Table 1 Baseline characteristics, initial treatments, and outcomes for 194 patients with MPA in the REVEAL cohort

Age, year

76.2 (71.5–83.1)

72.3 (65.9–77.2)

< 0.0001

Female, n (%)

29(48.3)

78 (58.2)

0.215

Observation period from baseline, weeks

128.5 (35.3–248)

229 (112–392)

< 0.0001

Smoking history, n (%)

31 (56.4)^a

63 (51.6)^b

0.627

Smoking index

950 (800–1200)

600 (300–920)

0.002

Laboratory findings

 WBC, /mm3

12005 (8063–16,648)

10110(7300–13,815)

0.086

 Hb, g/dL

9.7 (8.2–11.9)

10.3 (9.0–11.9)^c

0.325

 Alb, g/dL

2.5 (2.1–2.9)

2.8 (2.3–3.3)^c

0.025

 Cre, mg/dL

1.34 (0.8–2.2)

1.0 (0.67–2.04)

0.056

 CRP, mg/dL

9.3 (4.19–13.29)

6.8 (1.48–12.0)

0.031

 Positive, anti-MPO-ANCA, n (%)

59(98.3)

133 (99.3)

0.524

 Positive, anti-PR3-ANCA, n (%)

3 (5.0)

7 (5.2)

1

 MPO-ANCA titre, U/mL

122.5 (57.7–240.8)

119 (46.1–251.0)

0.95

BVAS at onset

17.5 (11.3–22.8)

12 (7.0–18.0)

0.005

Five factor score 2009

 ≤1

5 (8.3)

34 (25.4)

0.019

 2

39 (65.0)

68 (50.8)

 ≥3

16 (26.7)

32 (23.9)

EUVAS categorization system

 Localized

1 (1.7)

6 (4.5)

0.037

 Early systemic

12 (20.0)

34 (25.4)

 Systemic

30 (50.0)

78 (58.2)

 Severe

17 (28.3)

16 (11.9)

Initial treatment

 PDN, mg/kg/day

1.0 (0.6–1.0)

1.0 (0.6–1.0)^c

0.829

 MPDN pulse therapy, n (%)

20 (35.1)

40 (32.0)

0.735

Immunosuppressants

 IVCY, n (%)

21 (37.5)

50 (42.7)

0.621

 Total IVCY dose (g)

0.13 (0–1.0)

0.0 (0–2.0)

0.589

 RTX, n (%)

4 (6.7)

12 (9.0)

0.78

 IVIG, n (%)

1 (1.7)

7 (5.2)

0.439

 AZA, n (%)

33 (55.0)

70 (52.2)

0.757

 MMF, n (%)

1 (1.7)

2 (1.5)

1

 Plasma exchange, n (%)

9 (15.0)

6 (4.5)

0.018

Haemodialysis, n (%)

7 (11.7)

10 (7.5)

0.411

Relapse, n (%)

22 (36.7)

50 (37.3)

1

Relapse, time

0 (0–1)

0 (0–1)

0.83

Cause of death, n (%)

MPA-related vasculitis 11 (18.3), infections 30 (50.0), others 19 (31.7)

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