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 |