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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