Canakinumab in the routinary clinical practice in cryopyrin-associated periodic syndromes (CAPS): one year of follow-up

Background

No clear information on the optimal dosage of Canakinumab in CAPS is available.

Aim

To analyse the modification of dosage schedule of Canakinumab in CAPS in 12 months of routinely clinical practice.

Methods

12 patients (9 children and 3 adults) with Muckle-Wells syndrome (3), MWS/CINCA overlap (3) and CINCA (6) were analyzed. Patients were previously enrolled in the CACZ885D2306 trial and studied for the following 12 months.

Results

At baseline, 7 patients were treated with the initial dosage of 2 mg/kg (or 150 mg, if > 40 Kg) every 8 weeks. In 5 patients (2 MWS/CINCA overlap, 5 CINCA) the dosage was 4 mg/kg (or 300 mg) every 8 weeks.

During the following 12 months modification of dosage of frequency was performed in 7/12 patients. The 5 patients at higher dosage during the CACZ885D2306 study needed to increase the frequency of administration with a mean frequency of 6 weeks (range 4-8). The mean reason was the presence of mild clinical manifestation and/or persistent elevation of acute phase reactants. In one of these patients the therapy was subsequently discontinued due to persistent disease activity. An increased frequency (6 and 7 weeks) was also performed in 1 MWS and 1 CINCA patient, respectively.

In 5 patients the treatment was not modified being effective in the control of the disease.

Conclusions

This study confirms the efficacy of Canakinumab in CAPS. However, pediatric patients and those with a more severe phenotype require higher and more frequent dosage than previously described.