Zanubrutinib in Patients With IgG4-Related Disease

Matthew C. Baker

Status and phase

Completed

Phase 2

Conditions

IgG4 Related Disease

Treatments

Drug: Zanubrutinib 80 MG

Study type

Interventional

Funder types

Other

Identifiers

NCT04602598

58497

Details and patient eligibility

About

The aim of this clinical trial is to evaluate the safety and efficacy of zanubrutinib in treating patients with IgG4-related disease

Full description

This will be a single-site, open-label study in symptomatic patients with IgG4-related disease affecting the submandibular and/or lacrimal glands. All patients will receive zanubrutinib orally at a dose of 80mg BID for 24 weeks.

The primary objective of this study is to demonstrate that zanubrutinib treatment reduces reduces the volume of the submandibular and/or lacrimal glands on PET/MRI at week 24 compared to baseline.

Enrollment

10 patients

Sex

All

Ages

18 to 85 years old

Volunteers

No Healthy Volunteers

Inclusion criteria

Men or women aged 18 to 85, inclusive, at the time of initial screening
Have histopathologically confirmed IgG4-RD in the submandibular gland and/or the lacrimal gland confirmed by international consensus pathology criteria
- Presence of a lymphoplasmacytic infiltrate with 10 IgG4+ plasma cells per high-power field and/or an IgG4+/IgG+ plasma cell ratio of 40%
All women must test negative for pregnancy and agree to use a reliable method of birth control
No current treatment with immunosuppressive medications other than prednisone 40mg daily (or other glucocorticoid equivalent) with stable dosing for 28 days

Exclusion criteria

Unstable prescribed dose of glucocorticoids within 28 days prior to baseline
Any treatment with a synthetic DMARD including but not limited to hydroxychloroquine, methotrexate, leflunomide, or sulfasalazine within 28 days prior to baseline
Any treatment with a cytotoxic or immunosuppressive drug including but not limited to cyclophosphamide, mycophenolic acid, azathioprine, cyclosporine, sirolimus, or tacrolimus within 28 days prior to baseline
Any treatment with a BTK inhibitor within 6 months before baseline
Any treatment with a JAK inhibitor within 28 days prior to baseline
Use of biologic agents including infliximab, abatacept, or tocilizumab within 56 days prior to baseline
Use of a B cell depleting therapy (such as rituximab) within 12 months prior to baseline
A history of, or current, inflammatory or autoimmune disease (that could affect the interpretation of safety or efficacy outcomes) other than IgG4-related disease
Evidence of active tuberculosis, HIV, or hepatitis B or C infection
History of cancer other than non-melanoma skin cancer, cervical dysplasia or carcinoma in situ (cured >1 year), prostate cancer (cured >5 years), or colon cancer (cured >5 years)