The UKPID Registry : Update 2015 Bangs C1,2,

The UKPID Registry : Update 2015 Bangs C1,2,

The UKPID Registry : Update 2015
Bangs C1,2, Farrell S1,2, Edgar D1,3 , Guzman D1,4, Buckland M1,5
On 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
1

Background:
Background: The
The UKPID
UKPID online
online registry
registry has
has been
been in
in operation
operation since
since 2009
2009 and
and contributes
contributes to
to the
the ESID
ESID registry.
registry. ItIt provides
provides aa secure
secure

method
method of
of data
data collection,
collection, both
both to
to provide
provide statistics
statistics and
and as
as aa valuable
valuable resource
resource for
for research
research into
into primary
primary immune
immune deficiencies.
deficiencies. To
To date
date over
over
4000
4000 patients
patients from
from 35
35 immunology
immunology centres
centres in
in the
the UK
UK have
have consented
consented to
to join
join the
the registry.
registry. A
A further
further 22 centres
centres are
are actively
actively seeking
seeking R&D
R&D
approval.
approval.

Methods:
Methods: Data
Data is
is entered
entered at
at baseline
baseline and
and as
as yearly
yearly updates.
updates. The
The new
new ESID
ESID design,
design, adopted
adopted in
in October
October this
this year,
year, comprises
comprises 33 datasets.
datasets.
Level
Level 1:
1: demographic
demographic data
data plus
plus details
details of
of immunoglobulin
immunoglobulin replacement
replacement and
and transplant.
transplant. Level
Level 22 :: details
details specific
specific to
to the
the disease.
disease. Level
Level 3:
3: for
for
dedicated
dedicated studies,
studies, which
which may
may be
be industry
industry funded,
funded, to
to address
address specific
specific questions
questions and
and will
will have
have aa fixed
fixed time
time frame.
frame.

Results:
Results: This
This year
year the
the UKPID
UKPID registry
registry has
has provided
provided data
data for
for an
an NIHR
NIHR rare
rare diseases
diseases study
study into
into CVID
CVID and
and complement
complement deficiencies
deficiencies and
and for
for

the
the first
first stage
stage of
of aa potential
potential level
level 33 project
project looking
looking at
at the
the effectiveness
effectiveness of
of polyclonal
polyclonal immunoglobulin.
immunoglobulin. A
A level
level 33 project
project on
on the
the recently
recently identified
identified
combined
combined immunodeficiency
immunodeficiency Activated
Activated P13
P13 Kinase
Kinase Delta
Delta Syndrome
Syndrome (APDS)
(APDS) has
has recently
recently been
been adopted
adopted from
from the
the ESID
ESID registry.
registry.
The
The UKPID
UKPID registry
registry has
has 3,396
3,396 live
live patients
patients (i.e.
(i.e. not
not deceased
deceased or
or lost
lost to
to follow
follow up)
up) with
with aa majority
majority of
of 65%
65% (( 2208)
2208) in
in the
the Predominantly
Predominantly
Antibody
Antibody Disorders
Disorders group.
group. The
The breakdown
breakdown for
for the
the other
other groups
groups is
is shown
shown in
in Figure1.
Figure1.
Of
Of the
the 2,033
2,033 patients
patients on
on immunoglobulin
immunoglobulin replacement,
replacement, 52%
52% are
are on
on IV
IV and
and 48%
48% on
on subcutaneous
subcutaneous therapy,
therapy, which
which shows
shows aa gradual
gradual trend
trend from
from
2010
2010 to
to the
the subcutaneous
subcutaneous route,
route, see
see Figure
Figure 2.
2. 56%
56% of
of those
those on
on replacement
replacement are
are on
on home
home therapy.
therapy.

Fig 1 : Disease categories (excluding
Predominantly Antibody Deficiency)

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.

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 Childrens, Williams A: Southampton, Heath P: St Georges,.

Contact:
Contact: UKPID
UKPID Registry
Registry Co-ordinators:
Co-ordinators: Catherine
Catherine Bangs
Bangs (Manchester
(Manchester Royal
Royal Infirmary):
Infirmary): [email protected]
[email protected]
Stephanie
Stephanie Farrell
Farrell (Manchester
(Manchester Royal
Royal Infirmary):
Infirmary): [email protected]
[email protected]
Chair
Chair UKPID
UKPID Registry
Registry Committee:
Committee: Dr
Dr Matthew
Matthew Buckland
Buckland (London):
(London): [email protected]
[email protected]

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