The Effects of Epidermal Growth Factor (EGFR) Inhibition on Pulmonary Arterial Hypertension Associated with Systemic Sclerosis.
- Conditions
- 18-year old patients with systemic sclerosis-associated pulmonary arterial hypertension (SScPAH)
- Registration Number
- NL-OMON27131
- Lead Sponsor
- VU University Medical Center
- Brief Summary
/A
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Suspended
- Sex
- Not specified
- Target Recruitment
- 20
A subject is eligible for inclusion in this study only if all of the following criteria apply:
1. Written informed consent;
2. Systemic sclerosis;
3. PAH with a mean PAP of above 25 mmHg measured during rest;
4. PVR above 300 dynes;
5. TLC > 70 %;
6. NYHA class III and/or 6 Minute Walk Test < 80% predicted;
7.Conventional PAH treatment and/or bosentan and/or sildenafil treatment;
8. Stability on medication during the previous 3 months (defined as stable or decrease of 6 MWT after 3 months of treatment).
A subject will be excluded from this study in case of the following criteria:
1. Left ventricular dysfunction;
2. Valvular heart disease;
3. Pericardial constriction;
4. Wedge pressure >/= 15 mmHg;
5. Chronic thromboembolic pulmonary hypertension;
6. Uncontrolled sleep apnea;
7. History of malignancies;
8. Overt right heart failure;
9. History or presence of skin ulcerations;
10. Women of child-bearing potential (WOCB) who are unwilling or unable to use contraceptives;
11. Sexually active fertile man not using effective birth control if their partners are WOCB;
12. Severe abnormality of the cornea;
13. Inadequate hematologic function defined by an absolute neutrophil count < 1,500/mm3, platelet count < 80.000/mm3 and hemoblobin level of < 9 g/dL;
14. Inadequate hepatic function defined by a total bilirubin level 1.5 times the upper limit of normal (ULN) and ASAT levels 2.5 times ULN;
15. Inadequate renal function defined by a serum creatinine level > 1,5 times ULN (alternative: Cockroft <50 ml/min);
16. Substances that inhibit CYP3A4 activity, such as rifampicin, phenytoin, ketoconazole, itraconazole (see section 6.4.5).
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method Safety.
- Secondary Outcome Measures
Name Time Method Efficacy, measured by effects on six minute walk test, stroke volume, changes n HRCT, NT-pro-BNP.