A Single Cycle of Intravenous Immunoglobulin as Adjuvant to Rituximab in Patients With Pemphigus: A Retrospective Cohort Study at a Tertiary Referral Center

NCT06949241 | Active Not Recruiting

• 1 other identifier: MR-31-25-027185
• Study Type: observational
• Target: 76
• Locations: 1 country
• Sites: 1

Brief Summary

Pemphigus is a critical autoimmune skin condition mediated by pathogenic autoantibodies mainly against desmoglein (Dsg)1 and Dsg3, and is traditionally managed with systemic corticosteroids and immunosuppressants.The revolutionary introduction of rituximab (RTX) has opened up a new era of pemphigus treatment by enabling treatment strategies to specifically target CD20+ B cells.Studies have highlighted the superior efficacy of RTX combined with systemic corticosteroids, making this therapy first-line treatment for pemphigus patients.Currently, the main challenge for pemphigus management is to maximize efficacy while preventing relapses and reducing risks over the course of the disease. RTX achieves maximum effect typically at 4-8 weeks post-treatment, and is associated with an elevated risk of infection.On the other hand, intravenous immunoglobulin (IVIg), historically employed for the management of refractory pemphigus, is appreciated for its rapid action and lack of added immunosuppressive risk.It has also been shown to induce long-term clinical remission, and continues to serve as a rescue therapy for difficult cases. While there have been reports on the successful use of RTX and concomitant IVIg for treating refractory pemphigus, there is a lack of extensive research weighing the pros and cons of adding IVIg to the currently recommended RTX regimen (Rheumatoid arthritis protocol, RAP). To investigate the efficacy and safety of this combined therapy, we conducted an retrospective cohort study to evaluate the impact of incorporating IVIg into the RTX and systemic corticosteroid treatment protocol for pemphigus patients.

Conditions

Pemphigus Disease; Rituximab (RTx); Intravenous Immunoglobulin

Keywords

Pemphigus; Rituximab; IVIg; Adverse effect

Outcome Measures

Primary Outcomes (3)

• numbers of patients under disease control

  according to Pemphigus S2 Guideline, patients' new lesions cease to form and established lesions begin to heal

  week 4, 8, 12, 24, 36, 48 after treatment

• Number of patients with relapses

  according to Pemphigus S2 Guideline，patient who has achieved disease control appears ≥3 new lesions/month that do not heal spontaneously within 1 week, or patient's established lesions extend

  week 4, 8, 12, 24, 36, 48 after treatment

• Adverse event

  week 4, 8, 12, 24, 36, 48 after treatment

Secondary Outcomes (5)

• peripheral CD19+ cell counts

  week 4, 8, 12, 24, 36, 48 after treatment

• Serum rituximab concentration

  week 4, 8, 12, 24, 36, 48 after treatment

• immunoglobulin levels

  week 4, 8, 12, 24, 36, 48 after treatment

• serum anti-Dsg1 and Dsg3 autoantibody levels

  week 4, 8, 12, 24, 36, 48 after treatment

• Serum anti-rituximab antibody concentration

  week 4, 8, 12, 24, 36, 48 after treatment

Study Arms (2)

RTX group

Adult patients receiving rituximab and systemic corticosteroids without adjuvant IVIg

RTX+IVIg group

Adult patients receiving rituximab and systemic corticosteroids with adjuvant IVIg

Eligibility Criteria

• Age: 18 Years - 100 Years
• Sex: all
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients who had a clinical presentation of active pemphigus combined with immunological or pathological evidence, and received rituximab and systemic corticosteroids.

You may qualify if:

• Patients' files between January 1st, 2019, and December 31st, 2024 from department of dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine
• with a diagnosis of pemphigus vulgaris or foliaceus in active stage based on guideline indicators, including typical clinical presentation, positive direct immunofluorescence microscopy findings, and/or detection of serum anti-Dsg3 and/or Dsg1 immunoglobin (Ig)G autoantibodies
• treated with RTX in association with oral corticosteroids
• with a follow-up of at least 48 weeks after RTX treatment initiation

You may not qualify if:

• with a diagnosis of other variants of pemphigus (e.g., paraneoplastic pemphigus, herpetiform pemphigus, IgA pemphigus);
• concomitant use of other immunosuppressants (e.g., azathioprine, mycophenolate mofetil, methotrexate)
• previous administration with IVIg for other indications within 12 weeks

Sponsors & Collaborators

• Ruijin Hospital: lead

Study Sites (1)

Department of Dermatology, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine

Shanghai, Shanghai Municipality, China

Location

MeSH Terms

Conditions

Pemphigus

Condition Hierarchy (Ancestors)

Skin Diseases, Vesiculobullous; Skin Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 3, 2025
• First Posted: April 29, 2025
• Study Start: December 1, 2019
• Primary Completion: February 2, 2026
• Study Completion: February 2, 2026
• Last Updated: April 29, 2025

Record last verified: 2025-04

Locations

CN (1)