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

2. maj 2016 opdateret af: Virginia Steen, MD, Georgetown University

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.

Studieoversigt

Status

Afsluttet

Betingelser

Detaljeret beskrivelse

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.

Undersøgelsestype

Observationel

Tilmelding (Faktiske)

602

Kontakter og lokationer

Dette afsnit indeholder kontaktoplysninger for dem, der udfører undersøgelsen, og oplysninger om, hvor denne undersøgelse udføres.

Studiesteder

Forenede Stater
- California
  - Los Angeles, California, Forenede Stater, 90095
    - UCLA Medical Center
  - Stanford, California, Forenede Stater, 94305
    - Stanford University
- Colorado
  - Denver, Colorado, Forenede Stater, 80206
    - National Jewish Medical and Research Center
- District of Columbia
  - Washington, District of Columbia, Forenede Stater, 20007
    - Georgetown University Medical Center
- Illinois
  - Chicago, Illinois, Forenede Stater, 60611
    - Northwestern University
  - Chicago, Illinois, Forenede Stater, 60637
    - University of Chicago
- Louisiana
  - New Orleans, Louisiana, Forenede Stater, 70112
    - Louisiana State University Health Science Center
- Maryland
  - Baltimore, Maryland, Forenede Stater, 21224
    - John Hopkins University Medical Center
- Massachusetts
  - Boston, Massachusetts, Forenede Stater, 02111
    - Tufts Medical Center
  - Boston, Massachusetts, Forenede Stater, 02118
    - Boston University Medical School
  - Worcester, Massachusetts, Forenede Stater, 01605
    - University of Massachussetts Memorial Medical Center
- Michigan
  - Ann Arbor, Michigan, Forenede Stater, 48109
    - University of Michigan-Scleroderma Program
- Minnesota
  - Minneapolis, Minnesota, Forenede Stater, 55415
    - Hennepin County Medical Center
  - Minneapolis, Minnesota, Forenede Stater, 55455
    - University of Minnesota
- New Jersey
  - New Brunswick, New Jersey, Forenede Stater, 08903
    - University of Medicine and Dentistry of New Jersey
- New York
  - Albany, New York, Forenede Stater, 12206
    - Center for Rheumatology
  - New Hyde Park, New York, Forenede Stater, 11040
    - North Shore Long Island Jewish Medical Center
  - New York, New York, Forenede Stater, 10065
    - Cornell University
  - New York, New York, Forenede Stater, 10021
    - Hospital for Special Surgery
- Pennsylvania
  - Philadelphia, Pennsylvania, Forenede Stater, 19104
    - University of Pennsylvania
  - Pittsburgh, Pennsylvania, Forenede Stater, 15261
    - University of Pittsburgh
- South Carolina
  - Charleston, South Carolina, Forenede Stater, 29425
    - Medical University of South Carolina
- Texas
  - Houston, Texas, Forenede Stater, 77030
    - The University of Texas Health Science Center
- Utah
  - Salt Lake City, Utah, Forenede Stater, 84132
    - University of Utah
- Wisconsin
  - Milwaukee, Wisconsin, Forenede Stater, 53226
    - The Medical College of Wisconsin

Deltagelseskriterier

Forskere leder efter personer, der passer til en bestemt beskrivelse, kaldet berettigelseskriterier. Nogle eksempler på disse kriterier er en persons generelle helbredstilstand eller tidligere behandlinger.

Berettigelseskriterier

Aldre berettiget til at studere

18 år til 75 år (Voksen, Ældre voksen)

Tager imod sunde frivillige

Ingen

Køn, der er berettiget til at studere

Alle

Prøveudtagningsmetode

Ikke-sandsynlighedsprøve

Studiebefolkning

Primary care, rheumatology and pulmonary hypertension clinics.

Beskrivelse

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.

Studieplan

Dette afsnit indeholder detaljer om studieplanen, herunder hvordan undersøgelsen er designet, og hvad undersøgelsen måler.

Hvordan er undersøgelsen tilrettelagt?

Design detaljer

Observationsmodeller: Kun etui
Tidsperspektiver: Fremadrettet

Hvad måler undersøgelsen?

Primære resultatmål

Resultatmål	Foranstaltningsbeskrivelse	Tidsramme
Pulmonary Hypertension Progression Tidsramme: 10 years	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

Samarbejdspartnere og efterforskere

Det er her, du vil finde personer og organisationer, der er involveret i denne undersøgelse.

Sponsor

Georgetown University

Samarbejdspartnere

Gilead Sciences

Efterforskere

Ledende efterforsker: Virginia D. Steen, MD, Georgetown University

Publikationer og nyttige links

Den person, der er ansvarlig for at indtaste oplysninger om undersøgelsen, leverer frivilligt disse publikationer. Disse kan handle om alt relateret til undersøgelsen.

Generelle publikationer

Datoer for undersøgelser

Disse datoer sporer fremskridtene for indsendelser af undersøgelsesrekord og resumeresultater til ClinicalTrials.gov. Studieregistreringer og rapporterede resultater gennemgås af National Library of Medicine (NLM) for at sikre, at de opfylder specifikke kvalitetskontrolstandarder, før de offentliggøres på den offentlige hjemmeside.

Studer store datoer

Studiestart

1. februar 2005

Primær færdiggørelse (Faktiske)

1. januar 2016

Datoer for studieregistrering

Først indsendt

18. september 2006

Først indsendt, der opfyldte QC-kriterier