Table 2 Characteristics of 25 EGPA patients treated with mepolizumab for at least 3 years

Age at study admission (years), mean ± SD

59.1 ± 10.9

58.9 ± 11.7

NS

Sex (M/F)

8/7

2/8

NS

Age at asthma onset (years), mean ± SD

34.1 ± 16.9

39.4 ± 19.6

NS

Age at EGPA onset (years), mean ± SD

42.6 ± 16.1

45.9 ± 13.3

NS

Period of time from EGPA onset to study admission (years), mean ± SD

16.3 ± 8.7

13.2 ± 8.8

NS

Laboratory data at diagnosis

 WBC count (/μL), mean ± SD

18 195.3 ± 7045.1

13 232.9 ± 9150.7

 < 0.05

 Blood eosinophils (/μL), mean ± SD

10 580.4 ± 6370.4

6935.3 ± 7549.5

 < 0.05

 MPO-ANCA yes/no (%)

6/9 (40.0)

2/8 (20.0)

NS

 PR3-ANCA yes/no (%)

1/14 (6.7)

0/10 (0)

NS

 IC > 1.5 mg/mL yes/no (%)

3/8 (27.3)

2/5 (28.6)

NS

 RF > 15 IU/mL yes/no (%)

5/7 (41.7)

3/4 (42.9)

NS

 BNP > 18.7 pg/mL yes/no (%)

3/9 (25.0)

3/4 (42.9)

NS

 IgE (IU/mL), median (range)

545 (35–4950)

342 (34–28,403)

NS

 IgG (IU/mL), mean ± SD

1612.4 ± 747.9

1802.1 ± 684.0

NS

Blood eosinophil count

 Before mepolizumab (/μL), mean ± SD

203.2 ± 267.9

78.6 ± 125.8

NS

 At last examination (/μL), mean ± SD

14.2 ± 36.9

7.6 ± 14.9

NS

 Lowest serum IgG before administration of mepolizumab (IU/mL), mean ± SD

801.7 ± 194.0

608.7 ± 211.0

 < 0.05

Clinical manifestations at diagnosis, yes/no (%)

 Asthma

15/0 (100)

10/0 (100.0)

NS

 Paranasal sinusitis

15/0 (100)

9/1 (90.0)

NS

 Eosinophilic otitis media

4/11 (26.7)

4/6 (40.0)

NS

 Multiple polyneuropathy

14/1 (93.3)

10/0 (100)

NS

 Minimum MMT score

3.8 ± 0.8

3.3 ± 0.9

NS

 Pulmonary involvement

6/7 (46.2)

4/5 (44.4)

NS

 Myocardial involvement

11/4 (73.3)

9/1 (90.0)

NS

 Gastrointestinal tract involvement

13/2 (86.7)

8/2 (80.0)

NS

 Liver, gall bladder, pancreas

2/11 (15.4)

3/9 (33.3)

NS

 Renal involvement

6/9 (40.0)

4/6 (40.0)

NS

 Skin involvement

10/4 (71.4)

8/1 (88.9)

NS

 Arthralgia

7/8 (46.7)

8/1 (88.9)

 < 0.05

 Myalgia

2/12 (14.3)

5/4 (55.6)

 < 0.05

 Central nervous system involvement

5/10 (33.3)

3/7 (30.0)

NS

BVAS

 At diagnosis, mean ± SD

32.3 ± 5.9

35.5 ± 7.6

NS

 Before mepolizumab, mean ± SD

15.1 ± 4.9

20.2 ± 4.6

 < 0.05

 At last visit on mepolizumab, mean ± SD

5.2 ± 5.3

13.0 ± 5.0

 < 0.01

Relapse rates before mepolizumab treatment (times/year), mean ± SD

 Frequency of relapse from diagnosis to 1 year or more before mepolizumab

0.4 ± 0.4

1.0 ± 0.8

NS

 Frequency of relapse within 1 year before mepolizumab initiation

2.2 ± 0.8

2.8 ± 0.9

NS

 Frequency of relapse 1 year after mepolizumab initiation

1.1 ± 0.6

3.7 ± 2.1

 < 0.01

 Frequency of relapse 2 years after mepolizumab initiation

0.8 ± 0.7

3.1 ± 2.3

 < 0.01

 Frequency of relapse 3 years after mepolizumab initiation

0.6 ± 0.6

3.5 ± 2.4

 < 0.01

 Frequency of relapse from mepolizumab initiation to final visit

0.4 ± 0.4

3.4 ± 2.4

 < 0.01

Initial treatment

 Pulsed mPSL yes/no (%)

10/5 (66.7)

5/5 (50.0)

NS

 PSL (mg), mean ± SD

48.0 ± 12.1

41.5 ± 8.8

NS

 Immunosuppressant yes/no (%)

13/2 (86.7)

10/0 (100)

NS

 CYC/AZA/CsA/MTX/RTX

9/1/2/1/0

6/1/2/1/0

NS

Maintenance treatment before mepolizumab

 Immunosuppressant yes/no (%)

10/5 (66.7)

10/0 (100.0)

 < 0.05

 CYC/AZA/CsA/MTX/RTX

0/2/2/6/0

0/2/0/6/2

NS

 PSL (mg), mean ± SD

11.7 ± 3.7

15.5 ± 8.6

NS

 IVIG yes/no (%)

12/3 (80.0)

10/0 (100)

NS

 Duration of mepolizumab administration (years), mean ± SD

4.3 ± 1.0

4.2 ± 1.0

NS

Maintenance treatment at last visit on mepolizumab

 Immunosuppressant yes/no (%)

11/4 (73.3)

10/0 (100.0)

NS

 CYC/AZA/CsA/MTX/RTX

0/2/2/7/0

0/3/0/4/3

NS

 PSL (mg), mean ± SD

7.6 ± 2.2

14.2 ± 6.4

 < 0.01