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
N1 - Funding Information:
MH has received research grants and/or honoraria from Abbott Japan Co., Ltd, Astellas Pharma Inc., Bristol-Myers Squibb K.K., Chugai Pharmaceutical Co., Ltd, Eisai Co., Ltd, Janssen Pharmaceutical K.K., Mitsubishi Tanabe Pharma Co., Santen Pharmaceutical Co., Ltd, Takeda Pharmaceutical Co., Ltd, Teijin Pharma, Ltd, and Pfizer Japan Inc. TF has received research grants from Abbott Japan Co., Ltd, Astellas Pharma Inc., Bristol-Myers Squibb K.K., Chugai Pharmaceutical Co., Ltd, Daiichi-Sankyo Pharmaceutical Co. Ltd, Eisai Co., Ltd, Mitsubishi Tanabe Pharma Co, Takeda Pharmaceutical Co., Ltd, and Pfizer Japan Inc. HM serves as a consultant to AbbVie Inc., Astellas Pharma Inc., and Teijin Pharma Ltd; received honoraria from Astellas Pharma Inc., MSD K.K., Takeda Pharmaceutical Co., Ltd, and Mitsubishi Tanabe Pharma Co.; and received research funding from Astellas Pharma Inc., Daiichi Sankyo Inc., Dainippon Sumitomo Pharma Co., Ltd, MSD K.K., Novo Nordisk Pharma Ltd, and Takeda Pharmaceutical Co., Ltd.
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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U2 - 10.1186/ar4550
DO - 10.1186/ar4550
M3 - Article
C2 - 24758294
AN - SCOPUS:84900436550
SN - 1478-6354
VL - 16
JO - Arthritis Research and Therapy
JF - Arthritis Research and Therapy
IS - 2
M1 - R101
ER -