, Uretsky BF, Clayton LM, Jobsis MM, Blackburn SD, Shortino D, Crow JW; Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996 Feb 1;334(5):296-301. doi: 10.1056/NEJM199602013340504.

    PubMed 8532025

  • Steen VD, Ziegler GL, Rodnan GP, Medsger TA Jr. Clinical and laboratory associations of anticentromere antibody in patients with progressive systemic sclerosis. Arthritis Rheum. 1984 Feb;27(2):125-31. doi: 10.1002/art.1780270202.

    PubMed 6607734

  • Murata I, Takenaka K, Yoshinoya S, Kikuchi K, Kiuchi T, Tanigawa T, Ito K. Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. A Doppler echocardiographic study of 135 Japanese patients. Chest. 1997 Jan;111(1):36-43. doi: 10.1378/chest.111.1.36.

    PubMed 8995990

  • Denton CP, Cailes JB, Phillips GD, Wells AU, Black CM, Bois RM. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997 Feb;36(2):239-43. doi: 10.1093/rheumatology/36.2.239.

    PubMed 9133938

  • MacGregor AJ, Canavan R, Knight C, Denton CP, Davar J, Coghlan J, Black CM. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). 2001 Apr;40(4):453-9. doi: 10.1093/rheumatology/40.4.453.

    PubMed 11312386

  • Yousem SA. The pulmonary pathologic manifestations of the CREST syndrome. Hum Pathol. 1990 May;21(5):467-74. doi: 10.1016/0046-8177(90)90002-m.

    PubMed 2186993

Questions about this trial

What is this clinical trial studying?

An observational study of Systemic Sclerosis and Scleroderma, run by Georgetown University at 25 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 3 May 2016. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 25 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Pulmonary Hypertension Progression (time frame: 10 years). It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT00377949, which also lists 9 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 3 May 2016.

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