The Effects of Epidermal Growth Factor Receptor (EGFR) Inhibition on Pulmonary Arterial Hypertension Associated with Systemic Sclerosis A phase II controlled open-label safety and efficacy study.
- Conditions
- high pulmonary vascular pressure associated with scleroderma100192801001076110037454
- Registration Number
- NL-OMON30491
- Lead Sponsor
- Vrije Universiteit Medisch Centrum
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Withdrawn
- 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)
17. Severe interstitial fibrosis on HRCT
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>To describe the safety of cetuximab in scleroderma associated PAH. </p><br>
- Secondary Outcome Measures
Name Time Method <p>To explore the efficacy of cetuximab in terms of: stroke volume, 6 minute walk<br /><br>test, changes on HRCT, changes in nailfold microcirculation, changes in<br /><br>molecular parameters (NT-proBNP) </p><br>