Skip to main content

Table 2 Demographics, organ involvement, disease assessments and treatment of the most common subtypes of vasculitis registered in Reuma.pt/vasculitis

From: Reuma.pt/vasculitis – the Portuguese vasculitis registry

 

TAK

GCA

PAN

MPA

GPA

EGPA

CV

BD

Number of patients (N)

28

125

26

22

41

29

20

298

Demographics

 Female/male ratio

6

1.8

0.9

3.4

1.6

2.2

4

3

 Mean age at onset, years (SD)

32.1

74.4

40.4

59.8

47.6

48.1

57.8

27

(13.8)

(8)

(20.5)

(12.5)

(14.8)

(16.3)

(12.8)

(12.7)

 Mean age at diagnosis, years (SD)

34.5

74.7

42.5

60.7

50

51.7

58.7

33.8

(14.6)

(7.8)

(18.9)

(12.3)

(14.1)

(14.9)

(13.6)

(12.1)

 Mean age at last visit, years (SD)

40

77.6

55.8

62.3

56.4

57.6

59.4

43.9

(15.8)

(7.9)

(18.1)

(15.2)

(12.1)

(15.5)

(15.3)

(14.9)

Organ involvement (%)

 Constitutional symptoms

56.5

59.5

62.5

60.0

69.4

45.8

47.1

26.6

 Musculoskeletal

34.8

60.7

79.2

70.0

65.7

54.2

76.5

60.2

 Skin

4.5

1.7

75.0

30.0

47.2

60.0

75.0

74.3

 Eyes

21.7

47.1

12.5

10.0

31.4

16.7

11.8

36.8

 ENT

8.7

50.0

8.7

30.0

82.9

62.5

11.8

4.5

 Chest / pulmonary

9.1

1.7

20.8

45.0

58.3

100

4.5

 Cardiovascular

87.5

69.7

50.0

10.0

8.6

29.2

17.6

17.3

 Gastrointestinal

29.2

4.9

20.8

20.0

17.1

16.7

11.8

98.5

  Oral ulceration

4.2

15.0

8.6

8.3

98.5

  Other symptoms

25.0

4.9

20.8

5.0

8.6

8.3

11.8

16.4

 Genitourinary

52.2

3.3

33.3

80.0

58.3

12.5

5.9

83.6

  Genital ulceration

4.3

83.6

  Other symptoms

47.8

3.3

33.3

80.0

58.3

12.5

5.9

4.0

 Neurologic

54.4

88.6

45.8

40.0

42.9

66.7

47.1

19.0

Diagnostic tests

 c-ANCA or ANCA-PR3 positivity

N/N of patients (%)

0/14

0/36

0/21

4/21

26/36

3/23

0/15

0/93

(0.0)

(0.0)

(0.0)

(19.0)

(72.2)

(13.0)

(0.0)

(0.0)

 p-ANCA or ANCA-MPO positivity

N/N of patients (%)

0/14

0/36

0/21

16/21

6/36

10/23

0/15

1/92

(0.0)

(0.0)

(0.0)

(76.2)

(16.7)

(43.5)

(0.0)

(1.1)

 Vascular imaging with vasculitis a

N/N of patients (%)

22/22

85/95

4/5

−/−

−/−

−/−

−/−

5/8

(100)

(89.5)

(80.0)

(62.5)

 Biopsy compatible with vasculitis b

N/N of patients (%)

2/3

47/72

16/22

6/8

7/21

6/11

1/1

−/−

(66.6)

(65.3)

(72.3)

(75.0)

(33.3)

(54.5)

(100)

Disease assessments

 FFS 1996

N/N of patients (%)

FFS = 0

16/23

7/20

17/23

(69.6)

(35.0)

(73.9)

FFS = 1

5/23

5/20

6/23

(21.7)

(25.0)

(26.1)

FFS ≥ 2

2/23

8/20

0/23

(8.7)

(40.0)

(0.0)

 FFS 2011

N/N of patients (%)

FFS = 0

18/23

6/20

18/36

10/24

(78.3)

(30.0)

(50.0)

(41.7)

FFS = 1

4/23

9/20

13/36

13/24

(17.4)

(45.0)

(36.1)

(54.2)

FFS ≥ 2

1/23

5/20

5/36

1/24

(4.3)

(25.0)

(13.9)

(4.1)

Median BVAS v3 at first visit (IQR)

12.0

17.5

17.0

15.0

8.0

(6.0)

(9.0)

(15.5)

(12.0)

(10.5)

Median VDI at first visit (IQR)

3.0

3.0

1.5

3.0

1.0

(3.0)

(3.0)

(2.8)

(3.0)

(3.0)

Treatment with DMARDs (%)

Synthetic DMARDs

 Azathioprine

16.0

0.8

70.8

68.4

41.0

39.3

22.2

30.3

 Colchicine

.

5.6

75.3

 Cyclophosphamide oral

8.3

5.3

23.1

5.6

0.8

 Cyclophosphamide IV

16.0

0.8

41.7

42.1

41.0

17.9

11.1

5.2

 Cyclosporine

5.3

2.6

16.7

12.7

 Hydroxychloroquine

4.2

5.1

16.7

2.0

 Leflunomide

5.6

 Methotrexate

64.0

36.1

33.3

10.5

43.6

17.9

16.7

10.0

 Mycophenolate mofetil

8.3

21.1

5.1

3.6

5.6

0.8

 Sulfasalazine

4.8

Biological DMARDs

 Adalimumab

2.4

 Etanercept

1.2

 Infliximab

4.0

8.3

5.6

 Mepolizumab

3.6

 Rituximab

8.3

26.3

41.0

3.6

16.7

 Tocilizumab

12.0

0.8

0.4

  1. ANCA Anti-neutrophil cytoplasmic antibody, BD Behçet’s disease, BVAS v3 Birmingham Vasculitis Activity Score version 3, c-ANCA Cytoplasmic ANCA:CV Cryoglobulinaemic vasculitis, DMARDs Disease-modifying anti-rheumatic drugs, EGPA Eosinophilic granulomatosis with polyangiitis, ENT Ear nose and throat, FFS Five Factor Score, GCA Giant cell arteritis, GPA Granulomatosis with polyangiitis, IQR Interquartile range, IV Intravenous, LVV Large vessel vasculitis, MPA Microscopic polyangiitis, MPO Myeloperoxidase, PAN Polyarteritis nodosa, p-ANCA Perinuclear ANCA, PR3 Proteinase 3, SD Standard deviation, TAK Takayasu’s arteritis, VDI Vasculitis Damage Index
  2. aIn LVV, ultrasound showing halo sign, angiography showing vascular wall thickening, enhancement, stenosis, occlusion, or aneurysms, or PET showing FDG uptake of the aorta or its major branches. In PAN, angiography showing multiple microaneurysms. In BD, angiography showing thrombosis, vascular wall thickening, enhancement, stenosis, occlusion, or aneurysms
  3. bIn LVV, presence of arteritis, often granulomatous, affecting the aorta or its major branches. In PAN, necrotizing arteritis of medium or small arteries. In AAV, necrotizing vasculitis predominantly affecting small vessels: in EGPA eosinophil-rich and necrotizing granulomatous inflammation, in GPA necrotizing granulomatous inflammation, and in MPA necrotizing inflammation with absence of granulomas. In CV, vasculitis affecting small vessels with cryoglobulin immune deposits