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Table 1 Patients’ characteristics

From: Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Number

Disease duration (months)

Organ involvement

FFS

ANCA IF ELISA

BVAS before induction

BVAS at RTX infusion

Organ involvement at RTX

Eosinophils/μl (%)

IgE IU/ml

Previous treatment

CYC (sum dose, g)

GC dose (mg)

Concomitant treatment after RTX

Total observation (months)

Additional RTX courses

1

24

L, ENT, C

2

p

10

10

L↔, ENT↔, C↑

281 (6.9)

50

None

7.1

15

AZA

19

None

MPO

2

12

L, ENT, C, PNS

1

(p)

18

18

L↑, ENT↑, C↔, PNS ↔

8,677 (43)

869

AZA

9.8

40

AZA

6

None

(-)

3

66

L, ENT, PNS, CNS

1

p

14

14

L↓, ENT↔, PNS↑, CNS↑

663 (6.2)

269

MTX, AZA

(-)

10

AZA

6

None

MPO

4

5

L, ENT, C, PNS, S

2

(-)

28

16

L↔, ENT↔, C↓, PNS↑, S↓

1,057 (9.7)

1,030

None

10

25

AZA

32

1 more course at 6 months (preemptive)

(-)

5

16

L, ENT, C

1

(-)

19

19

L↑, ENT↔, C↔

4,235 (35)

76

MMF, AZA

9.7

20

AZA

34

3 more courses 6 monthly (preemptive)

(-)

6

184

L, ENT, PNS, S, K

1

p

19

13

L↓, ENT↑, PNS↑, S↑, K↓

3,542 (22)

17

CYC, AZA, MTX, LEF

8

7.5

MMF

36

5 more courses 6 monthly (preemptive)

MPO

7

35

L, ENT, C, S, PNS, CNS, K

1

(-)

36

23

L↑, ENT↓, C↑, S↓, PNS↔, CNS↓, K↓

8,434 (41)

528

CYC, MTX

12

40

AZA

13

None

(-)

8

7

L, PNS, K

1

p

13

9

L↔, PNS↑, K↓

2,700 (27)

332

AZA

9.9

15

AZA

6

None

MPO

9

8

L, ENT, PNS

0

c

13

10

L↑, ENT↑, PNS↑

3,536 (52)

206

MTX

(-)

15

MTX

6

None

 

MPO

 
  1. The disease duration refers to the time from diagnosis to first RTX treatment. BVAS and organ involvement was evaluated before CYC induction and before the first RTX treatment. Eosinophil counts and IgE concentration were measured before induction therapy with CYC (n = 7), MTX (n = 1), or AZA (n = 1). Abbreviations for organ involvement: ear nose throat (ENT), cardiac (C), kidney (K), lung (L), peripheral nervous system (PNS), central nervous system (CNS), and skin (S). FFS, five-factor score; IF, immunofluorescence; ELISA, enzyme-linked immunosorbent assay; BVAS, Birmingham Vasculitis Activity Score. Arrows, organ involvement at the time of RTX treatment compared with initial manifestation before standard therapy: ↑, worsening of organ involvement; ↓, improvement of organ involvement; ↔ stable organ involvement.