Background Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), Churg-Srauss syndrome (CSS) and microscopic polyangiitis (MPA) constitute the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Treatment response and prognosis, as well as disease evolution and organ involvement, may differ substantially between these diseases. In contrast with the predominance of GPA in Western countries, MPA is much more common than GPA in Japan.
Objectives To investigate the effectiveness and safety of remission induction therapy currently performed for AAV in Japan.
Methods Patients with newly diagnosed AAV were enrolled in the RemIT-JAV study by members of the MHLW intractable vasculitis research group. Information regarding patients’ disease status and initial immunosuppressive therapy was collected, and their responses were analyzed by categorizing into 3 groups with different disease severity according to BSR/BHPR guidelines.
Results Data from 132 patients, of the 156 patients enrolled in the study (CSS n=17, GPA n=34, MPA n=94, unclassified n=11), became available at the end of March, 2011. All patients received corticosteroids (CS) in the initial treatment for AAV; and yclophosphamide (CY) was added within 2 weeks in 33.3% (11/33) of patients with localized/early systemic disease and in 35.3% (35/99) of those with generalized or severe disease. 90% and 95% of patients with localized/early systemic disease achieved remission within 6 months when treated with or without CY combination, respectively. Similarly, 86% and 91% of patients with generalized or severe disease did so with and without CY combination, respectively. A total of 7 patients with generalized and severe disease died due to disease progression and concomitant infections, in which 5 patients had been treated with CS alone and 2 patients with CS plus CY therapy.
Conclusions In our study, Japanese AAV patients, irrelevant to disease severity, showed good responses to CS alone therapy in terms of initial remission rates, which was mostly equivalent to the results of CS plus CY therapy. Further analysis is warranted to determine the role of CY combination in long-term outcomes.
Disclosure of Interest None Declared
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