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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).