OP0260 VACCINATION SAFETY AND COVERAGE IN AN ITALIAN COHORT OF AUTOINFLAMMATORY DISEASES

Abstract

Background Vaccine-preventable diseases are again emerging in our population after anti-vaccine campaign has started. In autoinflammatory diseases (AID), vaccine triggered-disease is a well known phenomenon for Hyper-IgD/Mevalonate-Kinase Deficiency (MKD). In CAPS, severe flares have been experienced after pneumococcus vaccine, while PFAPA patients did not achieve sufficient and protective levels of antibodies. This evidence has raised doubts in physicians and families about the safety of vaccines. Objectives To evaluate, in a cohort of italian AID, the vaccination coverage of the Italian Vaccination Schedule and the prevalence of adverse reactions and disease flares induced by vaccinations. Methods An anamnestic questionnaire was applied to AID patients refering to the AID Unit of the Istituto Giannina Gaslini from August 2017 to August 2018. Acquired data were revised for quality of information. Data about disease triggers in AID were obtained from the EUROFEVER registry for statistical reference. Results Triggers in AID Eurofever Registry: In august 2018 a total of 3783 patients were enrolled in the EUROFEVER registry (1908 female, 50,43%). The mean age of symptoms at disease onset was 7.04 +/- 9,48 SD yrs, (minimal 0 - maximum 75,92 yrs). The distribution among the periodic hereditary fevers was: 28,75% FMF (n=1081); 17,66% PFAPA (n=666); 9% Undefined inflammatory disease (UND n = 347); 7,85% CAPS (n=297); 7,16% TRAPS (n=271) and 5,39% MKD (n=204). Vaccines triggered the disease in 70% of the MKD, while PFAPA, TRAPS and UND had a rate of reactions of 20%. This was also found in 12.34% of CAPS, whereas FMF and inflammatory bone disorders had a rate of 6% and 3%, respectively. Excluding other causes of reactions, and isolating just vaccines as a cause, MKD had a higher percentage of reactions (7,14%), while PFAPA and UND had 1% and CAPS, TRAPS, FMF and inflammatory bone disorders had less than 1%. Triggers in IGG cohort: 150 questionnaires were distributed with 70% rate of response. Quality of data was 100% for coverage and adverse reactions. 105 patients were identified: PFAPA (n=26); CAPS (n=5); TRAPS (n=6); FMF (n=14); MKD (n=8); Inflammatory Bone Disorders (CRMO and PAPA, n=4) and UND (n=41). Rate of coverage was lower than 90% for Hib3 (83,11%), MMR/MMRV (88,9%) and for Rota C (1,85%). For DTP3, Hep3, PCV3 and IPV the rate of coverage was higher than 90% for all vaccines. 11 moderate/severe reactions were observed as following: 5 after DTPA+IPV (1 PFAPA; 2 TRAPS, 1 MKD and 1 UND); 1 after Hib (PFAPA); 1 after P10/13 (PFAPA); 4 after MPR (1 PFAPA, 1 TRAPS, 1 MKD and 1 UND). The general rate of severe reactions/shot was 6,36 for 1000 shots and no severe infection, death, persistent or significant disability or life-threatening condition was observed. Just one MKD patient had a severe disease flare requiring hospitalization following pneumococcal vaccine. Conclusion Data show that in AID patients vaccines may more frequently trigger the disease. Therefore, vaccination in AID may be cosidered a peculiar public health problem. Specific recommendations for vaccination in AID are warranted as well as further investigations for immunologic protection.