Pemphigus
Conditions
Interventions
Rituximab 100mg Group (Low-dose Group):Rituximab (Hanli Kang) 100mg intravenous infusion once at week 0, week 2, month 6, month 12, and month 18 (total 5 times, total dose 500mg)
combined with systemic corticosteroids (prednisone 1mg/kg/d or 1.5mg/kg/d) and compound sulfamethoxazole tablets for infection prevention
Rituximab 500mg Group (Standard-dose Group):Rituximab (Hanli Kang) 500mg intravenous infusion once at week 0, week 2, month 12, and month 18 (total 4 times, total dose 2000mg)
Control Group:Oral mycophenolate mofetil 1g twice daily
other immunosuppressants prohibited
Sponsors
Affiliated Dermatology Hospital of Shandong First Medical University
Eligibility
Sex/Gender
All
Age
18 Years to 75 Years
Inclusion criteria
Inclusion criteria: 1. Willing and able to provide written Informed Consent Form (ICF) and comply with all protocol-specified requirements. 2. Male or female patients, aged 18 to 75 years (inclusive). 3. Diagnosis of Pemphigus Vulgaris (PV) or Pemphigus Foliaceus (PF) meeting the following criteria: (1) Clinical presentation: For PV, presence of flaccid blisters or erosions on the skin and/or mucous membranes with a positive Nikolsky sign; for PF, presence of blisters in seborrheic areas or generalized distribution, evolving into greasy scales and foliaceous crusts, with a positive Nikolsky sign and typically no mucosal involvement. (2) Histopathology: For PV, intraepidermal blisters with suprabasal acantholysis; for PF, intraepidermal blisters with subcorneal or granular layer acantholysis. (3) Direct Immunofluorescence (DIF): Reticular (intercellular) deposition of IgG and/or C3 within the epidermis. (4) Enzyme-Linked Immunosorbent Assay (ELISA): For PV, positive for anti-Desmoglein 3 (Dsg3) antibodies (with or without positive anti-Dsg1); for PF, positive for anti-Dsg1 antibodies. 4. Moderate-to-severe pemphigus, defined by a Pemphigus Disease Area Index (PDAI) score: moderate (15–45 points) or severe (>45 points). 5. Rituximab-naïve (no prior exposure to rituximab therapy). 6. Female subjects of childbearing potential (WOCBP) must agree to use highly effective contraception (e.g., hormonal contraceptives, contraceptive patches, intrauterine devices, or barrier methods) throughout the study and for at least 12 months following the final dose. (Note: "Non-childbearing potential" is defined as being postmenopausal for at least 2 years, or having undergone total hysterectomy, bilateral salpingectomy, bilateral tubal ligation, bilateral oophorectomy, or having congenital infertility). 7. Male subjects with a female partner of childbearing potential must agree to use adequate contraception throughout the study and for 12 months after the final dose, and must refrain from sperm donation during this period. 8. In the investigator's clinical judgment, the patient is deemed capable of tolerating rituximab therapy.
Exclusion criteria
Exclusion criteria: 1. Age 75 years at the time of screening. 2. Known contraindications to rituximab or mycophenolate mofetil (MMF), including but not limited to severe cardiovascular disease, hepatic or renal impairment, or active infections. 3. Intolerance or contraindications to injectable glucocorticoids. 4. History of malignancy, including solid tumors, hematologic malignancies, or carcinoma in situ. (Exception: Subjects with basal cell carcinoma, cutaneous squamous cell carcinoma, cervical dysplasia, or Grade I cervical carcinoma in situ that was surgically excised and deemed cured at least 12 months prior to the screening visit are eligible). 5. History of severe infusion-related reactions, hypersensitivity to murine proteins, monoclonal antibodies, or other relevant drug allergies. 6. Medical history or current diagnosis of primary or secondary immunodeficiency, including a history of Human Immunodeficiency Virus (HIV) infection or a positive HIV test result. 7. Presence of severe, uncontrolled concomitant diseases—including but not limited to neurological, renal, hepatic, endocrine, or gastrointestinal disorders, diabetes, or hypertension—which, in the investigator’s judgment, may preclude participation in the study. 8. Any active infection (excluding onychomycosis); or a history of major infection requiring hospitalization or intravenous (IV) anti-infective therapy within 4 weeks prior to screening; or receipt of oral anti-infective therapy within 2 weeks prior to screening. 9. Laboratory abnormalities at screening: Aspartate aminotransferase (AST) or Alanine aminotransferase (ALT) > 2 $\times$ Upper Limit of Normal (ULN); Hemoglobin < 80 g/L; Absolute Neutrophil Count (ANC) < 1.5 $\times$ 10^9/L; or Platelet count < 75 $\times$ 10^9/L. (Medical supportive therapies, such as colony-stimulating factors, anti-anemic agents, hepatoprotective/enzyme-lowering drugs, or blood transfusions, are prohibited within 2 weeks prior to screening). 10. Female subjects who are pregnant or breastfeeding, or who plan to become pregnant or breastfeed during the study or within 12 months after the final dose. 11. Prior use of biologics, including rituximab, within 6 months prior to the screening visit. 12. Receipt of live or live-attenuated vaccines within 12 weeks prior to screening, or planned vaccination with live/live-attenuated vaccines during the 24-week treatment and assessment period. 13. Participation in another clinical trial involving an investigational product within 1 month prior to enrollment, or within 5 half-lives of the investigational drug, whichever is longer. 14. Any other medical condition or symptom that, in the opinion of the investigator, makes the patient unsuitable for the study, poses a risk to patient safety, or interferes with the assessment of adverse events (AEs).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Proportion of subjects and the time to onset achieving sustained Complete Remission; | — |
Secondary
| Measure | Time frame |
|---|---|
| Proportion of subjects achieving anti-Dsg1/3 antibody seroconversion (to negative) and the time to seroconversion.;Proportion of subjects with disease relapse and the time to relapse.;Proportion of subjects reaching the minimal maintenance dose of glucocorticoids and the time to achievement.;Cumulative Dose of Glucocorticoids (Prednisone Equivalent);Proportion of subjects and the time to onset (days) achieving sustained Complete Remission; | — |
Countries
China
Contacts
Public ContactZhang Furong
Affiliated Dermatology Hospital of Shandong First Medical University
Outcome results
None listed