Zanubrutinib in Patients With IgG4-Related Disease

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)