Clinical trial • Phase III • Rare Disease|Cardiology|Nephrology

venglustat for Fabry disease

Phase III trial of venglustat for Fabry disease.

Overview

Trial Therapeutic Area
Rare Disease|Cardiology|Nephrology
Trial Disease
Fabry disease
Trial Stage
Phase III
Drug Modality
Small molecule|Peptide/protein/enzyme
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
26-04-2024
First CTIS Authorization Date
20-06-2024

Trial design

Randomised, open-label, fabrazyme (agalsidase beta) iv infusion, max daily amount reported as 1 mg/kg; replagal (agalsidase alfa) iv infusion, max daily amount reported as 0.2 mg/kg; galafold (migalastat) oral capsules, 123 mg (authorized comparator). scheduling details not specified in the ctis data.-controlled Phase III trial across 22 sites in Austria, Greece, Spain and others.

Randomised
Yes
Open Label
Yes
Comparator
Fabrazyme (agalsidase beta) IV infusion, max daily amount reported as 1 mg/kg; Replagal (agalsidase alfa) IV infusion, max daily amount reported as 0.2 mg/kg; Galafold (migalastat) oral capsules, 123 mg (authorized comparator). Scheduling details not specified in the CTIS data.
Target Sample Size
53
Trial Duration For Participant
540

Eligibility

Recruits 53 Vulnerable population is selected. A signed informed consent must be provided prior to any study-related procedures. Participants are adults (aged 18–65); no provisions for assent of minors are provided in the criteria..

Pregnancy Exclusion
Contraception for male or female participants: not pregnant or breastfeeding; no sperm donating for male participant.
Vulnerable Population
Vulnerable population is selected. A signed informed consent must be provided prior to any study-related procedures. Participants are adults (aged 18–65); no provisions for assent of minors are provided in the criteria.

Inclusion criteria

  • {"criterion_text":"- Male and female participants aged 18 to 65 with previously confirmed diagnosis of Fabry disease and a history of clinical symptoms of Fabry disease.\n- Participants may be receiving treatment with agalsidase alfa, agalsidase beta, or migalastat, or may be untreated.\n- Left ventricular hypertrophy.\n- Contraception for male or female participants: not pregnant or breastfeeding; no sperm donating for male participant.\n- A signed informed consent must be provided prior to any study-related procedures."}

Exclusion criteria

  • {"criterion_text":"- History of transient ischemic attack, stroke, myocardial infarction, heart failure, major cardiovascular surgery or kidney transplantation.\n- Positive SARS-CoV-2 virus test within 2 weeks of enrollment, or COVID-19 requiring hospitalization within 6 months of enrollment.\n- History of drug and/or alcohol abuse.\n- Moderate to severe hepatic impairment.\n- History of or active hepatobiliary disease.\n- Liver enzymes (alanine aminotransferase/aspartate aminotransferase) or total bilirubin >2 times the upper limit of normal.\n- Strong or moderate inducers or inhibitors of cytochrome P450 CYP3A4 within 14 days or 5 half-lives, whichever is longer, prior to randomization.\n- Known contraindication to undergoing MRI or known hypersensitivity to gadolinium-based contrast agents.\n- History of seizures currently requiring treatment.\n- Underlying medical condition that may cause or contribute to left ventricular hypertrophy.\n- Asymmetric hypertrophy by cardiac MRI at screening if considered by central reader to be not related to Fabry disease.\n- Advanced cardiac fibrosis, defined as significant late gadolinium enhancement affecting 3 or more segments involving >50% of myocardial thickness on screening cardiac MRI.\n- History of clinically significant cardiac arrhythmia. Atrial fibrillation that is well controlled on a stable medical regimen for at least 12 months is not an exclusion if the CHA2DS2-VASc score is 0 for males or 1 for females.\n- Estimated glomerular filtration rate <45 mL/min/1.73m2.\n- Presence of severe depression as measured by Beck’s Depression Inventory (BDI)- II >28 and/or a history of an untreated, unstable major affective disorder within 1 year of the screening visit.\n- Patients with hepatitis C, HIV, or hepatitis B infection."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Slope of left ventricular mass index as measured by cardiac magnetic resonance imaging (MRI) (central reading)","definition_or_measurement_approach":"Left ventricular mass index slope measured by cardiac MRI with central reading (change over time / slope over 18 months)."}

Secondary endpoints

  • {"endpoint_text":"- Slope of estimated glomerular filtration rate (eGFR) as assessed by the chronic kidney disease epidemiology collaboration (CKD-EPI) creatinine equation","definition_or_measurement_approach":"Slope of eGFR over time calculated using the CKD-EPI creatinine equation."}
  • {"endpoint_text":"- Change in T1 relaxation time, measured by cardiac MRI (central reading)","definition_or_measurement_approach":"T1 relaxation time change measured by cardiac MRI with central reading."}
  • {"endpoint_text":"- Change in global longitudinal strain, measured by echocardiography (central reading)","definition_or_measurement_approach":"Change in global longitudinal strain measured by echocardiography with central reading."}
  • {"endpoint_text":"- Percent Change in tiredness component of FDPRO","definition_or_measurement_approach":"Percent change from baseline in the tiredness component of the FD-PRO patient-reported outcome instrument."}
  • {"endpoint_text":"- Percent Change in swelling in lower extremities component of FD-PRO","definition_or_measurement_approach":"Percent change from baseline in the lower extremity swelling component of the FD-PRO patient-reported outcome instrument."}
  • {"endpoint_text":"- Number of participants with adverse event (AE) and serious adverse event (SAE)","definition_or_measurement_approach":"Count of participants experiencing AEs and SAEs during the study period."}
  • {"endpoint_text":"- Change in Beck Depression Inventory-II (BDI-II) score","definition_or_measurement_approach":"Change from baseline in BDI-II score."}
  • {"endpoint_text":"- Change in the lens clarity by ophthalmological examination","definition_or_measurement_approach":"Change from baseline in lens clarity assessed by ophthalmological exam."}
  • {"endpoint_text":"- Plasma venglustat concentrations at prespecified visits over the study duration","definition_or_measurement_approach":"Plasma concentrations of venglustat measured at prespecified visits (PK sampling)."}

Recruitment

Registry Or Advocacy Recruitment
True, Fabry registry (document: L1-sis-icf-fabry-registry-cz)
Digital Remote Recruitment
True, use of direct-to-patient services and recruitment advertisements/materials (documents indicate direct-to-patient service and online/advertisement materials).
Planned Sample Size
53
Recruitment Window Months
67
Consent Approach
Signed informed consent required prior to any study-related procedures ("A signed informed consent must be provided prior to any study-related procedures."). Participant consent forms and related ICFs (including partner/pregnancy ICFs) are provided; translations and redacted ICFs are available for multiple countries/languages (documents show ICFs and redacted ICFs in EN, ES, FR, DE, NL, PL, EL, IT, NO, CS/CZ, DA and others). No assent process for minors is described (participants are adults 18–65).

Methods

  • Country-specific recruitment materials (posters, brochures, informational leaflets) as provided in recruitment documents (examples: brochures and posters in ES, IT, DE, PL, NL, DA, EN).
  • Advertisements and patient outreach letters (documented recruitment-arrangements and recruitment-material files).
  • Direct-to-patient service / home health approaches (documented through sponsor third parties providing Direct to Patient Service and Home Health Care / Nursing).
  • Use of patient registry linkage for recruitment in at least one country (see Fabry registry document for Czechia).
  • Waiver documents for recruitment arrangements in multiple countries (K1 recruitment-arrangements-en-waiver files).

Geography

Total Number Of Sites
22
Total Number Of Participants
57

Austria

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
25-06-2024
Processing Time Days
33
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Medical University Of Graz
Department Name
Universitatsklinik fur Innere Medizin Kardiologie
Principal Investigator Name
Nicolas Verheyen
Principal Investigator Email
nicolas.verheyen@medunigraz.at
Contact Person Name
Nicolas Verheyen
Contact Person Email
nicolas.verheyen@medunigraz.at

Greece

Earliest CTIS Part Ii Submission Date
18-07-2024
Latest Decision Or Authorization Date
22-08-2024
Processing Time Days
35
Number Of Sites
3
Number Of Participants
5

Sites

Site Name
University General Hospital Of Heraklion
Department Name
Nephrology Dept
Principal Investigator Name
Konstantinos Stylianou
Principal Investigator Email
kstylianu@gmail.com
Contact Person Name
Konstantinos Stylianou
Contact Person Email
kstylianu@gmail.com
Site Name
Laiko General Hospital Of Athens
Department Name
Department Of Nephrology And Renal Transplantation
Principal Investigator Name
Ioannis Boletis
Principal Investigator Email
laikneph@laiko.gr
Contact Person Name
Ioannis Boletis
Contact Person Email
laikneph@laiko.gr
Site Name
University General Hospital Attikon
Department Name
2nd Neurology Dept
Principal Investigator Name
Georgios Tsivgoulis
Principal Investigator Email
tsivgoulisgiorg@yahoo.gr
Contact Person Name
Georgios Tsivgoulis
Contact Person Email
tsivgoulisgiorg@yahoo.gr

Spain

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
21-06-2024
Processing Time Days
29
Number Of Sites
3
Number Of Participants
5

Sites

Site Name
Hospital Alvaro Cunqueiro
Department Name
Complexo Hospitalario Universitario de Vigo. Servicio de Medicina Interna
Principal Investigator Name
Alberto Jose Rivera
Principal Investigator Email
riveraalberto0@gmail.com
Contact Person Name
Alberto Jose Rivera
Contact Person Email
riveraalberto0@gmail.com
Site Name
Hospital General Universitario Dr. Balmis
Department Name
Servicio de Cardiologia. Consultas externas, 5ª planta. Ensayos clinicos de Cardiologia
Principal Investigator Name
Vicente Eduardo Climent
Principal Investigator Email
vcliment@coma.es
Contact Person Name
Vicente Eduardo Climent
Contact Person Email
vcliment@coma.es
Site Name
Hospital General Universitario Gregorio Maranon
Department Name
Servicio de Cardiologia
Principal Investigator Name
Javier Bermejo
Principal Investigator Email
javier.bermejo@salud.madrid.org
Contact Person Name
Javier Bermejo

Czechia

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
24-06-2024
Processing Time Days
32
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Vseobecna Fakultni Nemocnice V Praze
Department Name
II. interni klinika
Principal Investigator Name
Gabriela Dostalova
Principal Investigator Email
gabriela.dostalova@vfn.cz
Contact Person Name
Gabriela Dostalova
Contact Person Email
gabriela.dostalova@vfn.cz

Norway

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
21-06-2024
Processing Time Days
29
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Helse Bergen HF
Department Name
Klinisk Forskningspost Barn og Unge
Principal Investigator Name
Camilla Tondel
Principal Investigator Email
camilla.tondel@helse-bergen.no
Contact Person Name
Camilla Tondel
Contact Person Email
camilla.tondel@helse-bergen.no

Denmark

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
21-06-2024
Processing Time Days
29
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Rigshospitalet
Department Name
Department of Medical Endocrinology
Principal Investigator Name
Caroline Michaela Kistorp
Principal Investigator Email
caroline.michaela.kistorp@region.dk
Contact Person Name
Caroline Michaela Kistorp

Italy

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
25-06-2024
Processing Time Days
33
Number Of Sites
4
Number Of Participants
8

Sites

Site Name
Azienda Ospedaliero-Universitaria Di Bologna IRCCS Istituto Di Ricerca E Di Cura A Carattere Scientifico
Department Name
Cardiologia
Principal Investigator Name
Elena Biagini
Principal Investigator Email
elena.biagini73@gmail.com
Contact Person Name
Elena Biagini
Contact Person Email
elena.biagini73@gmail.com
Site Name
Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico
Department Name
UOS Attivita Diurne Malattie Rare Internistiche
Principal Investigator Name
Valeria Di Stefano
Principal Investigator Email
valeria.distefano@policlinico.mi.it
Contact Person Name
Valeria Di Stefano
Site Name
Azienda Ospedaliera Dei Colli
Department Name
Unita di malattie rare, genetiche e cardiovascolari
Principal Investigator Name
Giuseppe Limongelli
Principal Investigator Email
limongelligiuseppe@libero.it
Contact Person Name
Giuseppe Limongelli
Contact Person Email
limongelligiuseppe@libero.it
Site Name
Azienda Ospedaliera Universitaria Federico II Di Napoli
Department Name
U.O. di Nefrologia Dipartimento di Sanita Pubblica
Principal Investigator Name
Antonio Pisani
Principal Investigator Email
antonio.pisani13@gmail.com
Contact Person Name
Antonio Pisani
Contact Person Email
antonio.pisani13@gmail.com

Germany

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
24-06-2024
Processing Time Days
32
Number Of Sites
4
Number Of Participants
10

Sites

Site Name
Charite Universitaetsmedizin Berlin KöR
Department Name
Nephrologie und Intensivmedizin
Principal Investigator Name
Sima Canaan-Kuhl
Principal Investigator Email
sima.canaan-kuehl@charite.de
Contact Person Name
Sima Canaan-Kuhl
Contact Person Email
sima.canaan-kuehl@charite.de
Site Name
SphinCS GmbH
Department Name
Geheimrat-Hummel-Platz 2
Principal Investigator Name
Eugen Mengel
Principal Investigator Email
eugen.mengel@sphincs.de
Contact Person Name
Eugen Mengel
Contact Person Email
eugen.mengel@sphincs.de
Site Name
Universitaetsklinikum Wuerzburg AöR
Department Name
"Zentrum Innere Medizin (ZIM) Oberdurrbacherstr. 6"
Principal Investigator Name
Christoph Wanner
Principal Investigator Email
wanner_c@ukw.de
Contact Person Name
Christoph Wanner
Contact Person Email
wanner_c@ukw.de
Site Name
Universitaetsmedizin der Johannes Gutenberg-Universitaet Mainz KöR
Department Name
Kinderklinik - Villa Metabolica
Principal Investigator Name
Julia Hennermann
Principal Investigator Email
julia.hennermann@unimedizin-mainz.de
Contact Person Name
Julia Hennermann

France

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
20-06-2024
Processing Time Days
28
Number Of Sites
1
Number Of Participants
10

Sites

Site Name
Raymond Poincare Hospital
Department Name
Unite Fonctionnelle de Genetique Medicale
Principal Investigator Name
Dominique Germain
Principal Investigator Email
dominique.germain@uvsq.fr
Contact Person Name
Dominique Germain
Contact Person Email
dominique.germain@uvsq.fr

Poland

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
24-06-2024
Processing Time Days
32
Number Of Sites
2
Number Of Participants
4

Sites

Site Name
Samodzielny Publiczny Zaklad Opieki Zdrowotnej Centralny Szpital Kliniczny Uniwersytetu Medycznego W Lodzi
Department Name
Klinika Elektrokardiologii
Principal Investigator Name
Krzysztof Kaczmarek
Principal Investigator Email
krzysztof.kaczmarek@umed.lodz.pl
Contact Person Name
Krzysztof Kaczmarek
Site Name
Krakowski Szpital Specjalistyczny Im. Sw. Jana Pawla II
Department Name
Oddzial Kliniczny Chorob Serca i Naczyn z Pododdzialem Intensywnego Nadzoru Kardiologicznego
Principal Investigator Name
Piotr Podolec
Principal Investigator Email
p.podolec@szpitaljp2.krakow.pl
Contact Person Name
Piotr Podolec
Contact Person Email
p.podolec@szpitaljp2.krakow.pl

Netherlands

Earliest CTIS Part Ii Submission Date
23-05-2024
Latest Decision Or Authorization Date
21-06-2024
Processing Time Days
29
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Academisch Medisch Centrum
Department Name
Amsterdam Universitair Medische Centra(#1)
Principal Investigator Name
Mirjam Langeveld
Principal Investigator Email
m.langeveld@amsterdamumc.nl
Contact Person Name
Mirjam Langeveld
Contact Person Email
m.langeveld@amsterdamumc.nl

Sponsor

Primary sponsor

Full Name
Sanofi-Aventis Recherche & Developpement
Organisation Type
Pharmaceutical company
Country Of Registered Address
France

Contract research organisations

Name
Bioclinica Inc.
Responsibilities
Central Medical Reading /Imaging Reading
Name
Labcorp Central Laboratory Services LP
Name
QPS LLC
Name
Endpoint Clinical Inc.
Name
Azenta Germany GmbH

Third parties

  • {"country":"Germany","full_name":"Azenta Germany GmbH","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Eresearchtechnology Inc.","duties_or_roles":"Cardiac Safety","organisation_type":"Pharmaceutical company"}
  • {"country":"France","full_name":"Marken","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Bioclinica Inc.","duties_or_roles":"Central Medical Reading /Imaging Reading","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"Greenwood Genetic Center Inc.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"ESMS Global Limited","duties_or_roles":"Centralized 24-Hour Emergency System: eSMS","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"QPS LLC","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Marken LLP","duties_or_roles":"Direct to Patient Service","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Labcorp Central Laboratory Services LP","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Accellacare Limited","duties_or_roles":"Home Health Care / Nursing","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Eresearchtechnology Inc.","duties_or_roles":"Clinical Outcomes Assessment Instrument (COA)","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Endpoint Clinical Inc.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
venglustat GZ402671 - SAR402671
Active Substance
venglustat
Modality
Small molecule
Routes Of Administration
ORAL USE
Route
Oral
Authorisation Status
Not authorised (development)
Orphan Designation
Yes
Frequency
Daily
Maximum Dose
15 mg
Investigational Product Name
Galafold 123 mg hard capsules
Active Substance
migalastat
Modality
Small molecule
Routes Of Administration
ORAL USE
Route
Oral
Authorisation Status
Authorised
Orphan Designation
Yes
Frequency
Daily
Maximum Dose
123 mg
Investigational Product Name
Fabrazyme 35 mg powder for concentrate for solution for infusion
Active Substance
agalsidase beta
Modality
Peptide/protein/enzyme
Routes Of Administration
INTRAVENOUS USE
Route
Intravenous
Authorisation Status
Authorised
Maximum Dose
1 mg/kg
Investigational Product Name
Replagal 1 mg/ml concentrate for solution for infusion.
Active Substance
agalsidase alfa
Modality
Peptide/protein/enzyme
Routes Of Administration
INTRAVENOUS USE
Route
Intravenous
Authorisation Status
Authorised
Maximum Dose
0.2 mg/kg

Related trials

Other published trials that may interest you.