Table 4.
Treatment | |
Corticosteroids (oral and/or i.v.) | 83/86 (99) |
Orala | 69/83 (83) |
i.v. pulse therapy | 2/83 (2) |
Oral and i.v. pulse | 12/83 (14) |
CYC (oral or i.v.) | 24 (29) |
Oral | 14 (16) |
i.v. | 9 (10) |
Oral and i.v. | 1 (1) |
Other DMARDs | 46 (55) |
HCQ 7 patients | |
MTX 26 patients | |
AZA 12 patients | |
MMF 3 patients | |
Minocycline 1 patient | |
Biologic response modifiers | 21/74b (28) |
Anti-TNF agents 12 patients | |
Rituximab 6 patients | |
Abatacept 1 patient | |
Anakinra 2 patients | |
Outcomes | |
Follow-up, median (IQR), months | 16 (2.4–59) |
Response to treatment (6 months after treatment initiation) | |
Complete response | 33 (38) |
Partial response | 45 (52) |
No response | 8 (10) |
Relapse at 5 years (similar or different clinical presentation) | 36% |
Mortality rate (at 5 years) | 26% |
Values are n (%) unless indicated otherwise. aThe median starting dose of oral steroid was a prednisone equivalent of 40 mg (IQR 27.5–60). bData missing on 12 patients.