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Phase 3 N=106 Randomized Quadruple-blind Treatment

A Study to Evaluate the Efficacy and Safety of Dupilumab in Adult Patients With Bullous Pemphigoid

Bullous Pemphigoid

Enrolled (actual)
106
Serious AEs
38.7%
Results posted
Mar 2026
Primary outcome: Primary: Percent of Participants Achieving Sustained Remission at Week 36 — 4.0; 18.2 Estimated Percentage of Participants — p=0.0250

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
dupilumab (Drug); Matching Placebo (Drug); Oral corticosteroids (OCS) (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Regeneron Pharmaceuticals
Primary completion
Jul 2024

Outcome Measures

OutcomeResultp-value
PRIMARY
Percent of Participants Achieving Sustained Remission at Week 36
4.0; 18.2 0.0250 sig
SECONDARY
Total Cumulative Dose of Oral Corticosteroids (OCS) From Baseline to Week 36
5121.15; 4184.93 0.1092
SECONDARY
Percent Change in Weekly Average of Daily Peak Pruritus Numerical Rating Scale (NRS) Score From Baseline to Week 36
-26.7; -51.8 0.0025 sig
SECONDARY
Percent of Participants With Improvement (Reduction) of Weekly Average of Daily Peak Pruritus NRS ≥4 From Baseline to Week 36
10.5; 39.8 0.0006 sig
SECONDARY
Percent Change in Bullous Pemphigoid Disease Area Index (BPDAI) Activity Score From Baseline to Week 36
-50.5; -76.7 0.0021 sig
SECONDARY
Time to First Use of Rescue Medication Up to Week 36
126.8; 168.9 0.0023 sig
SECONDARY
Duration of Complete Remission While Not Requiring OCS Up to Week 36
12.9; 38.7 0.0112 sig
SECONDARY
Percent of Participants Who Did Not Achieve Control of Disease Activity Before Week 36
7.5; 1.9 0.2414
SECONDARY
Percent of Participants Who Relapsed After Achieving Control of Disease Activity Up to Week 36
91.8; 73.1 0.0165 sig
SECONDARY
Percent of Participants Who Did Not Achieve Complete Remission Before Week 36
50.9; 39.6 0.2758
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥50% From Baseline to Week 36
13.2; 41.0 0.0012 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥75% From Baseline to Week 36
12.5; 38.8 0.0024 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥90% From Baseline to Week 36
9.8; 38.6 0.0006 sig
SECONDARY
Change in Autoimmune Bullous Disease Quality of Life (ABQOL) Score From Baseline to Week 36
-3.41; -7.09 0.0476 sig
SECONDARY
Change in Percent Body Surface Area (BSA) of BP Involvement From Baseline to Week 36
-17.51; -24.07 0.0345 sig
SECONDARY
Change in BP180 Immunoglobulin G (IgG) Autoantibody Titer From Baseline to Week 36
-137.57; -136.33 0.9857
SECONDARY
Change in BP230 IgG Autoantibody Titer From Baseline to Week 36
-33.91; -18.49 0.6680
SECONDARY
Percent of Participants Achieving Sustained Remission at Week 52
4.0; 14.2 0.0736
SECONDARY
Total Cumulative Dose of OCS From Baseline to Week 52
7159.09; 5133.03 0.0463 sig
SECONDARY
Duration of Complete Remission While Not Requiring OCS Up to Week 52
24.7; 67.2 0.0198 sig
SECONDARY
Percent of Participants Who Did Not Achieve Control of Disease Activity Before Week 52
7.5; 1.9 0.2414
SECONDARY
Percent of Participants Who Relapsed After Achieving Control of Disease Activity Up to Week 52
91.8; 76.9 0.0461 sig
SECONDARY
Percent of Participants Who Did Not Achieve Complete Remission Before Week 52
50.9; 34.0 0.0843
SECONDARY
Percent Change in Weekly Average of Daily Peak Pruritus NRS From Baseline to Week 52
-23.4; -53.0 0.0002 sig
SECONDARY
Percent of Participants With Improvement (Reduction) of Weekly Average of Daily Peak Pruritus NRS ≥4 From Baseline to Week 52
8.3; 36.4 0.0006 sig
SECONDARY
Percent Change in BPDAI Activity Score From Baseline to Week 52
-49.1; -76.1 0.0014 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥50% From Baseline to Week 52
11.2; 37.2 0.0019 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥75% From Baseline to Week 52
10.6; 36.6 0.0016 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥90% From Baseline to Week 52
9.9; 34.4 0.0024 sig
SECONDARY
Change in ABQOL Score From Baseline to Week 52
-3.27; -7.29 0.0311 sig
SECONDARY
Change in Percent BSA of BP Involvement From Baseline to Week 52
-16.59; -23.89 0.0206 sig
SECONDARY
Change in BP180 IgG Autoantibody Titer From Baseline to Week 52
-133.02; -137.60 0.9471
SECONDARY
Change in BP230 IgG Autoantibody Titer From Baseline to Week 52
-33.33; -18.83 0.6876
SECONDARY
Percent of Participants in Complete Remission and Off OCS No Later Than Week 16
26.6; 34.6 0.5283
SECONDARY
Percent Change in BPDAI Activity Score From Baseline to Week 16
-56.3; -78.5 0.0088 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥50% From Baseline to Week 16
45.2; 67.3 0.0250 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥75% From Baseline to Week 16
37.1; 59.4 0.0250 sig
SECONDARY
Percent of Participants Achieving a Reduction in BPDAI Activity Score of ≥90% From Baseline to Week 16
32.7; 45.8 0.2089
SECONDARY
Percent Change in Weekly Average of Daily Peak Pruritus NRS From Baseline to Week 16
-36.4; -50.4 0.0767
SECONDARY
Percent of Participants With Improvement (Reduction) of Weekly Average of Daily Peak Pruritus NRS ≥4 From Baseline to Week 16
33.5; 50.9 0.0761
SECONDARY
Number of Participants With At Least One Treatment-emergent Adverse Event (TEAE) Through Week 52
51; 51
SECONDARY
Number of Participants With At Least One Serious TEAE Through Week 52
16; 12
SECONDARY
Number of Participants With At Least One TE Adverse Event of Special Interest (AESI) Through Week 52
0; 4
SECONDARY
Number of Participants With At Least One TEAE Through Week 64
23; 25
SECONDARY
Number of Participants With At Least One Serious TEAE Through Week 64
8; 8
SECONDARY
Number of Participants With At Least One TE AESI Through Week 64
0; 0
SECONDARY
Concentrations of Functional Dupilumab in Serum
0; 50.2; 66.7; 71.0; 78.6; 8.73
SECONDARY
Number of Participants With TE Anti-drug Antibody (ADA) Response Per Maximum Titer Category
0; 1; 0; 1; 0; 0

Summary

The main purpose of this study is to investigate whether dupilumab is effective and safe for the treatment of bullous pemphigoid. Dupilumab is a type of drug called a "monoclonal antibody". An antibody is a special kind of protein that the immune (defense) system normally makes to fight bacteria and viruses. Bullous pemphigoid is an autoimmune subepidermal blistering disease, predominately affecting the elderly (typical onset after age 60). The study is looking at several other research questions, including: * Side effects that may be experienced by people taking dupilumab * How dupilumab works in the body and affects the body * How dupilumab affects quality of life * How much dupilumab is present in the blood * To see if dupilumab works to wean the patient off oral corticosteroids

Eligibility Criteria

Key Inclusion Criteria

  • Patients must have characteristic clinical features of bullous pemphigoid (BP) (eg, urticarial or eczematous or erythematous plaques, bullae, pruritus) at the screening and baseline visits.
  • Study participants are required to have a confirmed diagnosis of BP based on histopathology, immunopathology, and serology at the baseline visit, as defined in the protocol.
  • Bullous Pemphigoid Disease Area Index (BPDAI) activity score ≥24 at baseline and screening visits.
  • Baseline peak pruritus NRS score for maximum itch intensity ≥4
  • Karnofsky performance status score ≥50% at the screening visit.

Key Exclusion Criteria

  • Forms of pemphigoid other than classic BP (eg, Brunsting-Perry cicatricial pemphigoid, anti-p200 pemphigoid, epidermolysis bullosa acquisita, or BP with concomitant pemphigus vulgaris)
  • Patients who are receiving treatments known to cause or exacerbate BP (eg, angiotensin converting enzyme inhibitors, penicillamine, furosemide, phenacetin, dipeptidyl peptidase 4 inhibitor) who have not been on a stable dose of these medications for at least 4 weeks prior to the screening visit
  • Have ever received treatment with an IL-4 or IL-13 antagonist such as dupilumab, tralokinumab, or lebrikizumab.
  • Treatment with systemic corticosteroids within 7 days before the baseline visit
  • Treatment with topical corticosteroids of medium potency or higher, topical calcineurin inhibitor, or topical crisaborole within 7 days before the baseline visit
  • Treatment with non-steroidal immunosuppressive/immunomodulating drug(s) (eg, mycophenolate mofetil, azathioprine, or methotrexate) within 4 weeks before the baseline visit.
  • Treatment with BP-directed biologics as follows:
  • Any cell-depleting agents including but not limited to rituximab: within 12 months before the baseline visit, or until lymphocyte and CD 19+ lymphocyte count returns to normal, whichever is longer
  • Other biologics (such as IL-5 inhibitors benralizumab or mepolizumab): within 5 half-lives (if known) or 16 weeks prior to the baseline visit, whichever is longer
  • Intravenous immunoglobulin within 16 weeks prior to the baseline visit

NOTE: Other Protocol Defined Inclusion/Exclusion Criteria Apply

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04206553). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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