Status:

ACTIVE_NOT_RECRUITING

IVIG With Rituximab vs Rituximab as First Line Treatment of Pemphigus

Lead Sponsor:

The University of Hong Kong

Conditions:

Pemphigus

Eligibility:

All Genders

18-75 years

Phase:

PHASE2

Brief Summary

Pemphigus is a rare acquired autoimmune disease in which immunoglobulin G (IgG) antibodies target desmosomal proteins to produce intraepithelial, and mucocutaneous blisters. It is potentially fatal an...

Detailed Description

Pemphigus is a rare acquired autoimmune disease in which immunoglobulin G (IgG) antibodies target desmosomal proteins to produce intraepithelial, and mucocutaneous blisters. It is potentially fatal an...

Eligibility Criteria

Inclusion

  • Inclusion Criteria:
  • Written informed consent obtained from patient
  • Ages Eligible for Study: 18 years to 75 years (Adult, Older Adult)
  • Newly or recently diagnosed (less than 18 months) diagnosed pemphigus vulgaris or pemphigus foliaceus based on clinical features; histological features of acantholysis via skin or mucosal biopsy; and intercellular staining pattern of indirect immunofluorescence or serological detection of DSG 1 or DSG 3 by enzyme-linked immunosorbent assay (ELISA)
  • Moderate to severe active disease, as defined by overall PDAI \>= 15 or skin involvement BSA\>= 5%. 9 \[Annex 1\]
  • Receiving standard-of-care oral prednisolone up to 1.5 mg/kg/day
  • Women who are sexually active and not postmenopausal, agreement to remain abstinent or use 2 effective methods of contraception.
  • Ability to comply with study protocol as deemed by investigator's assessment
  • Exclusion criteria:
  • Age \<18 or \>75
  • Pregnant women or nursing mother
  • Already diagnosed pemphigus patients diagnosed \> 18 months
  • Non-consenting patients, or patient who cannot be followed up regularly
  • Patient with history of serious allergy or anaphylactic reaction to monoclonal antibody treatment
  • Severe heart failure (NYHA Class III or IV)
  • Unstable angina or myocardiac infarction within last 3 months or post-infarction heart failure
  • Anaemia (haemoglobin \<10g/dL), Neutropenia (\<1000/mm3), Lymphopenia (\<900/mm3), thrombocytopenia (\<100,000/mm3)
  • Renal insufficiency eGFR \<60
  • Liver insufficiency of ALT/ALT \> 2 times normal limit range
  • Positive test results for hepatitis C (HCV) serology at screening \*Patients who are HepBs Ag positive, or HepBs Ag negative and anti-HepBc Ab - positive: Patients who are HepBs Ag positive - will be started on entecavir 0.5mg daily, and will be referred to a gastroenterologist for further follow up.
  • Patients who are HepBs Ag negative, and HBc Ab positive, with detectable HepB DNA levels - will be started on entecavir 0.5mg daily, and will be referred to a gastroenterologist for further follow up.
  • Patients who are HepBs Ag negative, HBc Ab positive, with no detectable HepB DNA levels - will be started on entecavir 0.5mg daily, and will be continued on entecavir for at least 18 months after completion of last dose of rituximab.
  • Blood test positive for HIV
  • Signs of active infection on CXR
  • Positive interferon gamma release assay Quantiferon or T.Spot TB test: must be treated with at least 4 weeks post initiation of isoniazid or other TB therapy
  • Inherited or acquired severe immunodeficiency
  • History of malignancy
  • Patient with active severe infection (excluding fungal infections of the nail), which has required antibiotic treatment within 2 week prior to study enrolment
  • Infection requiring hospitalisation or intravenous antibiotic treatment within the last 8 weeks prior to enrolment
  • Past history of osteomyelitis, or fasciitis, septic arthritis within the last one year
  • Patients with drug induced pemphigus. A thorough medication history will be taken to rule out drug induced pemphigus including D-penicillamine, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers and cephalosporins
  • Evidence of any new or uncontrolled concomitant disease that in the investigators' judgement would preclude the patients participation
  • Patients with history of allergy or adverse events to IVIG or rituximab treatment10
  • Treatment with intravenous immunoglobulins, plasmaphoresis within the last 8 weeks prior to randomization
  • Previous treatment with rituximab or any monoclonal antibody inducing profound lymphopenia
  • Treatment with live or attenuated vaccine within the last 28 days prior to randomization

Exclusion

    Key Trial Info

    Start Date :

    June 20 2020

    Trial Type :

    INTERVENTIONAL

    Allocation :

    ACTUAL

    End Date :

    June 30 2026

    Estimated Enrollment :

    20 Patients enrolled

    Trial Details

    Trial ID

    NCT04400994

    Start Date

    June 20 2020

    End Date

    June 30 2026

    Last Update

    July 8 2025

    Active Locations (1)

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    Department of Medicine

    Central, Hong Kong