Age at last follow-up visit, years | 59.3±12.8 | 56.2±12.4 |
Male sex (%) | 17 (90) | 6 (66) |
BRAF V600E mutation (%) | 19 (100) | 3 (33) |
Clinical manifestations (%) | | |
Cardiovascular | 13 (68) | 56 (67) |
Pleuropulmonary | 13 (68) | 6 (67) |
Neurological and/or orbital | 18 (95) | 9 (100) |
Retroperitoneal | 16 (84) | 6 (67) |
Skeletal | 19 (100) | 9 (100) |
Previous therapies (%) | 16 (84) | 8 (89) |
Targeted therapy | 1 (5) | 2 (22) |
Interferon-α-2a | 12 (63) | 3 (33) |
Glucocorticoid | 8 (42) | 1 (11) |
Methotrexate | 3 (16) | 1 (11) |
Tocilizumab | 1 (5) | 0 (0) |
Anakinra | 0 (0) | 3 (33) |
Infliximab | 1 (5) | 0 (0) |
Imatinib | 1 (5) | 0 (0) |
Tofacitinib | 0 (0) | 1 (11) |
Concomitant therapies (%) | 8 (42) | 5 (56) |
Glucocorticoids | 8 (42) | 1 (11) |
Anakinra | 2 (11) | 5 (56) |
Adverse reactions (%) | 11 (58) | 4 (44) |
Renal | 6 (32) | 1 (11) |
Cutaneous | 5 (26) | 3 (33) |
Systemic inflammation | 2 (11) | 1 (11) |
Cardiovascular | 3 (16) | 1 (11) |
Haematological | 0 (0) | 1 (11) |
Mucosal/gastrointestinal | 0 (0) | 4 (44) |
Hepatic | 1 (5) | 0 (0) |
Treatment discontinued (%) | 5 (26) | 3 (33) |