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

This study is currently recruiting participants. (see Contacts and Locations)
Verified August 2014 by Georgetown University
Sponsor:
Collaborator:
Gilead Sciences
Information provided by (Responsible Party):
Virginia Steen, MD, Georgetown University
ClinicalTrials.gov Identifier:
NCT00377949
First received: September 18, 2006
Last updated: August 18, 2014
Last verified: August 2014
  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

Study Type: Observational
Study Design: Observational Model: Case-Only
Time Perspective: Prospective
Official Title: The Natural History and Outcome of Patients With Scleroderma at High Risk for or With Early Pulmonary Hypertension

Resource links provided by NLM:


Further study details as provided by Georgetown University:

Estimated Enrollment: 400
Study Start Date: February 2005
Estimated Primary Completion Date: January 2016 (Final data collection date for primary outcome measure)
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
  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.
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00377949

Contacts
Contact: Carolyn Fridley, BS 202-444-6211 cef65@georgetown.edu
Contact: Maia Zulmatashvili, BS 202-444-6210 mz381@georgetown.edu

  Hide Study Locations
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         
Sub-Investigator: Dinesh Khanna, MD, MS         
Stanford University Recruiting
Stanford, California, United States, 94305
Contact: Jennifer Hughes       jennifer.hughes12@gmail.com   
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    202-444-6211    anm42@georgetown.edu   
United States, Illinois
Northwestern University Recruiting
Chicago, Illinois, United States, 60611
Contact: Sofia Podlusky       fiapod@yahoo.com   
Contact: Anabel Garza    312-503-0377    a-garza@northwestern.edu   
Principal Investigator: Monique Hinchcliff, MD         
University of Chicago Recruiting
Chicago, Illinois, United States, 60637
Contact: Mardi Gomberg-Maitland, MD       mgomberg@medicine.bsd.uchicago.edu   
Principal Investigator: Mardi Gomberg-Maitland, MD         
United States, Louisiana
Louisiana State University Health Science Center Not yet recruiting
New Orleans, Louisiana, United States, 70112
Contact: Paula Lauto       PLauto@lsuhsc.edu   
Principal Investigator: Lesley Ann Saketkoo, MD MPH         
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         
Tufts Medical Center Recruiting
Boston, Massachusetts, United States, 02111
Contact: Karen Visnaw, RN    617-636-1334    Kvisnaw@tuftsmedicalcenter.org   
Principal Investigator: Nicholas Hill, 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: Julie Konkle, BSN, RN, CCRP    734-936-4555    jkonkle@med.umich.edu   
Principal Investigator: Dinesh Khanna, MD         
United States, Minnesota
University of Minnesota Recruiting
Minneapolis, Minnesota, United States, 55455
Contact: Valerie Ferment    612-624-9038    ferm0016@umn.edu   
Principal Investigator: Jerry Molitor, MD         
Hennepin County Medical Center Recruiting
Minneapolis, Minnesota, United States, 55415
Principal Investigator: Barbara Segal         
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: Donna Ammirati    516-708-2556    Dammirati@NSHS.edu   
Principal Investigator: Avram Goldberg, MD         
Cornell University Recruiting
New York, New York, United States, 10065
Contact: Madeline Yushak    212-746-2698    yushakm@med.cornell.edu   
Principal Investigator: Evelyn Horn, MD         
Hospital for Special Surgery Recruiting
New York, New York, United States, 10021
Contact: Kamini Doobay    212-774-2123    DoobayK@HSS.EDU   
Principal Investigator: Jessica Gordon, MD         
United States, Pennsylvania
University of Pennsylvania Not yet recruiting
Philadelphia, Pennsylvania, United States, 19104
Contact: Leah Madden, CCRP    215-614-4406    Leah.Madden@uphs.upenn.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: Dana Rosson    843-792-5290    rosson@musc.edu   
Principal Investigator: Marcy Bolster, MD         
United States, Texas
The University of Texas Health Science Center Recruiting
Houston, Texas, United States, 77030
Contact: Ann Saulino    713-500-7118    A.R.Saulino@uth.tmc.edu   
Principal Investigator: Maureen D Mayes, MD         
United States, Utah
University of Utah Recruiting
Salt Lake City, Utah, United States, 84132
Contact: Martha Finco, MS    801-585-6468    Martha.Finco@hsc.utah.edu   
Principal Investigator: Tracy Frech, 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
Gilead Sciences
Investigators
Principal Investigator: Virginia D. Steen, MD Georgetown University
  More Information

Publications:

Responsible Party: Virginia Steen, MD, Project Principal Investigator, Georgetown University
ClinicalTrials.gov Identifier: NCT00377949     History of Changes
Other Study ID Numbers: IRB # 04-227
Study First Received: September 18, 2006
Last Updated: August 18, 2014
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

Additional relevant MeSH terms:
Hypertension, Pulmonary
Scleroderma, Systemic
Scleroderma, Diffuse
Scleroderma, Localized
Hypertension
Sclerosis
Lung Diseases
Respiratory Tract Diseases
Connective Tissue Diseases
Skin Diseases
Vascular Diseases
Cardiovascular Diseases
Pathologic Processes

ClinicalTrials.gov processed this record on August 28, 2014