Clinical trial • Phase III • Rare Disease | Gastroenterology

FAZIRSIRAN for Alpha-1 antitrypsin deficiency-associated liver disease | Liver fibrosis (METAVIR F1)

Phase III trial of FAZIRSIRAN for Alpha-1 antitrypsin deficiency-associated liver disease | Liver fibrosis (METAVIR F1).

Overview

Trial Therapeutic Area
Rare Disease | Gastroenterology
Trial Disease
Alpha-1 antitrypsin deficiency-associated liver disease | Liver fibrosis (METAVIR F1)
Trial Stage
Phase III
Drug Modality
Oligonucleotide | Other
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
27-11-2023
First CTIS Authorization Date
27-03-2024

Trial design

Randomised, fazirsiran 200 mg (formulated as 200 mg/ml, 1.0 ml withdrawable volume) administered subcutaneously on day 1, week 4, then every 12 weeks vs placebo (0.9% sodium chloride injection) administered on the same schedule.-controlled Phase III trial across 20 sites in France, Austria, Ireland and others.

Randomised
Yes
Comparator
Fazirsiran 200 mg (formulated as 200 mg/mL, 1.0 mL withdrawable volume) administered subcutaneously on Day 1, Week 4, then every 12 weeks vs Placebo (0.9% sodium chloride injection) administered on the same schedule.
Target Sample Size
25
Trial Duration For Participant
924

Eligibility

Recruits 25 Vulnerable population selected (isVulnerablePopulationSelected = true). Participants are adults aged 18 to 75 years and provide informed consent. Subject information and informed consent form documents are provided (Main-ICF) and there are specific Pregnant-Partner ICF and optional genetic/DNA sample ICF documents included in the dossier. No assent/parental consent for minors is applicable as minimum age is 18..

Vulnerable Population
Vulnerable population selected (isVulnerablePopulationSelected = true). Participants are adults aged 18 to 75 years and provide informed consent. Subject information and informed consent form documents are provided (Main-ICF) and there are specific Pregnant-Partner ICF and optional genetic/DNA sample ICF documents included in the dossier. No assent/parental consent for minors is applicable as minimum age is 18.

Inclusion criteria

  • {"criterion_text":"- 1. The participant must have a diagnosis of the PiZZ genotype AATD. A diagnosis of PiZZ from source- verifiable medical records is permitted. Otherwise, participants must undergo PiZZ confirmatory testing (genotyping for PiS and PiZ alleles) at screening. PiMZ or PiSZ genotypes are not permitted.\n- 2. The participant, of any sex, is aged 18 to 75 years, inclusive.\n- 3. The participant has evidence of METAVIR stage 1 liver fibrosis, evaluated by a centrally-read baseline liver biopsy during the screening period; or confirmed as meeting all the entry criteria by central reading from a previous biopsy conducted within 1 year before the estimated enrollment date using an adequate liver biopsy and slides as defined in the study laboratory manual.\n- 4. The participant has a pulmonary status meeting the protocol requirements"}

Exclusion criteria

  • {"criterion_text":"- 1. Evidence of ≥ F2 fibrosis based on liver biopsy during the screening period.\n- 2. The participant has a history of liver decompensating events (overt hepatic encephalopathy documented by a physician or trained professional, clinically significant ascites, spontaneous bacterial peritonitis, gastrointestinal (GI) bleeding from varices, hepatopulmonary syndrome, hepatorenal syndrome, portal pulmonary hypertension, or bleeding portal hypertensive gastropathy).\n- 3. The participant has evidence of other forms of chronic liver diseases, including viral hepatitis B or C, primary biliary cholangitis, primary sclerosing cholangitis, Wilson disease, alcoholic hepatitis, hemochromatosis, liver cancer, history of biliary diversion, or autoimmune hepatitis. Additional diagnosis will be allowed as per the protocol.\n- 4. The participant has a history of malignancy within the last 5 years, except for adequately treated basal cell carcinoma, squamous cell skin cancer, superficial bladder tumors, or in situ cervical cancer. Participants with curatively treated malignancies who have no evidence of metastatic disease and a greater than 1 year disease-free interval may be enrolled after approval by the medical monitor.\n- 5. The participant is expected to have severe and unavoidable high-level exposure to inhaled pulmonary toxins during the study such as may occur with occupational exposure to mineral dusts or metals.\n- 6. The participant has a recent lower respiratory tract infection, such as pneumonia, within the last 6 months before screening. The participant may be screened earlier based on principal investigator (PI) assessment of clinical recovery and return to baseline pulmonary function in discussion with the medical monitor.\n- 7. The participant has a history of frequent pulmonary exacerbations (≥2 moderate or severe exacerbations within 52 weeks before screening).\n- 8. The participant is experiencing a pulmonary exacerbation at the time of screening (participant may be rescreened after the clinical resolution of an exacerbation).\n- 9. The participant is receiving long-term, around-the-clock oxygen supplementation, or supplemental oxygen with continuous positive airway pressure (CPAP) or bilevel positive airway pressure for acute respiratory failure. The following conditions are allowable for the participant to enter screening: short-term use of oxygen supplementation (eg, for the management of acute chronic obstructive pulmonary disease [COPD] exacerbation) or CPAP for obstructive sleep apnea."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- AEs and SAE including any pulmonary AEs or SAEs indicative of worsening pulmonary condition/function (eg, pulmonary exacerbation, respiratory infection, significant PFT decline).\n- Change from baseline over time to EOS in pulmonary function parameters at scheduled visits\n- Change from baseline in whole lung 15th percentile density as measured by computed tomography (CT) lung densitometry over time to Week 100.\n- Vital signs.\n- ECG measurements.\n- Clinical laboratory values (hematology, biochemistry including liver tests, coagulation, and urinalysis results).","definition_or_measurement_approach":"- AEs/SAEs: collection and reporting of adverse events and serious adverse events including pulmonary-specific events indicative of worsening pulmonary condition/function.\n- Pulmonary function: change from baseline over time to End Of Study (EOS) assessed at scheduled visits using pulmonary function tests (PFTs).\n- CT lung densitometry: change from baseline in whole lung 15th percentile density measured by CT lung densitometry to Week 100.\n- Vital signs: standard vital sign measurements at scheduled visits.\n- ECG measurements: standard ECG assessments at scheduled visits.\n- Clinical laboratory values: hematology, biochemistry (including liver tests), coagulation and urinalysis measured per protocol at scheduled timepoints."}

Secondary endpoints

  • {"endpoint_text":"- Change from baseline in serum Z-AAT protein over time to Week 106.\n- Percent change from baseline in intrahepatic liver Z-AAT protein at Week 106.\n- Change from baseline in intrahepatic Z-AAT protein polymer burden assessed by PAS+D staining at Week 106.\n- No change or decrease from baseline in intrahepatic Z-AAT protein polymer burden assessed by PAS+D staining at Week 106.\n- Change from baseline in intrahepatic portal inflammation at Week 106.\n- No change or decrease from baseline in intrahepatic portal inflammation at Week 106.\n- No change or decrease from baseline in histologic fibrosis score (METAVIR staging) at Week 106.\n- Change from baseline in VCTE-derived liver stiffness over time to Week 106.\n- Change from baseline in markers of liver injury (alanine aminotransferase [ALT], aspartate aminotransferase [AST], γ-glutamyl transferase [GGT]) over time to Week 106.","definition_or_measurement_approach":"- Serum Z-AAT protein: serial serum measurements over time to Week 106.\n- Intrahepatic Z-AAT protein: measured in liver tissue at Week 106 (biopsy) with percent change from baseline.\n- Z-AAT polymer burden: assessed histologically by PAS+D staining on liver biopsy at Week 106.\n- Portal inflammation: histologic assessment on liver biopsy at Week 106.\n- Histologic fibrosis score (METAVIR): central reading of liver biopsy at Week 106.\n- VCTE-derived liver stiffness: measured by vibration-controlled transient elastography (FibroScan) over time to Week 106.\n- Liver injury markers (ALT, AST, GGT): clinical laboratory measurements over time to Week 106."}

Recruitment

Digital Remote Recruitment
Yes
Planned Sample Size
25
Recruitment Window Months
53
Consent Approach
Informed consent obtained from each participant (adults aged 18–75). Main informed consent forms (Main-ICF) are provided and available in multiple country-specific/local languages (documents in English, French, Italian, Polish, Portuguese, Swedish, German, Dutch, Spanish are present). Optional and supplementary consent documents are provided (e.g. Optional Genetic/DNA sample ICFs, Pregnant-Partner ICF). Country-specific recruitment and informed consent procedures documents (K1) are included.

Methods

  • Site-based recruitment through listed hospital/clinic trial sites (country-specific site lists provided).
  • GP letters (country-specific GP letter documents present, e.g. Ireland, Italy).
  • Participant travel reimbursement and logistics notices (GreenPhire travel notice documents) to support participation.
  • Digital patient engagement and remote support via Medidata Patient Cloud App and concierge email templates (documents present).

Geography

Total Number Of Sites
20
Total Number Of Participants
25

France

Earliest CTIS Part Ii Submission Date
23-02-2024
Latest Decision Or Authorization Date
21-11-2025
Processing Time Days
637
Number Of Sites
4
Number Of Participants
4

Sites

Site Name
Centre Hospitalier Universitaire De Rennes
Department Name
Liver disease department
Contact Person Name
Edouard BARDOU-JACQUET
Site Name
Hopital De La Croix Rousse
Department Name
Hepatogastro enterology
Contact Person Name
Teresa ANTONINI
Contact Person Email
teresa.antonini@chu-lyon.fr
Site Name
Centre Hospitalier Universitaire De Lille
Department Name
Service des Maladies de l'Appareil Digestif et de la nutrition
Contact Person Name
Alexandre LOUVET
Contact Person Email
alexandre.louvet@chu-lille.fr
Site Name
Hopital Paul Brousse
Department Name
Centre Hépato-Biliaire
Contact Person Name
Audrey COILLY
Contact Person Email
audrey.coilly@aphp.fr

Austria

Earliest CTIS Part Ii Submission Date
07-05-2024
Latest Decision Or Authorization Date
01-12-2025
Processing Time Days
574
Number Of Sites
2
Number Of Participants
3

Sites

Site Name
Medical University Of Graz
Department Name
Department of Internal Medicine - Division of Gastroenterology and Hepatology
Contact Person Name
Martin Wagner
Contact Person Email
martin.wagner@medunigraz.at
Site Name
Landeskrankenanstalten-Betriebsgesellschaft Kabeg
Department Name
Department of Internal Medicine, Gastroenterology, Hepatology, Endocrinology, Rheumatology
Contact Person Name
Markus Peck-Radosavljevic

Ireland

Earliest CTIS Part Ii Submission Date
19-02-2024
Latest Decision Or Authorization Date
24-11-2025
Processing Time Days
644
Number Of Sites
1
Number Of Participants
1

Sites

Site Name
Beaumont Hospital
Department Name
Department of Medicine
Contact Person Name
Noel G McElvaney
Contact Person Email
gmcelvaney@rcsi.ie

Belgium

Earliest CTIS Part Ii Submission Date
04-03-2024
Latest Decision Or Authorization Date
25-11-2025
Processing Time Days
631
Number Of Sites
2
Number Of Participants
2

Sites

Site Name
Antwerp University Hospital
Department Name
Gastroenterology and hepatology
Contact Person Name
Sven Francque
Contact Person Email
sven.francque@uza.be
Site Name
UZ Leuven
Department Name
xx
Contact Person Name
Jef Verbeek
Contact Person Email
jef.verbeek@uzleuven.be

Poland

Earliest CTIS Part Ii Submission Date
05-03-2024
Latest Decision Or Authorization Date
25-11-2025
Processing Time Days
630
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
ID Clinic Arkadiusz Pisula
Contact Person Name
Ewa Janczewska
Contact Person Email
idclinic@idclinic.eu

Spain

Earliest CTIS Part Ii Submission Date
20-02-2024
Latest Decision Or Authorization Date
24-11-2025
Processing Time Days
633
Number Of Sites
1
Number Of Participants
2

Sites

Site Name
University Hospital Virgen Del Rocio S.L.
Department Name
Gastroenterology and Hepatology
Contact Person Name
Manuel Romero Gómez

Italy

Earliest CTIS Part Ii Submission Date
08-02-2024
Latest Decision Or Authorization Date
21-11-2025
Processing Time Days
652
Number Of Sites
1
Number Of Participants
1

Sites

Site Name
Fondazione IRCCS Policlinico San Matteo
Department Name
S.C Pneumologia
Contact Person Name
Angelo Guido Corsico
Contact Person Email
corsico@unipv.it

Germany

Earliest CTIS Part Ii Submission Date
26-01-2024
Latest Decision Or Authorization Date
22-12-2025
Processing Time Days
696
Number Of Sites
4
Number Of Participants
5

Sites

Site Name
Universitaetsklinikum Tuebingen AöR
Department Name
Innere Medizin Department I
Contact Person Name
Christoph Berg
Site Name
Universitaetsklinikum Aachen AöR
Department Name
Medizinische Klinik III
Contact Person Name
Pavel Strnad
Contact Person Email
pstrnad@ukaachen.de
Site Name
Charite Universitaetsmedizin Berlin KöR
Department Name
Medizinische Klinik m. S. Hepatologie und Gastroenterologie
Contact Person Name
Frank Tacke
Contact Person Email
frank.tacke@charite.de
Site Name
Medizinische Hochschule Hannover
Department Name
Klinik für Gastroenterologie, Hepatologie, Infektiologie und Endokrinologie
Contact Person Name
Heiner (Hans Heinrich) Wedemeyer

Portugal

Earliest CTIS Part Ii Submission Date
06-03-2024
Latest Decision Or Authorization Date
21-11-2025
Processing Time Days
625
Number Of Sites
3
Number Of Participants
3

Sites

Site Name
CCAB Centro Clinico Academico Braga Associacao
Department Name
Gastroenterology
Contact Person Name
Carla Rolanda
Contact Person Email
crolanda@med-uminho.pt
Site Name
Servico de Saude da Regiao Autonoma Da Madeira EPERAM
Department Name
Gastroenterology
Contact Person Name
Vítor Pereira
Contact Person Email
vitor.pereira@sesaram.pt
Site Name
Centro Hospitalar Universitario De Santo Antonio E.P.E.
Department Name
Gastroenterology
Contact Person Name
Luis Maia

Sweden

Earliest CTIS Part Ii Submission Date
01-03-2024
Latest Decision Or Authorization Date
24-11-2025
Processing Time Days
633
Number Of Sites
1
Number Of Participants
1

Sites

Site Name
Karolinska University Hospital
Department Name
Sektionen för lever och pankreassjukdomar
Contact Person Name
Staffan Wahlin
Contact Person Email
staffan.wahlin@ki.se

Sponsor

Primary sponsor

Full Name
Takeda Development Center Americas Inc.
Organisation Type
Pharmaceutical company
Country Of Registered Address
United States

Contract research organisations

Name
WCG Clinical Inc.
Responsibilities
Study site support services for patient reimbursement
Name
Certara USA Inc.
Name
Pharmaceutical Product Development LLC
Name
Perceptive Informatics Inc.
Responsibilities
Clinical Imaging
Name
Bioclinica Inc.
Responsibilities
Clinical Imaging
Name
Q2 Solutions LLC
Name
Suvoda LLC
Name
Cytel Inc.

Third parties

  • {"country":"United States","full_name":"WCG Clinical Inc.","duties_or_roles":"Study site support services for patient reimbursement","organisation_type":"Pharmaceutical company"}
  • {"country":"France","full_name":"Echosens","duties_or_roles":"VCTE Fibroscan Image analysis","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Medidata Solutions Inc.","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Certara USA Inc.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Greenphire LLC","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"Canada","full_name":"Charles River Laboratories Montreal ULC","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Bioclinica Inc.","duties_or_roles":"Clinical Imaging","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"Suvoda LLC","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Pharmaceutical Product Development LLC","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Perceptive Informatics Inc.","duties_or_roles":"Clinical Imaging","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Cytel Inc.","duties_or_roles":"","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United States","full_name":"Q2 Solutions LLC","duties_or_roles":"","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"Denmark","full_name":"Nordic Bioscience A/S","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Eresearchtechnology Inc.","duties_or_roles":"Spirometry PFT DLCO analysis","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Perspectum Limited","duties_or_roles":"","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
Fazirsiran
Active Substance
FAZIRSIRAN
Modality
Oligonucleotide
Routes Of Administration
Subcutaneous injection
Route
Subcutaneous injection
Authorisation Status
Authorised
Orphan Designation
Yes
Starting Dose
200 mg
Dose Levels
200 mg
Frequency
Day 1, Week 4, then every 12 weeks
Maximum Dose
200 mg
Investigational Product Name
SALINE
Active Substance
SALINE
Modality
Other
Routes Of Administration
Solution for injection
Route
Solution for injection
Frequency
Matched to active arm (Day 1, Week 4, then every 12 weeks)

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