Abstract
IntroductionThis study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).MethodsPatients with newly diagnosed MPA and GPA were enrolled in a nationwide, prospective, inception cohort study from 22 tertiary Japanese institutions, and treatment patterns and responses were evaluated for 24 months. Primary outcome measures were rates of remission (Birmingham Vasculitis Activity Score, 0) and remission with low-dose glucocorticoids (GC) (prednisolone ≤ 10 mg) (GC remission).ResultsOf 156 enrolled patients, 78 MPA patients and 33 GPA patients were included. Concomitant cyclophosphamide (CY) was used in 24 MPA (31 %) and 20 GPA (60 %) patients during the initial 3 weeks of treatment. After 6 months, remission was achieved in 66 MPA (85 %) and 29 GPA (87 %) patients, while GC remission was obtained in only 31 MPA (40 %) and 13 GPA (39 %) patients. During the 24-month period, 14 MPA patients and 2 GPA patients died; end stage renal disease (ESRD) was noted in 13 MPA patients but no GPA patients. Patients with severe disease, according to the European Vasculitis Study Group (EUVAS) classification, showed poorer ESRD-free and overall survival rates than those with generalized disease (p < 0.0001). There were no differences in relapse-free survival rates between GPA and MPA, among EUVAS-defined disease severity categories, and between anti-neutrophil cytoplasmic antibody subspecialties.ConclusionsThe majority of Japanese patients with MPA and GPA received treatment with high-dose GC and limited CY use, and showed high remission and relapse-free survival rates but low GC remission rates in clinical practice.Trial registrationUniversity Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.
Highlights
This study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA)
We have previously reported the demographic and baseline clinical characteristics of Japanese patients with associated vasculitis (AAV) who were enrolled in a nationwide, prospective, inception cohort study of Remission Induction Therapy in Japanese patients with Antineutrophil cytoplasmic antibody (ANCA)-associated Vasculitides (RemIT-JAV)
The criteria for enrolment included a diagnosis of AAV made by the site investigators that fulfilled the criteria for primary systemic vasculitis proposed by the European Medicines Agency (EMEA) algorithm: symptoms and signs characteristic of or compatible with a diagnosis of AAV or polyarteritis nodosa; at least one of histological proof of vasculitis, positive serology for ANCA, specific investigations strongly suggestive of vasculitis and/or granuloma, or eosinophilia; and no other diagnosis to account for symptoms/signs [2]
Summary
This study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). The guidelines of the British Society of Rheumatology (BSR) and the European League Against Rheumatism (EULAR) recommended assessment for treatment with high-dose glucocorticoids (GC) and concomitant cyclophosphamide (CY) or rituximab as the first-line option for all patients with newly diagnosed AAV, in principle [7, 8]. These recommendations are based on several randomized controlled trials (RCTs) enrolling more patients with GPA than with MPA [9,10,11]. Since patients with MPA were older and tended to exhibit higher levels of creatinine than those with GPA in our cohort of Japanese patients [5], these recommendations should be applied to Asian patients with caution in terms of safety
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