Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

Ken ei Sada, Masahiro Yamamura, Masayoshi Harigai, Takao Fujii, Hiroaki Dobashi, Yoshinari Takasaki, Satoshi Ito, Hidehiro Yamada, Takashi Wada, Junichi Hirahashi, Yoshihiro Arimura, Hirofumi Makino, Koichi Amano, Kunihiro Yamagata, Shouichi Fujimoto, Sakae Homma, Shunichi Kumagai, Eri Muso, Yohko Murakawa, Shogo Banno & 6 others Hitoshi Hasegawa, Wako Yumura, Hiroaki Matsubara, Masaharu Yoshida, Kensei Katsuoka, Noriyoshi Ogawa

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Abstract

Introduction: We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.Methods: In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.Results: Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).Conclusions: MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.Trial Registration: The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.

Original languageEnglish
Article numberR101
JournalArthritis Research and Therapy
Volume16
Issue number2
DOIs
Publication statusPublished - 2014 Apr 23
Externally publishedYes

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Antineutrophil Cytoplasmic Antibodies
Granulomatosis with Polyangiitis
Vasculitis
Cohort Studies
Microscopic Polyangiitis
Interstitial Lung Diseases
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Kidney
Peroxidase
Pharynx
Nose
Ear
Myeloblastin
Information Services
Nervous System
Registries

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. / Sada, Ken ei; Yamamura, Masahiro; Harigai, Masayoshi; Fujii, Takao; Dobashi, Hiroaki; Takasaki, Yoshinari; Ito, Satoshi; Yamada, Hidehiro; Wada, Takashi; Hirahashi, Junichi; Arimura, Yoshihiro; Makino, Hirofumi; Amano, Koichi; Yamagata, Kunihiro; Fujimoto, Shouichi; Homma, Sakae; Kumagai, Shunichi; Muso, Eri; Murakawa, Yohko; Banno, Shogo; Hasegawa, Hitoshi; Yumura, Wako; Matsubara, Hiroaki; Yoshida, Masaharu; Katsuoka, Kensei; Ogawa, Noriyoshi.

In: Arthritis Research and Therapy, Vol. 16, No. 2, R101, 23.04.2014.

Research output: Contribution to journalArticle

Sada, KE, Yamamura, M, Harigai, M, Fujii, T, Dobashi, H, Takasaki, Y, Ito, S, Yamada, H, Wada, T, Hirahashi, J, Arimura, Y, Makino, H, Amano, K, Yamagata, K, Fujimoto, S, Homma, S, Kumagai, S, Muso, E, Murakawa, Y, Banno, S, Hasegawa, H, Yumura, W, Matsubara, H, Yoshida, M, Katsuoka, K & Ogawa, N 2014, 'Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study', Arthritis Research and Therapy, vol. 16, no. 2, R101. https://doi.org/10.1186/ar4550
Sada, Ken ei ; Yamamura, Masahiro ; Harigai, Masayoshi ; Fujii, Takao ; Dobashi, Hiroaki ; Takasaki, Yoshinari ; Ito, Satoshi ; Yamada, Hidehiro ; Wada, Takashi ; Hirahashi, Junichi ; Arimura, Yoshihiro ; Makino, Hirofumi ; Amano, Koichi ; Yamagata, Kunihiro ; Fujimoto, Shouichi ; Homma, Sakae ; Kumagai, Shunichi ; Muso, Eri ; Murakawa, Yohko ; Banno, Shogo ; Hasegawa, Hitoshi ; Yumura, Wako ; Matsubara, Hiroaki ; Yoshida, Masaharu ; Katsuoka, Kensei ; Ogawa, Noriyoshi. / Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. In: Arthritis Research and Therapy. 2014 ; Vol. 16, No. 2.
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abstract = "Introduction: We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.Methods: In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.Results: Using the EMEA algorithm, we identified 14 patients (9.0{\%}) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2{\%}) with granulomatosis with polyangiitis (GPA), 78 (50.0{\%}) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9{\%}) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0{\%} and 0{\%} for EGPA, 54.6{\%} and 45.5{\%} for GPA, 97.4{\%} and 2.6{\%} for MPA/RLV, and 93.5{\%} and 3.2{\%} for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4{\%}) and nervous system (92.9{\%}) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9{\%}) and chest manifestations (66.7{\%}) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0{\%}) and GPA (63.6{\%}). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3{\%} for EGPA, 9.0{\%} for GPA, 47.4{\%} for MPA/RLV, and 61.3{\%} for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).Conclusions: MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.Trial Registration: The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.",
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TY - JOUR

T1 - Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

AU - Sada, Ken ei

AU - Yamamura, Masahiro

AU - Harigai, Masayoshi

AU - Fujii, Takao

AU - Dobashi, Hiroaki

AU - Takasaki, Yoshinari

AU - Ito, Satoshi

AU - Yamada, Hidehiro

AU - Wada, Takashi

AU - Hirahashi, Junichi

AU - Arimura, Yoshihiro

AU - Makino, Hirofumi

AU - Amano, Koichi

AU - Yamagata, Kunihiro

AU - Fujimoto, Shouichi

AU - Homma, Sakae

AU - Kumagai, Shunichi

AU - Muso, Eri

AU - Murakawa, Yohko

AU - Banno, Shogo

AU - Hasegawa, Hitoshi

AU - Yumura, Wako

AU - Matsubara, Hiroaki

AU - Yoshida, Masaharu

AU - Katsuoka, Kensei

AU - Ogawa, Noriyoshi

PY - 2014/4/23

Y1 - 2014/4/23

N2 - Introduction: We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.Methods: In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.Results: Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).Conclusions: MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.Trial Registration: The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.

AB - Introduction: We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.Methods: In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.Results: Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).Conclusions: MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.Trial Registration: The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.

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