Table 2 Treatment with canakinumab in CAPS patients
Patients | Phenotype | NLRP3 gene mutation | Concomitant treatments | Canakinumab dosing | Canakinumab duration (months) | Canakinumab at last follow-up | Disease activity at last follow-up | Concomitant treatments at last follow-up | Remaining symptoms | Remaining abnormal laboratory findings | Infectious adverse events | Other non-infections Adverse events | |||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
skin and subcutaneous tissue disorders | gastrointestinal disorders | blood and lymphatic system disorders | liver enzyme elevations | ||||||||||||
1 | FCAS | T348M | No | 4 mg/kg q8w | 3 | ongoing | Remission | No | No | No | No | No | No | No | No |
2 | MWS | Y443H | prednison (0.47 mg/kg.d) | 9 mg/kg q4w → 8 mg/kg q4w | 12 | ongoing | Minimal | No | Occasional rash | No | No | dry skin | diarrhea | No | No |
3 | MWS | D305N | No | 4 mg/kg q8w | 27 | ongoing | Minimal | No | No | CRP↑ESR↑ | No | No | No | No | No |
4 | MWS | T348M | No | 4 mg/kg q8w | 3 | ongoing | Remission | No | No | No | No | No | No | No | No |
5 | CINCA | L266P | prednison (1.18 mg/kg.d) | 2 mg/kg q4w → 8 mg/kg q4w | 38 | ongoing | Minimal | prednison (0.14 mg/kg.d) | Occasional rash and fever | CRP↑ | viral upper respirtory tract infectiom | No | diarrhea | No | No |
6 | CINCA | I336V | prednison (0.42 mg/kg.d) | 8 mg/kg q4w | 11 | ongoing | Minimal | No | Occasional fever | No | No | No | No | No | No |
7 | CINCA | K570N | prednison (0.48 mg/kg.d) | 4 mg/kg q8w → 6 mg/kg q8w | 27 | ongoing | Remission | No | No | No | gastroenteritis, bronchitis | rash | No | thrombopenia | No |
8 | CINCA | C631F | prednison (0.51 mg/kg.d) | 8 mg/kg q8w | 18 | ongoing | Remission | No | No | No | viral upper respirtory tract infectiom | No | No | No | No |
9 | CINCA | I482F | prednison (0.71 mg/kg.d) | 2 mg/kg q8w → 4 mg/kg q8w | 29 | ongoing | Remission | No | No | No | bronchitis | rash | No | No | No |
10 | CINCA | V404L | No | 4 mg/kg q4w → 4 mg/kg q8w | 29 | ongoing | Remission | No | No | No | No | No | No | No | No |