Status:
TERMINATED
Early, Simple and Reliable Detection of Pulmonary Arterial Hypertension (PAH) in Systemic Sclerosis (SSc)
Lead Sponsor:
Actelion
Conditions:
Systemic Sclerosis
Pulmonary Arterial Hypertension
Eligibility:
All Genders
18+ years
Brief Summary
A two-stage prospective observational cohort study in scleroderma patients to evaluate screening tests and the incidence of pulmonary arterial hypertension and pulmonary hypertension
Eligibility Criteria
Inclusion
- Inclusion criteria
- Male or female
- Age ≥ 18 years
- Patients with definite diagnosis of SSc by American College of Rheumatology (ACR) criteria (18); including all patients with any other connective tissue diseases (CTD) who, in parallel, meet the ACR criteria for SSc (18)
- SSc disease duration \> 3 years dated from onset of first non-Raynaud feature
- Diffusing capacity of the lung for carbon monoxide (DLCO) \< 60% of predicted
- Exclusion criteria
- PH confirmed by RHC before enrolment, i.e. mean pulmonary arterial pressure (mPAP) \> or = 25 mmHg at rest or \> or = 30 mmHg at exercise, independent of PCWP (11)
- RHC within the 12 months before enrolment
- Use of therapy that is considered definite PAH/PH treatment (19) for any indication, within the 6 weeks before enrolment and/or for a total of more than 6 weeks during the 12 months before enrolment: i.e. endothelin receptor antagonists (ERA; e.g. bosentan, sitaxsentan, ambrisentan), phosphodiesterase type 5 inhibitors (PDE5; e.g. sildenafil, tadalafil, vardenafil), prostanoids (e.g. epoprostenol, treprostinil, iloprost, beraprost), and any experimental PAH/PH drugs. Intermittent use of PDE5 inhibitors for male erectile dysfunction is permitted
- Forced vital capacity (FVC) \< 40%
- Renal insufficiency, defined as estimated glomerular filtration rate (eGFR) \< 40 ml/min/1.73 m2 (20), assessed according to local practice
- Previous evidence or previous diagnosis of clinically relevant left heart disease and other relevant conditions, i.e. at least one of the following:
- Previous ECHO with estimated left ventricular (LV) ejection fraction \< 50%, previous history of cardiogenic pulmonary edema, increased size of left atrium (\> 50 mm)
- Known significant diastolic dysfunction associated with clinical heart failure or PCWP \> 15mmHg
- Known significant coronary disease or significant valvular heart disease
- Evidence of inadequately treated blood pressure, defined as \> 160/90 mmHg and/or blood pressure during exercise \> 220/120 mmHg (if evaluated)
- Known hypertrophic cardiomyopathy or left ventricular hypertrophy (interventricular septum thickness (IVS) or posterior wall thickness (PWD) \> 1.2 cm)
- Patients referred with overt heart failure
- Known congenital heart defects such as single ventricle, transposition, or Eisenmenger physiology
- Previous closure of systemic pulmonary shunt, heart valve replacement, or cardiac transplantation
- Pregnancy: pregnancy testing in females with child-bearing potential is mandatory and must be done before enrolment
- Patients unlikely to be available for annual follow up over an anticipated 3 years of study (e.g. survival estimate, psychological, logistics; based on investigator discretion)
- Patients with clinically relevant left heart disease as defined above, diagnosed by ECHO at baseline (i.e. after enrolment), will be included in the study.
- Additional exclusion criteria after patient enrolment
- During the study, use of therapy that is considered definite PAH/PH treatment (19) is prohibited and will result in discontinuation of the patient from the study, unless the total treatment duration per year is less than 6 weeks. Intermittent use of PDE5 inhibitors for male erectile dysfunction is permitted.
- During the study, use of therapy that is considered definite PAH/PH treatment (19) is prohibited within the 6 weeks preceding the follow-up visits. Violation of this rule will result in discontinuation of the patient from the study.
Exclusion
Key Trial Info
Start Date :
October 1 2008
Trial Type :
OBSERVATIONAL
Allocation :
ACTUAL
End Date :
March 1 2012
Estimated Enrollment :
490 Patients enrolled
Trial Details
Trial ID
NCT00706082
Start Date
October 1 2008
End Date
March 1 2012
Last Update
June 25 2012
Active Locations (83)
Enter a location and click search to find clinical trials sorted by distance.
1
University of Alabama at Birmingham, 311 THT
Birmingham, Alabama, United States, 35294
2
UCLA Division of Rheumatology
Los Angeles, California, United States, 90095
3
Harbor UCLA Medical Center
Torrance, California, United States, 90509
4
University of CT
Farmington, Connecticut, United States, 06030