Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: Countrywide data in 50 patients.

OBJECTIVE: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. METHODS: In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150-300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3-84) months. RESULTS: Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. CONCLUSION: High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab.

A study of dietary knowledge and its religious relationship in patients receiving haemodialysis.

It is widely known that haemodialysis patients do not readily comply with nutrition guidance. At least 6 months after initiating haemodialysis, the patients' dietary knowledge was tested by use of a questionnaire. The relationship between patient dietary compliance and religion and educational level was studied. Seventy patients were included in the study (33 Christians and 37 Muslims), ages ranging from 24 to 87 years. All patients had received haemodialysis for a period of the time ranging from 6 to 216 months. The questionnaire included questions related to salt, potassium and phosphate. Regarding questions, concerning salt, the Christians answered 85.7 +/- 22.8% correctly whilst the Muslims answered 60.1 +/- 34.6% correctly, a difference which was statistically significant (p=0.0001). However, more Christians arrived at the hospital with pulmonary oedema over a two-year period for emergency haemodialysis than Muslims (p=0.017), whilst there was no difference in the number of patients with residual diuresis between the two groups (p=NS). The difference in the incidence of pulmonary oedema could be attributed to the fact that more Christians had heart failure than Muslims (12/33 vs 6/37, p=0.09). Regarding questions concerning potassium, the Christians answered 74.6 +/- 33.9% correctly whilst the Muslims answered 41.1 +/- 38.5% correctly, a difference that was statistically significant (p=0.0001). However despite this difference, no Muslims attended hospital for emergency haemodialysis related to hyperkalaemia over the two-year period. Finally, regarding questions concerning phosphates, the answers from both group of patients were disappointing. Specifically, Christians answered 49.1 +/- 43% correctly, whilst Muslims answered 19.1 +/- 3.3% of the questions correctly, a difference which was statistically significant (p=0.0001). Patients did not acquire the necessary dietary knowledge but despite insufficient knowledge, they had a low incidence of fluid overload, hyperkalaemia and hyperphosphataemia. The Christians, despite superior knowledge, compared to the Muslims had more episodes of fluid overload.