An open-label administration study investigating the efficacy and safety of ACZ885 for Schnitzler's syndrome

JRCT ID: jRCT2051220139

Registered date:23/12/2022

Basic Information

• Recruitment status: Not Recruiting
• Health condition(s) or Problem(s) studied: Schnitzler syndrome
• Date of first enrollment: 02/02/2023
• Target sample size: 5
• Study type: Interventional
• Intervention(s): Canakinumab (recombinant) will be administered subcutaneously at a dose of 150mg (2 mg/kg for patients with body weight =<40 kg) every 8 weeks.

Outcome(s)

Primary Outcome

Efficacy: The proportion of subjects with complete clinical response after 7 days of initial administration
A complete clinical response was a PGA score of 5 or less and no greater than 1 in any of the 5 constituent signs/symptoms.

Secondary Outcome

Safety:Frequency and severity of adverse events and side effects
Efficacy:
1)Percentage of each clinical response
2)Change in PGA score
3)Change in disease activity (Schnitzler activity score)
4)Change in inflammatory markers (WBC, neutrophil count, CRP, SAA, ALP)
5)Quality of life (QOL) assessment
Effect of skin rash (DLQI:Dermatology Life Quality Index)
Health-related QOL (SF-36)
6)Serum IgM, IgG, and free L-chain kappa/lambda ratio (FLC)

Key inclusion & exclusion criteria

• Age minimum: >= 18age old
• Age maximum: Not applicable
• Gender: Both

Include criteria

1)Patients with Schnitzler syndrome who meet the diagnostic criteria established by Lipsker et al. (Strasbourg diagnostic criteria)
2)Patients who are at least 18 years of age at the time consent is obtained.
3)Patients who have given written consent to participate in this study.
4)Patients must have a mean Schnitzler syndrome disease score of at least 3.
5)Patients must have a total PGA score of 8 or higher.
6)Any of the laboratory tests (WBC, neutrophil count, and CRP) exceeds the facility's reference value range in the laboratory tests at the site.

Exclude criteria

1) Those with serious infections
2) Those with active tuberculosis (QFT/T-SPOT positive)
3) Those positive for anti-HIV antibodies,HBs antigen,HBs or HBc antibodies with HBV-DNA detected, or those with active hepatitis C (except for those positive for HCV antibodies if they are inactive and have normal liver function values)
4) Patients with serious complications (immunodeficiency,renal disease, liver disease, cardiac disease, cancer, etc.)
5) Those with a history of malignancy (except hematologic tumors due to current disease and non-metastatic skin cancer, basal cell carcinoma, squamous cell carcinoma and/or cancer in situ that is considered to be cured)
6) Any of the following in the examination prior to registration
Creatinin>2.0x upper limit of institutional reference value
Platelet count<100000/microL
AST > 3.0 x upper limit of institutional reference value
ALT > 3.0 x upper limit of institutional reference value
7) Received live vaccine within 4 weeks prior to enrollment
8) Participation in other clinical studies or trials involving intervention within 3 months prior to obtaining consent
9) Previously treated with an IL-1 inhibitor
10) Those with a history of hypersensitivity to canakinumab (genetical recombination)
11) Any history of substance abuse (drugs or alcohol) within the past 5 years or other factors (e.g., serious mental health conditions) that may limit the ability to comply with the procedures of this clinical trial
12) Women of childbearing potential who are unable to agree to use adequate contraception from the time consent is obtained until the end of the study period
13) Pregnant women, lactating women, or women who may be pregnant (pregnancy test will be performed to confirm pregnancy)
14) Other subjects who, in the judgment of the investigator or subinvestigator, are considered inappropriate to participate in this clinical trial.

Related Information

• Primary Sponsor: Kamb Naotomo
• Source(s) of Monetary Support: Japan Agency for Medical Research and Development