Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
2 maggio 2016 aggiornato da: 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.
Panoramica dello studio
Stato
Completato
Descrizione dettagliata
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.
Tipo di studio
Osservativo
Iscrizione (Effettivo)
602
Contatti e Sedi
Questa sezione fornisce i recapiti di coloro che conducono lo studio e informazioni su dove viene condotto lo studio.
Luoghi di studio
-
-
California
-
Los Angeles, California, Stati Uniti, 90095
- UCLA Medical Center
-
Stanford, California, Stati Uniti, 94305
- Stanford University
-
-
Colorado
-
Denver, Colorado, Stati Uniti, 80206
- National Jewish Medical and Research Center
-
-
District of Columbia
-
Washington, District of Columbia, Stati Uniti, 20007
- Georgetown University Medical Center
-
-
Illinois
-
Chicago, Illinois, Stati Uniti, 60611
- Northwestern University
-
Chicago, Illinois, Stati Uniti, 60637
- University of Chicago
-
-
Louisiana
-
New Orleans, Louisiana, Stati Uniti, 70112
- Louisiana State University Health Science Center
-
-
Maryland
-
Baltimore, Maryland, Stati Uniti, 21224
- John Hopkins University Medical Center
-
-
Massachusetts
-
Boston, Massachusetts, Stati Uniti, 02111
- Tufts Medical Center
-
Boston, Massachusetts, Stati Uniti, 02118
- Boston University Medical School
-
Worcester, Massachusetts, Stati Uniti, 01605
- University of Massachussetts Memorial Medical Center
-
-
Michigan
-
Ann Arbor, Michigan, Stati Uniti, 48109
- University of Michigan-Scleroderma Program
-
-
Minnesota
-
Minneapolis, Minnesota, Stati Uniti, 55415
- Hennepin County Medical Center
-
Minneapolis, Minnesota, Stati Uniti, 55455
- University of Minnesota
-
-
New Jersey
-
New Brunswick, New Jersey, Stati Uniti, 08903
- University of Medicine and Dentistry of New Jersey
-
-
New York
-
Albany, New York, Stati Uniti, 12206
- Center for Rheumatology
-
New Hyde Park, New York, Stati Uniti, 11040
- North Shore Long Island Jewish Medical Center
-
New York, New York, Stati Uniti, 10065
- Cornell University
-
New York, New York, Stati Uniti, 10021
- Hospital for Special Surgery
-
-
Pennsylvania
-
Philadelphia, Pennsylvania, Stati Uniti, 19104
- University of Pennsylvania
-
Pittsburgh, Pennsylvania, Stati Uniti, 15261
- University of Pittsburgh
-
-
South Carolina
-
Charleston, South Carolina, Stati Uniti, 29425
- Medical University of South Carolina
-
-
Texas
-
Houston, Texas, Stati Uniti, 77030
- The University of Texas Health Science Center
-
-
Utah
-
Salt Lake City, Utah, Stati Uniti, 84132
- University of Utah
-
-
Wisconsin
-
Milwaukee, Wisconsin, Stati Uniti, 53226
- The Medical College of Wisconsin
-
-
Criteri di partecipazione
I ricercatori cercano persone che corrispondano a una certa descrizione, chiamata criteri di ammissibilità. Alcuni esempi di questi criteri sono le condizioni generali di salute di una persona o trattamenti precedenti.
Criteri di ammissibilità
Età idonea allo studio
Da 18 anni a 75 anni (Adulto, Adulto più anziano)
Accetta volontari sani
No
Sessi ammissibili allo studio
Tutto
Metodo di campionamento
Campione non probabilistico
Popolazione di studio
Primary care, rheumatology and pulmonary hypertension clinics.
Descrizione
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.
Piano di studio
Questa sezione fornisce i dettagli del piano di studio, compreso il modo in cui lo studio è progettato e ciò che lo studio sta misurando.
Come è strutturato lo studio?
Dettagli di progettazione
- Modelli osservazionali: Solo caso
- Prospettive temporali: Prospettiva
Cosa sta misurando lo studio?
Misure di risultato primarie
Misura del risultato |
Misura Descrizione |
Lasso di tempo |
|---|---|---|
|
Pulmonary Hypertension Progression
Lasso di tempo: 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
|
Collaboratori e investigatori
Qui è dove troverai le persone e le organizzazioni coinvolte in questo studio.
Sponsor
Collaboratori
Investigatori
- Investigatore principale: Virginia D. Steen, MD, Georgetown University
Pubblicazioni e link utili
La persona responsabile dell'inserimento delle informazioni sullo studio fornisce volontariamente queste pubblicazioni. Questi possono riguardare qualsiasi cosa relativa allo studio.
Pubblicazioni generali
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
Studiare le date dei record
Queste date tengono traccia dell'avanzamento della registrazione dello studio e dell'invio dei risultati di sintesi a ClinicalTrials.gov. I record degli studi e i risultati riportati vengono esaminati dalla National Library of Medicine (NLM) per assicurarsi che soddisfino specifici standard di controllo della qualità prima di essere pubblicati sul sito Web pubblico.
Studia le date principali
Inizio studio
1 febbraio 2005
Completamento primario (Effettivo)
1 gennaio 2016
Date di iscrizione allo studio
Primo inviato
18 settembre 2006
Primo inviato che soddisfa i criteri di controllo qualità
18 settembre 2006
Primo Inserito (Stima)
19 settembre 2006
Aggiornamenti dei record di studio
Ultimo aggiornamento pubblicato (Stima)
3 maggio 2016
Ultimo aggiornamento inviato che soddisfa i criteri QC
2 maggio 2016
Ultimo verificato
1 maggio 2016
Maggiori informazioni
Termini relativi a questo studio
Parole chiave
Termini MeSH pertinenti aggiuntivi
- Processi patologici
- Malattia cardiovascolare
- Malattie vascolari
- Malattie della pelle
- Malattie delle vie respiratorie
- Malattie polmonari
- Malattie del tessuto connettivo
- Sclerosi
- Ipertensione
- Sclerodermia, sistemica
- Sclerodermia, Diffusa
- Ipertensione arteriosa polmonare
- Ipertensione, polmonare
- Sclerodermia, localizzata
Altri numeri di identificazione dello studio
- IRB # 04-227
Queste informazioni sono state recuperate direttamente dal sito web clinicaltrials.gov senza alcuna modifica. In caso di richieste di modifica, rimozione o aggiornamento dei dettagli dello studio, contattare register@clinicaltrials.gov. Non appena verrà implementata una modifica su clinicaltrials.gov, questa verrà aggiornata automaticamente anche sul nostro sito web .