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Table 1 Comparison of demographics and disease states among AAV diseases

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

  EGPA GPA MPA/RLV Unclassifiable
(n = 14) (n = 33) (n = 78) (n = 31)
Male/female 5/9 12/21 35/43 9/22
Mean (median) age (years)a,c 58.0 ± 16.9 (62) 63.6 ± 12.6 (61) 71.1 ± 10.0 (73) 70.6 ± 11.8 (73)
MPO-ANCAa,c 7 (50.0) 18 (54.6) 76 (97.4) 29 (93.5)
PR3-ANCAb,c 0 (0) 15 (45.5) 2 (2.6) 1 (3.2)
ANCA-negativea,b 7 (50.0) 3 (9.1) 1 (1.3) 2 (6.5)
Serum creatinine (mg/dl)a 0.71 ± 0.39 1.51 ± 1.32 2.46 ± 2.18 0.69 ± 0.23
Disease severityc
 Localised 0 (0) 4 (12.1) 0 (0) 0 (0)
 Early systemic 1 (7.1) 5 (15.1) 15 (19.2) 15 (48.4)
 Generalised 13 (92.9) 18 (54.6) 47 (60.3) 13 (41.9)
 Severe 0 (0) 6 (18.2) 16 (20.5) 3 (9.7)
General performanced
 0/1/2/3/4 1/7/2/4/0 8/11/2/11/1 10/29/17/16/6 3/11/7/9/1
Nongranulomatous pulmonary involvement
 Interstitial lung diseasea 2 (14.3) 3 (9.0) 37 (47.4) 19 (61.3)
 Alveolar haemorrhage 0 (0) 2 (6.1) 9 (11.5) 2 (6.5)
  1. Values expressed as mean ± standard deviation or number (percentage) unless otherwise noted. AAV, antineutrophil cytoplasmic antibody-associated vasculitis; ANCA, antineutrophil cytoplasmic antibody; EGPA, eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome); GPA, granulomatosis with polyangiitis (Wegener’s granulomatosis); MPA, microscopic polyangiitis; MPO, myeloperoxidase; PR3, proteinase-3; RLV, renal-limited vasculitis. Comparisons between the EGPA, GPA, and MPV/RLV groups were made by Student t test or Mann–Whitney U test. Statistical significance was determined by P < 0.05/3 using Bonferroni correction: aEGPA versus MPA/RLV, bEGPA versus GPA, cGPA versus MPA/RLV. Unclassifiable AAV was not compared with other forms of AAV. dGeneral performance was categorised according to the World Health Organization performance status except category 5 (death).