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Multi-Center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
This study is currently recruiting participants.
Verified by Georgetown University, October 2008
Sponsored by: Georgetown University
Information provided by: Georgetown University
ClinicalTrials.gov Identifier: NCT00377949
  Purpose

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.


Condition
Systemic Sclerosis
Scleroderma
Pulmonary Hypertension
Pulmonary Arterial Hypertension

Genetics Home Reference related topics: pulmonary arterial hypertension
MedlinePlus related topics: High Blood Pressure Pulmonary Hypertension Scleroderma
U.S. FDA Resources
Study Type: Observational
Study Design: Case-Only, Prospective
Official Title: The Natural History and Outcome of Patients With Scleroderma at High Risk for or With Early Pulmonary Hypertension

Further study details as provided by Georgetown University:

Estimated Enrollment: 400
Study Start Date: February 2005
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.

  Eligibility

Ages Eligible for Study:   18 Years to 75 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population

Primary care, rheumatology and pulmonary hypertension clinics.

Criteria

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.

Inclusion Criteria

  • Diagnosis of "pre" pulmonary hypertension defined as:
  • Echocardiogram with a resting SPAP of >40 mmHg Or
  • Pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLCO ratio >1.6
  • Diagnosis of definite pulmonary hypertension
  • Patients with symptomatic pulmonary hypertension with a right heart catheterization showing a mean PA pressure > 25mmHg and diagnosed in the past 6 months.

Exclusion Criteria:

  • Diagnosis and treatment of pulmonary hypertension for > 6 months.
  Contacts and Locations
Please refer to this study by its ClinicalTrials.gov identifier: NCT00377949

Contacts
Contact: Aida Manu 202-687-6318 amanu@georgetown.edu
Contact: Catherine Ford 202-687-6317 cf283@georgetown.edu

Locations
United States, California
UCLA Medical Center Recruiting
Los Angeles, California, United States, 90095
Contact: Amber Betchel     310-825-0425     abetchel@mednet.ucla.edu    
Principal Investigator: Daniel Furst, MD            
Stanford University Recruiting
Stanford, California, United States, 94305
Contact: Louise McManus     650-493-5000 ext 64571     louise.mcmanus@va.gov    
Principal Investigator: Lorinda Chung, MD            
United States, Colorado
National Jewish Medical and Research Center Recruiting
Denver, Colorado, United States, 80206
Contact: Shontel Sarmiento         SarmientoS@njc.org    
Principal Investigator: Aryeh Fischer, MD            
United States, District of Columbia
Georgetown University Medical Center Recruiting
Washington, District of Columbia, United States, 20007
Contact: Aida Manu     202-687-6318     anm42@georgetown.edu    
United States, Illinois
Northwestern University Recruiting
Chicago, Illinois, United States, 60611
Contact: Anabel Garza     312-503-0377     a-garza@northwestern.edu    
Principal Investigator: Monique Hinchcliff, MD            
United States, Maryland
John Hopkins University Medical Center Recruiting
Baltimore, Maryland, United States, 21224
Contact: Adrienne Woods     410-550-6820     awoods9@jhmi.edu    
Sub-Investigator: Laura Hummers, MD            
Principal Investigator: Fred Wigley, MD            
United States, Massachusetts
Boston University Medical School Recruiting
Boston, Massachusetts, United States, 02118
Contact: Kim Finch     617-638-7503     kimtobin@bu.edu    
Principal Investigator: Robert Simms, MD            
University of Massachussetts Memorial Medical Center Recruiting
Worcester, Massachusetts, United States, 01605
Contact: Firas Alkassab, MD         AlkassaF@ummhc.org    
Principal Investigator: Firas Alkassab, MD            
United States, Michigan
University of Michigan-Scleroderma Program Recruiting
Ann Arbor, Michigan, United States, 48109
Contact: Ann Impens     734-936-1439     aimpens@med.umich.edu    
Contact: Matthew Shankin     734-232-2090     shankinm@med.umich.edu    
Principal Investigator: James Seibold, MD            
United States, Minnesota
University of Minnesota Recruiting
Minneapolis, Minnesota, United States, 55455
Contact: Valerie Ferment     612-624-9038     ferm0016@umn.edu    
Principal Investigator: Barbara Segal, MD            
United States, New Jersey
University of Medicine and Dentistry of New Jersey Recruiting
New Brunswick, New Jersey, United States, 08903
Contact: Julianne Wilson     732-418-8483     wilsonj6@umdnj.edu    
Principal Investigator: Vivien Hsu, MD            
United States, New York
Center for Rheumatology Recruiting
Albany, New York, United States, 12206
Contact: Justine Feder-Lailer     518-533-1349     jfeder-lailer@joint-docs.com    
Principal Investigator: Lee Shapiro, MD            
North Shore Long Island Jewish Medical Center Recruiting
New Hyde Park, New York, United States, 11040
Contact: Michelle Kong     516-708-2557     MKong@NSHS.edu    
Principal Investigator: Avram Goldberg, MD            
United States, Pennsylvania
Thomas Jefferson University Recruiting
Philadelphia, Pennsylvania, United States, 19107
Contact: Chris Derk, MD         chris.derk@jefferson.edu    
Principal Investigator: Chris Derk, MD            
University of Pittsburgh Recruiting
Pittsburgh, Pennsylvania, United States, 15261
Contact: Robyn Domsic, MD         rtd4@pitt.edu    
Principal Investigator: Thomas Medsger, MD            
Sub-Investigator: Robyn Domsic, MD            
United States, South Carolina
Medical University of South Carolina Recruiting
Charleston, South Carolina, United States, 29425
Contact: Donna Jordan     843-792-5290     jordands@musc.edu    
Principal Investigator: Marcy Bolster, MD            
United States, Texas
The University of Texas Health Science Center Recruiting
Houston, Texas, United States, 77030
Contact: Marcia Kerr     713-500-7118     Marcia.L.Kerr@uth.tmc.edu    
Principal Investigator: Maureen D Mayes, MD            
United States, Wisconsin
The Medical College of Wisconsin Recruiting
Milwaukee, Wisconsin, United States, 53226
Contact: Mary E Csuka, MD         mecsuka@mcw.edu    
Principal Investigator: Mary E Csuka, MD            
Sponsors and Collaborators
Georgetown University
Investigators
Principal Investigator: Virginia D. Steen, MD Georgetown University
  More Information

Publications:
Young RH, Mark GJ. Pulmonary vascular changes in scleroderma. Am J Med. 1978 Jun;64(6):998-1004.
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.
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.
Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996 Feb 1;334(5):296-302.
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.
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.
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.
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.
Yousem SA. The pulmonary pathologic manifestations of the CREST syndrome. Hum Pathol. 1990 May;21(5):467-74. Review.

Responsible Party: Georgetown University ( Virginia Steen, MD )
Study ID Numbers: IRB # 04-227
Study First Received: September 18, 2006
Last Updated: October 6, 2008
ClinicalTrials.gov Identifier: NCT00377949  
Health Authority: United States: Institutional Review Board

Keywords provided by Georgetown University:
Systemic Sclerosis
Scleroderma
Pulmonary Hypertension
PHAROS
PHROS
Exercise Echocardiogram
Pulmonary Functional Tests
Six minute walk tests
6 minute walk tests
Right heart catheterization

Study placed in the following topic categories:
Idiopathic pulmonary hypertension
Skin Diseases
Respiratory Tract Diseases
Hypertension, Pulmonary
Lung Diseases
Vascular Diseases
Connective Tissue Diseases
Sclerosis
Scleroderma, Systemic
Scleroderma, Localized
Hypertension

Additional relevant MeSH terms:
Pathologic Processes
Cardiovascular Diseases

ClinicalTrials.gov processed this record on January 15, 2009