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A Study to Evaluate Efficacy and Safety of Abatacept in Participants of Pemphigus Vulgaris (PV)

Phase 4
Conditions
Pemphigus Vulgaris
Interventions
Registration Number
NCT05303272
Lead Sponsor
Tongji Hospital
Brief Summary

Pemphigus vulgaris (PV) is a rare, chronic, debilitating, and potentially life-threatening autoimmune disorder that is characterized by mucocutaneous blisters.Abatacept is a biologic drug that belongs to the class of T-cell co-stimulation modulators and is used for the treatment of autoimmune diseases.

Detailed Description

The background therapy is based on prednisolone administration. PV is a rare disorder, therefore this study is designed as a crossover that may require fewer patients than a parallel study. The study enrolled participants with moderate-to-severely active PV requiring ≥ 50 milligrams per day (mg/day) oral prednisone or equivalent. The purpose of this study was to evaluate the efficacy, tolerability, and safety of abatacept injection for subcutaneous use (abatacept SC) 150 mg administered once in a week in subjects with PV. It was anticipated that with sustained immune suppression in the presence of abatacept SC that clinical remission of the disease would be improved.

Recruitment & Eligibility

Status
UNKNOWN
Sex
All
Target Recruitment
60
Inclusion Criteria
  1. Adults (18 through 80 years of age) with clinically-documented diagnosis of PV for >2 months and <10 years.
  2. History of biopsy consistent with PV (Hematoxylin and Eosin staining and direct immunofluorescence). Confirmed diagnosis of PV within the previous 24 months, based on the presence of histological features of acantholysis via skin or mucosal biopsy and one of the following: tissue bound immunoglobulin G (IgG) antibodies by direct immunofluorescence on the surface of affected epithelium or serological detection of serum desmoglein-3 (DSg3) autoantibodies against epithelial cell surface either by indirect immunofluorescence microscopy or by enzyme-linked immunosorbent assay.
  3. At least 1 previous episode of a failed steroid taper (ie, disease flare/relapse) at a prednisone/prednisolone dose >10 mg/day. The following criteria must have been met as evidence of disease severity at the time of the failed steroid taper: a) A Pemphigus Severity of Clinical Disease score of moderate (2) or severe (3) (may be historical/retrospective assessment). b) Required a treatment change at the time of the failed steroid taper of at least one of the following: i) A steroid increase to >=20 mg/day OR ii) The addition of immunosuppressive/immunomodulatory agent/treatment OR iii) A dose increase of immunosuppressive/immunomodulatory agent/treatment
  4. Screening anti-Dsg antibodies consistent with a diagnosis of PV (ie, elevated antiDsg3 antibodies).
  5. Has initiated and received a stable dose of prednisone/prednisolone from a minimum of 20 mg/day (example: 0.25 mg/kg/day for an 80 kg person) up to a maximum of 120 mg/day or 1.5 mg/kg/day (whichever is higher) for >=2 weeks prior to randomization.
  6. Has exhibited PV disease control, defined as no new lesions for >=2 weeks. A female subject is eligible to enter the study if she: Is of non-child bearing potential, who 7. is either surgically sterile (bilateral tubal ligation, bilateral oophorectomy, or post-hysterectomy) or is postmenopausal without menses for >2 years. Women who are <2 years postmenopausal are required to have menopausal status confirmed by follicle-stimulating hormone (FSH) and estradiol levels at the screening evaluation. If FSH and estradiol levels do not provide confirmation of menopause, subject will be considered to be of childbearing potential.
Exclusion Criteria
  1. Diagnosis of pemphigus foliaceus, paraneoplastic pemphigus, or other autoimmune blistering disease (other than pemphigus vulgaris).
  2. Past or current history of hypersensitivity to components of the investigational product or medically significant adverse effects (including allergic reactions) from cetirizine (or antihistamine equivalent) or paracetamol/acetaminophen.
  3. Prior treatment with rituximab without achieving disease control within 6 months of initiating rituximab dosing.
  4. Prior treatment with immunosuppressant or immunomodulation agents within the protocol specified periods
  5. Evidence or history of clinically significant infections
  6. Past or current malignancy, except for cervical carcinoma Stage 1B or less, noninvasive basal cell and squamous cell skin carcinoma and cancer diagnoses with a duration of complete response (remission) >5 years
  7. Significant concurrent, uncontrolled medical condition that could affect the subject's safety, impair the subject's reliable participation in the study, impair the evaluation of endpoints, or necessitate the use of medication not allowed by the protocol. This includes subjects who require any systemic steroid treatment for a concurrent medical condition (other than pemphigus vulgaris).
  8. Use of an investigational drug or other experimental therapy within 4 weeks, 5 pharmacokinetic half-lives, or the duration of biological effect (whichever is longer) prior to Screening.
  9. Electrocardiogram (ECG) showing a clinically significant abnormality or showing a QTc interval ≥450 msec (≥480 msec for subjects with a bundle branch block)
  10. Woman who is breastfeeding.
  11. Positive test results for hepatitis B surface antigen (HBsAg), hepatitis B core antibody (HBcAb), or hepatitis C virus (HCV) serology at screening

Study & Design

Study Type
INTERVENTIONAL
Study Design
CROSSOVER
Arm && Interventions
GroupInterventionDescription
Mycophenolate mofetilMycophenolate Mofetil 500Mg TabSubject received subcutaneous administration of matching placebo of abatacept once a weeks through the 24 week double blind period. A washout period of MMF for 4 weeks (24 th-28th week) is used to ensure data integrity. Subsequently,subject were administered subcutaneous administration of abatacept 125 mg once every week through the 28-52 week open label period.
AbataceptAbatacept Prefilled SyringeSubject received subcutaneous administration of abatacept 125 mg once every week through the 52 week double blind period.
Primary Outcome Measures
NameTimeMethod
Percentage of Participants Who Achieved Sustained Complete Remission, Evaluated by the Pemphigus Disease Area Index (PDAI) Activity Score24 Weeks

PADAI and ABSIS was proposed by the German Blistering Disease Group in 2007 which was accepted as the most sensitive and reliable systems for evaluation of pemphigus severity. PADAI was developed by the International Pemphigus Definitions Group in 2009

Percentage of Participants Who Achieved Sustained Complete Remission, Evaluated byAutoimmune bullous skin disorder intensity score (ABSIS)24 Weeks

PADAI and ABSIS was proposed by the German Blistering Disease Group in 2007 which was accepted as the most sensitive and reliable systems for evaluation of pemphigus severity. PADAI was developed by the International Pemphigus Definitions Group in 2009

Secondary Outcome Measures
NameTimeMethod
Ulcer Severity Score (USS) for the assessment of skin, oral ulcer improvementFrom baseline up to 4th, 8th,12th, 24th, 36th and 52th Week

The USS incorporates six ulcer characteristics: number, size, duration, ulcer-free period, site, and pain. This scoring template may be of value to future studies assessing treatment efficacy.

Physician global assessment (PGA)From baseline up to 4th, 8th,12th, 24th, 36th and 52th Week

Physician global assessment was assessed by an individual researcher

Change From Baseline for CD19+ B Cell CountFrom Baseline up to 12th, 24th and 52th Week

CD19+ B cell count will be performed using Flow Cytometry

Autoimmune bullous disease quality of life (ABQoL)From baseline up to 4th, 8th,12th, 24th, 36th and 52th Week

Total ABQoL scores range from 0 to 30 with higher DLQI scores reflecting greater impairment in a participant's health-related quality of life. The ABQoL score is calculated by summing the score of each question resulting in a maximum of 30 and a minimum of 0. The higher the score, the more quality of life is impaired. The measure type mean is the estimated mean from adjusted MMRM.

Anti-desmoglein 1 (anti-Dsg1) and anti-Dsg3 autoantibody titersFrom Baseline up to 12th, 24th and 52th Week

anti-Dsg1 and anti-Dsg3 will be performed using ELISA

Cumulative Oral Corticosteroid DoseFrom 12th, 24th, 36th and 52th Week

Calculate the Cumulative Oral Corticosteroid Dose during 52 week

Trial Locations

Locations (1)

Tongji Hospital

🇨🇳

Wuhan, Hubei, China

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