Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.
There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan.



We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse.



Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p < 0.01). The proportion of patients with proteinase 3 (PR3)-ANCA was lower in Japan than in the UK (61.0% vs 85.2%, p < 0.01), while the proportion of myeloperoxidase-ANCA was higher in Japan than the UK (34.1% vs 8.6%, p < 0.01). Serum creatinine at diagnosis was lower in Japan than the UK (68.1 μmol/l vs 101.0 μmol/l, p < 0.01). Respiratory involvement was more frequent in Japan than the UK (69.5% vs 40.6%, p < 0.01). In both countries, most patients received both glucocorticoids and cyclophosphamide. At 60 months the cumulative survival rates were 87.6% and 94.3% in Japan and the UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p < 0.01).



Japanese patients with GPA were older at disease onset, with less PR3-ANCA positivity, milder renal dysfunction, and more frequent respiratory involvement than UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark. shfuruta@chiba-u.jp.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge. shfuruta@chiba-u.jp.
Shunsuke
S
Furuta
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Afzal N
AN
Chaudhry
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Yoshihiro
Y
Arimura
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Hiroaki
H
Dobashi
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Shouichi
S
Fujimoto
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Sakae
S
Homma
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
Niels
N
Rasmussen
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.|S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
David R
DR
Jayne
D000328:Adult; D017668:Age of Onset; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D018572:Disease-Free Survival; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D006113:United Kingdom
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids
ANTINEUTROPHIL CYTOPLASMIC ANTIBODY–ASSOCIATED VASCULITIS; EUROPE; GRANULOMATOSIS WITH POLYANGIITIS; JAPAN; OUTCOME; PHENOTYPE