The UKPID Registry : Update 2015
Bangs C1,2, Farrell S1,2, Edgar D1,3 , Guzman D1,4, Buckland M1,5
1On
behalf of the UKPID Registry Committee, UKPIN, 2Immunology, Manchester Royal Infirmary,
Manchester, UK, 3Immunology, The Royal Hospitals, Belfast, N Ireland, 4Department of Immunology,
UCL Medical School, Royal Free campus, London 5Immunology, 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 P13 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 Figure1.
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)
48
Combined ID
46
26 18 20
47
Phagocytic disorders
223
50
Other well defined PIDS
113
Diseases of immune
dysregulation
48
46
44
43
42
40
40
Defects in Innate immunity
182
39
38
38
Autoinfammatory disorders
36
2010
2011
Fig 1 : Disease categories (excluding
Predominantly Antibody Deficiency)
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.
250
200
120
217
179
177
173
100
127
80
150
100
71
55
51
39
60
50
40
0
20
106
97
76
72
54
47
19
31
30
36
0
Fig 3: Number of patients in the
Predominantly Antibody Deficiency group
(excluding CVID)
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 1st 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, McDermott 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