Status:

COMPLETED

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

Lead Sponsor:

Georgetown University

Collaborating Sponsors:

Gilead Sciences

Conditions:

Systemic Sclerosis

Scleroderma

Eligibility:

All Genders

18-75 years

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...

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 i...

Eligibility Criteria

Inclusion

  • 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
  • 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.

Exclusion

    Key Trial Info

    Start Date :

    February 1 2005

    Trial Type :

    OBSERVATIONAL

    Allocation :

    ACTUAL

    End Date :

    Estimated Enrollment :

    602 Patients enrolled

    Trial Details

    Trial ID

    NCT00377949

    Start Date

    February 1 2005

    Last Update

    May 3 2016

    Active Locations (25)

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    Page 1 of 7 (25 locations)

    1

    UCLA Medical Center

    Los Angeles, California, United States, 90095

    2

    Stanford University

    Stanford, California, United States, 94305

    3

    National Jewish Medical and Research Center

    Denver, Colorado, United States, 80206

    4

    Georgetown University Medical Center

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