FRI0585 Frequent, unusually severe, long lasting, local and systemic pneumococcal vaccine reactions in patients with cryopyrin-associated periodic syndromes (CAPS): results of a prospective registry based study

Background Pneumococcal, tetanus and influenza vaccinations are recommended for patients with Cryopyrin-Associated Periodic Syndromes (CAPS) when treated with immunosuppressive medication. Objectives This study aims to report the safety of pneumococcal and other vaccinations administered to CAPS patients. Methods All CAPS patients followed in the β-CONFIDENT (Clinical Outcomes and Safety Registry study of Ilaris® patients) registry between 07/2010 and 12/2015 were analysed if they had received a vaccination. The β-CONFIDENT registry is a global, long-term, prospective, observational registry, capturing and monitoring CAPS patients treated with canakinumab [1]. Results 68 CAPS patients (56% female, age range 3.5–73 years) had received a total of 159 vaccine injections, 107 injections against influenza, 19 pneumococcal vaccinations (15 pneumococcal polysaccharide vaccines [PPV], 2 pneumococcal conjugate vaccines [PCV], 2 unknown), 12 against tetanus/diphtheria antigens and 21 other vaccinations. A reaction was observed in 22 vaccine injections (14%) administered to 18 patients. 13 vaccine reactions (68% of all pneumococcal vaccine injections) occurred in 12 patients receiving pneumococcal vaccines. 12 PPV injections (80% of all PPV injections) elicited a vaccine reaction while none was elicited by the PCV injections The high frequency of pneumococcal vaccine reactions contrasted with that of the reactions to other vaccine types: only 17% and 7% of the tetanus/diphtheria and influenza vaccinations, respectively, elicited a vaccine reaction. The odds ratios to react to the pneumococcal vaccines compared to influenza and tetanus/diphtheria vaccines were 31.0 (95% confidence interval (CI) 8–119) and 10.8 (95% CI 2–74). Vaccine reactions after pneumococcal vaccinations were more severe and lasted significantly longer compared to the reactions after other vaccinations. Fever was elicited by almost half of all PPV injections. All symptoms after pneumococcal vaccination were observed very rapidly, usually within hours. Unlike the symptoms observed after influenza and tetanus/diphtheria vaccination, which resolved rapidly, the symptoms related to PPV were much more prolonged and in some cases lasted more than 3 weeks. In 2 patients, pneumococcal vaccination also elicited symptoms consistent with systemic inflammation due to CAPS reactivation. All 5 vaccine related serious adverse events were associated with pneumococcal vaccination. Conclusions Pneumococcal vaccines, unlike other vaccines, frequently trigger severe local and systemic inflammation in CAPS patients. Clinicians must balance potential benefits of pneumococcal immunisation against safety concerns. References Kuemmerle-Deschner JB, et al. Safety and Efficacy of Canakinumab in Patients with CAPS: Interim Results from the Beta-Confident Registry [abstract]. Arthritis Rheumatol. 2015;67(suppl 10). Acknowledgements The β-CONFIDENT registry was supported by Novartis Pharma AG, Basel, Switzerland. Disclosure of Interest V. Jaeger: None declared, H. Hoffmann Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis., T. van der Poll Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis., H. Tilson Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis., J. Seibert Employee of: Novartis Pharma AG, Basel, Switzerland, A. Speziale Employee of: Novartis Pharma AG, Basel, Switzerland, G. Junge Employee of: Novartis Pharma AG, Basel, Switzerland, K. Franke Employee of: QuintilesIMS who served as the CRO that conducted the β-CONFIDENT registry., E. Vritzali Employee of: Novartis Pharma AG, Basel, Switzerland, P. Hawkins Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis., J. Kuemmerle-Deschner Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis., U. Walker Consultant for: the steering committee of the β-CONFIDENT registry and in this function received consulting fees from Novartis.