Table 4.
Treatment characteristics at baseline and at the end of follow-up.
| R92Q patients with pediatric onset (n = 11) | R92Q patients with adult onset (n = 7) | All R92Q patients (n = 18) | |
|---|---|---|---|
| Initial treatment | |||
| NSAIDs | 3 (OD) | 3 (OD) | 6 (OD) |
| Colchicine | 1 (C) | 1 (C)c,d | 2 (C) |
| Glucocorticoids | 5 (OD); 2 (C)a,b | 1 (OD); 3 (C)c,d,e | 6 (OD); 5 (C) |
| Biological agents | 1 AN (C)a; 1 ET (C)b | 1 ET/AN (C)d | 1 AN (C); 1 ET (C); 1 ET/AN (C) |
| Treatment at the end of follow-up | |||
| No treatment | 2 | 3 | 5 |
| NSAIDs | 4 (OD) | 1 (OD) | 5 (OD) |
| Colchicine | 0 | 0 | 0 |
| Glucocorticoids | 3 (OD) | 1 (OD) | 4 (OD) |
| Biological agents | 1 AN (OD)a; 1 ET (C)b | 1 AN (OD)d; 1 AN (C)e | 3 AN (2 OD, 1 C); 1 ET (C) |
AN, anakinra; C, continuous; ET, etanercept; OD, on demand; NSAIDs, non-steroidal anti-inflammatory drugs.
Patients a and b initially received glucocorticoids and a biological agent; and at the end of follow-up, patient a is receiving anakinra on demand and patient b, etanercept 50 mg/week. Patient c received colchicine and glucocorticoid therapy. Patient d was treated with colchicine, prednisone, etanercept, and finally, anakinra (on an initial continuous administration, which could be switched to on demand during the disease course). Patient e started with continuous glucocorticoids and was later switched to anakinra.