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

The Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Introduction: 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). Methods: Patients 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). Results: Of 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. Conclusions: The 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 registration: University Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.

Original languageEnglish
Article number305
JournalArthritis Research and Therapy
Volume17
Issue number1
DOIs
Publication statusPublished - 2015 Nov 2
Externally publishedYes

Fingerprint

Microscopic Polyangiitis
Granulomatosis with Polyangiitis
Cohort Studies
Glucocorticoids
Therapeutics
Vasculitis
Survival Rate
Cyclophosphamide
Chronic Kidney Failure
Recurrence
Antineutrophil Cytoplasmic Antibodies
Information Services

Keywords

  • Antineutrophil cytoplasmic antibody-associated vasculitis
  • Cyclophosphamide
  • Glucocorticoid
  • Granulomatosis with polyangiitis
  • Inception cohort
  • Microscopic polyangiitis
  • Prospective cohort

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

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. / The Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan.

In: Arthritis Research and Therapy, Vol. 17, No. 1, 305, 02.11.2015.

Research output: Contribution to journalArticle

@article{37c4379249434668bf90b55ccd78112a,
title = "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 = "Introduction: 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). Methods: Patients 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). Results: Of 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. Conclusions: The 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 registration: University Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.",
keywords = "Antineutrophil cytoplasmic antibody-associated vasculitis, Cyclophosphamide, Glucocorticoid, Granulomatosis with polyangiitis, Inception cohort, Microscopic polyangiitis, Prospective cohort",
author = "{The Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan} and Sada, {Ken ei} and Masahiro Yamamura and Masayoshi Harigai and Takao Fujii and Yoshinari Takasaki and Koichi Amano and Shouichi Fujimoto and Eri Muso and Yohko Murakawa and Yoshihiro Arimura and Hirofumi Makino and Hiroaki Dobashi and Hidehiro Yamada and Kunihiro Yamagata and Satoshi Ito and Sakae Homma and Takashi Wada and Shunichi Kumagai and Junichi Hirahashi and Shogo Banno and Hitoshi Hasegawa and Wako Yumura and Hiroaki Matsubara and Masaharu Yoshida and Kensei Katsuoka and Noriyoshi Ogawa",
year = "2015",
month = "11",
day = "2",
doi = "10.1186/s13075-015-0815-y",
language = "English",
volume = "17",
journal = "Arthritis Research and Therapy",
issn = "1478-6354",
publisher = "BioMed Central",
number = "1",

}

TY - JOUR

T1 - Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis

T2 - A nationwide prospective inception cohort study

AU - The Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan

AU - Sada, Ken ei

AU - Yamamura, Masahiro

AU - Harigai, Masayoshi

AU - Fujii, Takao

AU - Takasaki, Yoshinari

AU - Amano, Koichi

AU - Fujimoto, Shouichi

AU - Muso, Eri

AU - Murakawa, Yohko

AU - Arimura, Yoshihiro

AU - Makino, Hirofumi

AU - Dobashi, Hiroaki

AU - Yamada, Hidehiro

AU - Yamagata, Kunihiro

AU - Ito, Satoshi

AU - Homma, Sakae

AU - Wada, Takashi

AU - Kumagai, Shunichi

AU - Hirahashi, Junichi

AU - Banno, Shogo

AU - Hasegawa, Hitoshi

AU - Yumura, Wako

AU - Matsubara, Hiroaki

AU - Yoshida, Masaharu

AU - Katsuoka, Kensei

AU - Ogawa, Noriyoshi

PY - 2015/11/2

Y1 - 2015/11/2

N2 - Introduction: 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). Methods: Patients 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). Results: Of 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. Conclusions: The 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 registration: University Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.

AB - Introduction: 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). Methods: Patients 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). Results: Of 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. Conclusions: The 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 registration: University Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.

KW - Antineutrophil cytoplasmic antibody-associated vasculitis

KW - Cyclophosphamide

KW - Glucocorticoid

KW - Granulomatosis with polyangiitis

KW - Inception cohort

KW - Microscopic polyangiitis

KW - Prospective cohort

UR - http://www.scopus.com/inward/record.url?scp=84955749820&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84955749820&partnerID=8YFLogxK

U2 - 10.1186/s13075-015-0815-y

DO - 10.1186/s13075-015-0815-y

M3 - Article

VL - 17

JO - Arthritis Research and Therapy

JF - Arthritis Research and Therapy

SN - 1478-6354

IS - 1

M1 - 305

ER -