Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study

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.

Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

“…As for clinical course, we elucidated that most Japanese patients with MPA and GPA received treatment with high-dose glucocorticoid and limited-dose cyclophosphamide (CY), and showed high remission and relapse-free survival rates. However, remission with prednisolone at <10 mg/day was observed only in 40% of the patients with remission at 6 months [8,9]. To further analyze the The extent and pattern of organ involvement were quite different between patients with AAV, and the European League Against Rheumatism (EULAR) recommends that patients with vasculitis should be categorized into clearly defined disease states.…”

“…Currently, the European Vasculitis Study Group (EUVAS)-defined disease severity, Five-Factor Score (FFS), and RPGN clinical grading system could be used for this purpose [10][11][12]. The RemIT-JAV study revealed that the EUVAS-defined disease severity, especially the severe form, was useful for predicting poor renal and vital prognoses [9].…”

Granulomatose com poliangiite

“…4 . A forma localizada manifesta-se primariamente com envolvimento de ouvido, nariz e garganta, é limitada ao trato respiratório superior e inferior sem envolvimento sistêmico ou sintomas constitucionais 1,4,11,20 . A forma sistêmica inicial pode acometer qualquer órgão ou sistema, sem conferir risco de dano irreversível a órgãos ou risco de morte.…”

“…Recomenda-se a combinação de corticosteroides sistêmicos com outras drogas imunossupressoras 20,21 . Nesse caso, prednisona ou prednisolona 0,5 a 1mg/kg/ dia (dose máxima de 80mg/dia) pode ser utilizada por 1 a 4 semanas.…”

Azathioprine Intolerance in Japanese Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitis

“…A nationwide prospective inception cohort study in Japan revealed that AAV classification, the type of organs involved, the age of onset, and the treatment patterns were different from those in Western countries (2, 3). …”