Efficacy and safety of iguratimod on patients with relapsed or refractory IgG4-related disease

Objective To evaluate the therapeutic efficacy and safety of iguratimod on patients with relapsed or refractory IgG4-related disease (IgG4-RD). Methods We conducted a retrospective single-center study in 17 IgG4-RD patients admitted to Peking University People’s Hospital. Patients were given iguratimod, 25 mg, twice daily and clinical data were collected at 0, 12, and 24 weeks. The baseline treatments include prednisone, cyclophosphamide, leflunomide, mycophenolate mofetil, and methotrexate. Clinical manifestation, IgG4-RD responder index (IgG-RD RI), serological indexes, gland ultrasound findings, and adverse drug effect were recorded. IgG4-RD RI scores < 3 and declining ≥ 2 were recognized as complete response (CR); IgG4-RD RI scores declining ≥ 2 but remaining ≥ 3 were recognized as partial response (PR). If a patient’s IgG4-RD RI score was 3 at the beginning, PR was considered as a 1-point decrease after the therapy. Results Serum IgG4 decreased significantly from 708 (321–902) mg/dl at baseline to 446 (138–396) mg/dl at 24 weeks ( P = 0.0016). IgG4-RD RI decreased significantly from 9.79 ± 3.07 at baseline to 3.57 ± 1.09 at 24 weeks ( P < 0.0001). Overall, 2 (14.3%) patients achieved CR, 11 (78.6%) patients achieved PR, and 1 (7.14%) patient had no response to treatment at week 24. Serum IgG level and salivary glands major diameter also decreased significantly at week 12 and 24 after treatment. Conclusion Iguratimod can be a therapeutic strategy to achieve remission in relapsed or refractory IgG4-RD patients inadequately responding to corticosteroid treatment with or without other immunosuppressant treatment. Key messages • Iguratimod was effective for relapsed or refractory IgG4-RD patients. • Iguratimod can improve the clinical symptoms of patients, reduce the serum IgG and IgG4 levels, and can also reduce the volume of involved glands.