Immunoglobulin G4-Related Disease complicated by allergic disease.
Conditions
Interventions
Start corticosteroid on Day 1, add Dupilumab by Day 14, and continued for the 52 weeks.
Sponsors
Furuta Shunsuke
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) The patient is at least 18 years old at the time of informed consent. 2) Fulfillment the Comprehensive Diagnostic Criteria for IgG4-Related Diseases Revised in 2020. (If the patient meets the criteria by organ-specific diagnostic criteria, it is still acceptable.) 3) Patients newly diagnosed with IgG4-RD according to 2), who have active IgG4-RD signs that require the initiation of GC(Glucocorticoid) therapy. Or, among patients already diagnosed with IgG4RD and on immunosuppressive therapy, who have active IgG4-RD signs that require the increased dose of GC. 4) Patients with concomitant allergic diseases who meet the following criteria Bronchial asthma: symptomatic with inhaled steroids Chronic sinusitis: symptomatic with nasal steroids Atopic dermatitis: symptomatic with topical steroids Chronic urticaria: symptomatic with histamine receptor antagonists Allergic rhinitis: symptomatic with nasal steroids and histamine receptor antagonists 5) Patients who have been fully informed, fully understood, and have given their written consent to participate in this study.
Exclusion criteria
Exclusion criteria: 1) History of B-cell elimination therapy or B-cell targeted therapy within 6 months. 2) Comorbidity of other systemic autoimmune diseases 3) HIV infection, hepatitis B virus, hepatitis C virus infection, or a history of such infection 4) Coexisting active tuberculosis 5) Coexisting active severe infection. 6) Pregnant, possibly pregnant, within 28 days postpartum, or breastfeeding. 7)History of malignancy within 5 years (except for intraepithelial carcinoma that is considered curatively resected). 8) Patients with uncontrolled complications. 9) The principal investigator or subinvestigator determines that the study is not appropriate for safe conduct of the study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Percentage of patients who remained in remission and achieved corticosteroid-free at 52 weeks on protocol-specified therapy. *Definition of Remission The following 1 and 2 must be satisfied. (1) GC is less than or equal to 5 mg/day of prednisolone equivalent. (2) Absence of clinical signs of IgG4-related disease (equivalent to a score of 0 in the Organ/Site score of the IgG4-RD Responder Index, regardless of serum IgG4 levels). Clinical signs associated with irreversible organ damage due to IgG4-RD are excluded.) | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary efficacy endpoints: 1) Percentage of relapse and relapse-free survival up to 52 weeks 2) Percentage of remission induction failure up to 52 weeks 3) Percentage of patients for remission induction failure or relapse up to 52 weeks 4) Changes in blood test values (IgG, IgG4, IgE, eosinophils) 5) Changes in IgG4-RD Responder Index(Organ/site score) 6) Changes in IgG4-RD Responder Index (sum of Organ/site score and Serum IgG4 score) 7) Response rate at 4, 12, 26 and 52 weeks 8) Dose and cumulative dose of corticosteroids at 4, 12, 26, and 52 weeks 9) Dose and cumulative dose of corticosteroids at relapse 10) Change in disease-specific patient-reported outcomes for each allergic disease 11) Proportion of patients who achieved the minimal clinically important difference in all coexisting allergic diseases 12) Proportion of patients who achieved the minimal clinically important difference in each allergic disease 13) Proportion of patients who had an Organ/Site score of 3 at baseline and achieved a score of 0 at Week 52 for each organ involvement. Allergic rhinitis: JRQLQ (Japanese rhino conjunctivitis quality of life questionnaire) Atopic dermatitis: POEM score (Patient-Oriented Eczema Measure) Chronic urticaria: UCT (Urticaria Control Test) Bronchial asthma: ACQ5 (Asthma Control Questionnaire) Chronic sinusitis: SNOT-22 (22-item Sino-Nasal Outcome Test) Secondary safety endpoint: percentage of adverse events *Definition of Relapse After remission, the following 1 and 2 must be satisfied (1) Worsening of clinical signs due to IgG4-RD (equivalent to increase of 2 points or more from the previous score in the Organ/Site score of the IgG4-RD Responder Index. (regardless of serum IgG4 levels.)) (2) Intensified immunosuppressive therapy (increased dose of GC or addition of new therapy) | — |
Contacts
Public ContactShuhei Kameda
Chiba University Hospital
Outcome results
None listed