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

NCT00377949 | Completed

• 1 other identifier: IRB # 04-227
• Study Type: observational
• Target: 602
• Locations: 1 country
• Sites: 25

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.

Conditions

Systemic Sclerosis; Scleroderma; Pulmonary Hypertension; Pulmonary Arterial Hypertension

Keywords

Systemic Sclerosis; Scleroderma; Pulmonary Hypertension; PHAROS; PHROS; Exercise Echocardiogram; Pulmonary Functional Tests; Six minute walk tests; 6 minute walk tests; Right heart catheterization

Outcome Measures

Primary Outcomes (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

  10 years

Eligibility Criteria

• Age: 18 Years - 75 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Primary care, rheumatology and pulmonary hypertension clinics.

You may qualify if:

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

You may not qualify if:

• 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.

Sponsors & Collaborators

• Georgetown University: lead
• Gilead Sciences: collaborator

Study Sites (25)

UCLA Medical Center

Los Angeles, California, 90095, United States

Location

Stanford University

Stanford, California, 94305, United States

Location

National Jewish Medical and Research Center

Denver, Colorado, 80206, United States

Location

Georgetown University Medical Center

Washington D.C., District of Columbia, 20007, United States

Location

Northwestern University

Chicago, Illinois, 60611, United States

Location

University of Chicago

Chicago, Illinois, 60637, United States

Location

Louisiana State University Health Science Center

New Orleans, Louisiana, 70112, United States

Location

John Hopkins University Medical Center

Baltimore, Maryland, 21224, United States

Location

Tufts Medical Center

Boston, Massachusetts, 02111, United States

Location

Boston University Medical School

Boston, Massachusetts, 02118, United States

Location

University of Massachussetts Memorial Medical Center

Worcester, Massachusetts, 01605, United States

Location

University of Michigan-Scleroderma Program

Ann Arbor, Michigan, 48109, United States

Location

Hennepin County Medical Center

Minneapolis, Minnesota, 55415, United States

Location

University of Minnesota

Minneapolis, Minnesota, 55455, United States

Location

University of Medicine and Dentistry of New Jersey

New Brunswick, New Jersey, 08903, United States

Location

Center for Rheumatology

Albany, New York, 12206, United States

Location

North Shore Long Island Jewish Medical Center

New Hyde Park, New York, 11040, United States

Location

Hospital for Special Surgery

New York, New York, 10021, United States

Location

Cornell University

New York, New York, 10065, United States

Location

University of Pennsylvania

Philadelphia, Pennsylvania, 19104, United States

Location

University of Pittsburgh

Pittsburgh, Pennsylvania, 15261, United States

Location

Medical University of South Carolina

Charleston, South Carolina, 29425, United States

Location

The University of Texas Health Science Center

Houston, Texas, 77030, United States

Location

University of Utah

Salt Lake City, Utah, 84132, United States

Location

The Medical College of Wisconsin

Milwaukee, Wisconsin, 53226, United States

Location

Related Publications (9)

• 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.

  PMID: 148843 BACKGROUND

• 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.

  PMID: 848800 BACKGROUND

• 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.

  PMID: 3707629 BACKGROUND

• 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.

  PMID: 8532025 BACKGROUND

• 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.

  PMID: 6607734 BACKGROUND

• 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.

  PMID: 8995990 BACKGROUND

• 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.

  PMID: 9133938 BACKGROUND

• 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.

  PMID: 11312386 BACKGROUND

• 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.

  PMID: 2186993 BACKGROUND

Biospecimen

Retention: SAMPLES WITHOUT DNA

Serum stored for future analysis

MeSH Terms

Conditions

Scleroderma, Systemic; Scleroderma, Diffuse; Hypertension, Pulmonary; Pulmonary Arterial Hypertension

Condition Hierarchy (Ancestors)

Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Virginia D. Steen, MD

  Georgetown University

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Project Principal Investigator

Study Record Dates

• First Submitted: September 18, 2006
• First Posted: September 19, 2006
• Study Start: February 1, 2005
• Primary Completion: January 1, 2016
• Last Updated: May 3, 2016

Record last verified: 2016-05

Locations

US (25)