The UKPID Registry Update 2015 Bangs C 1

The UKPID Registry : Update 2015 Bangs C 1, 2, Farrell S 1, 2, Edgar D 1, 3 , Guzman D 1, 4, Buckland M 1, 5 1 On behalf of the UKPID Registry Committee, UKPIN, 2 Immunology, Manchester Royal Infirmary, Manchester, UK, 3 Immunology, The Royal Hospitals, Belfast, N Ireland, 4 Department of Immunology, UCL Medical School, Royal Free campus, London 5 Immunology, Barts Health NHS Trust, London Background: The UKPID online registry has been in operation since 2009 and contributes to the ESID registry. It provides a secure method of data collection, both to provide statistics and as a valuable resource for research into primary immune deficiencies. To date over 4000 patients from 35 immunology centres in the UK have consented to join the registry. A further 2 centres are actively seeking R&D approval. Methods: Data is entered at baseline and as yearly updates. The new ESID design, adopted in October this year, comprises 3 datasets. Level 1: demographic data plus details of immunoglobulin replacement and transplant. Level 2 : details specific to the disease. Level 3: for dedicated studies, which may be industry funded, to address specific questions and will have a fixed time frame. Results: This year the UKPID registry has provided data for an NIHR rare diseases study into CVID and complement deficiencies and for the first stage of a potential level 3 project looking at the effectiveness of polyclonal immunoglobulin. A level 3 project on the recently identified combined immunodeficiency Activated P 13 Kinase Delta Syndrome (APDS) has recently been adopted from the ESID registry. The UKPID registry has 3, 396 “live” patients (i. e. not deceased or lost to follow up) with a majority of 65% ( 2208) in the “Predominantly Antibody Disorders” group. The breakdown for the other groups is shown in Figure 1. Of the 2, 033 patients on immunoglobulin replacement, 52% are on IV and 48% on subcutaneous therapy, which shows a gradual trend from 2010 to the subcutaneous route, see Figure 2. 56% of those on replacement are on home therapy. Complement deficiencies ( excluding HAE) 26 18 20 47 48 Combined ID 46 Phagocytic disorders 223 113 50 Other well defined PIDS Diseases of immune dysregulation Defects in Innate immunity 182 Autoinfammatory disorders 48 46 44 43 42 40 40 39 38 38 36 2010 Fig 1 : Disease categories (excluding Predominantly Antibody Deficiency) 2011 2012 2013 2014 2015 Fig 2: Percentage of patients on subcutaneous immunoglobulin by year The data shown below is for the Predominantly Antibody Group. 37% (809) have bronchiectasis, 4% (78) granulomatous disease, 6% (124) interstitial pulmonary disease and 8% (185) a neoplasm. Figure 4 shows the incidence of autoimmune conditions in this group (excluding secondary hypogammaglobulinemia). The total with some form of autoimmunity is 568 (26%). 1193 (54%) have CVID and the numbers for the other conditions are shown in Figure 3. 217 179 250 177 120 173 100 127 200 150 71 55 80 51 39 100 de f 106 S M IE IG H om a ym Th H ID ss la ed Su G Ig Fig 3: Number of patients in the Predominantly Antibody Deficiency group (excluding CVID) 97 76 72 54 20 bc ifi AD ss la nc a m Ag am ifi ss SP U U nc d la H G G ed Ab 0 2 n 60 40 50 47 31 19 0 AI HA ITP d roi thy po Hy 2 B 1 cy s riti ien fic de m R u he th Ar Vit s liti o ilig ive c Ul t era Co 36 30 ns oh Cr ro Th ia en top cy o mb se ea ac li oe dis C Fig 4: Number of patients in the Predominantly Antibody group with autoimmune conditions (excluding secondary HGG) Discussion: . Following the transfer of level 1 data to the new design registry on the 1 st October this year, the diagnosis of all entries must be validated against the ESID diagnostic criteria. All Principal Investigators (PIs) have been notified and it is intended that the validation process will be completed in the next year. Although this process is time consuming, there are real benefits in having a more robust dataset available for statistics and research and we hope all PIs will fully engage in the validation process. With thanks to contributing centres: Herriot R: Aberdeen, Longhurst H: The Royal London, Edgar D: Belfast, Huissoon A: Birmingham Heartlands, Noorani S: Birmingham City, Richter A: Queen Elizabeth Hospital Birmingham, Bernatoniene J: University Hospitals Bristol, Kelleher W, P: Brompton, Kumararatne D: Addenbrookes, Baxendale H: Papworth, Jolles S: Cardiff, Bhole M: Dudley, Marshall S: Dundee, Turner M: Edinburgh, Hayman G: Epsom & St Helier, Yong P: Frimley Park, Thomas M: Glasgow, Davies G: GOSH, Gordins P: Hull, Wood P: Leeds, Darroch J: Liverpool, Paulus S: Alderhey, Arkwright P: Manchester, Slatter M: Newcastle paediatrics, Spickett G: Newcastle adults, Goddard S: North Midlands, Mc. Dermott L: Nottingham, Patel S: Oxford, Bethune C: Plymouth, Grimbacher B: Royal Free, Alachkar H: Salford, Sargur R: Sheffield, Waruiru C: Sheffield Children’s, Williams A: Southampton, Heath P: St Georges, . Contact: UKPID Registry Co-ordinators: Catherine Bangs (Manchester Royal Infirmary): catherine. bangs@cmft. nhs. uk Stephanie Farrell (Manchester Royal Infirmary): stephanie. farrell@cmft. nhs. uk Chair UKPID Registry Committee: Dr Matthew Buckland (London): matthew. buckland@bartshealth. nhs. uk