Pediatric Rheumatology 2017, 15(Suppl 1):37 Page 56 of 259

Introduction: Since 2013 PRES has initiated an European project called P71
SHARE (Single Hub and Access point for Paediatric Rheumatology in Young people’s beliefs about research priorities in rheumatology
Europe, PI Wulffraat). The project aimed to identify the specific needs research
for optimal care in pediatric rheumatic diseases (PRD) in each European Suzanne Parsons1, Janet McDonagh2, Wendy Thomson2
1
country, and provide these countries with recommendations for the Public Programmes Team, Central Manchester University Hospitals’ NHS
care of these group of children. Optimal care includes diagnosis, man- Foundation Trust, Manchester, UK; 2Centre for Musculoskeletal Research,
agement of disease and providing both drug and non-drug therapies. University of Manchester, Manchester, United Kingdom
The European countries were grouped regarding geographic and eco- Presenting author: Suzanne Parsons
nomic characteristics into West and East-Europe, in order to analyze if Pediatric Rheumatology 2017, 15(Suppl 1):P71
there are important differences in health care of these regions. Israel
was the first non-European country to participate of the SHARE survey. Introduction: Involving people of all ages in health-related research is
Objectives: To show an updated data about families view on stand- now widely advocated. However, research priorities are still driven pri-
ard of care for children with Juvenile Idiopathic Arthritis (JIA) in marily by professional agendas, with evidence from the adult literature
Europe and Israel. reporting a mismatch between researcher and patient generated lists
Methods: In order to identify the specific needs for the optimal care of research topics. To date, there have been no studies exploring the
in PRD, patients and doctors surveys have been performed at research priorities of young people with rheumatic disease.
pediatric rheumatology centers and patient organisations involved in Objectives: The aim of this study was to explore young people’s re-
providing care for these children. Families of patients with diagnosis search priorities for rheumatic conditions to feed into the develop-
of JIA according ILAR (International League of Associations for ment of the research strategy for the Barbara Ansell National
Rheumatology) criteria answered a single time-point survey that en- Network for Adolescent Rheumatology (BANNAR).
compasses information about specialists referral patterns and referral Methods: Focus groups were undertaken with young people aged
delays, disease diagnosis, multidisciplinary health care team follow 11-24 years across the UK and Northern Ireland. Young people were
up, access to treatment, participation in clinical research and transi- recruited via members of the BANNAR and relevant national charities.
tion of care to the adult service. Families filled in the questionnaires Focus group participants discussed their understanding and beliefs
anonymously on PRINTO (Pediatric Rheumatology INternational Trials about what should be researched in the following areas; Basic Sci-
Organisation) website. ence; Clinical Medicine and Science; Health Services, Psychosocial,
Results: Until May 2016, 508 families from 22 different countries an- and Public Health. Participants were asked to order these areas in
swered the questionnaires: 350 families from West-Europe, 129 from terms of which should receive the most funding. All focus groups
East-Europe and 29 from Israel. The table shows the participant coun- were audiotaped and transcribed for thematic analysis.
tries and the number of answered surveys of each country. The large Results: 12 focus groups (11-15 years (n = 5) and 16-24 years (n = 7))
difference in the number of answered questionnaires between West have been held with 58 participants. Young people’s research prior-
and East-Europe is a reflex of their population, as West is three times ities were influenced by whether they felt that investment in a par-
more populous than East. The families that replied this survey may ticular area was likely to: 1. Achieve benefits for all patients, 2.
present a bias to urban population, where the pediatric rheumatol- Achieve long term or short term goals (finding a cure versus improv-
ogy centers are. Patients characteristics regarding gender, age of ing quality of life). Other influences on participants’ priorities were
diagnosis and JIA subtypes are similar in East and West-Europe, as their beliefs about whether research areas are already well funded
well as in Israel. Families view showed good access to pediatric rheu- and whether increasing funding could lead to faster results.
matologists, uveitis screening and high cost medications in all these Basic Science was considered to be a key priority. Participants were
regions. The main differences were found in pain and fatigue man- especially interested in understanding the genetic basis of their con-
agement, information about transition for the adults service, use of dition and how this could affect their prognosis and treatment side
supportive care and knowledge about patient organizations. It seems effects. However, there was an appreciation that progress in this area
that these items were less emphasized in East-Europe. was unlikely to happen overnight, and that for this reason a steady
Conclusion: The families view is extremely important to identify the stream of funding was likely to be important.
topics on standard of care that need improvement, in order to Participants also felt that psychosocial research currently did not receive
achieve a high quality health care in Europe. A substantial number of considerable investment. However, there were varying views on whether
replies (508) have been obtained from 22 different countries. The re- it should. Those who felt that the psychosocial aspects of their condition
sults showed good access to pediatric rheumatology care in Europe had been neglected tended to feel that Psychosocial research should be
and Israel, although tools like patient organizations and transition a priority. However, a number expressed uncertainty as to who could
care were less used by East-Europe. For the future, the aim of SHARE provide this support believing their doctors were already overstretched.
is not only to expand the families survey to a larger number of Euro- Finally, some participants expressed a wish not to focus too much on
pean countries, but also to another continents. the psychosocial aspects of their condition as part of their overall coping
Disclosure of Interest strategies. Health Services Research was considered by many to be a
None Declared low priority, as for the majority of participants, consultant care was their
preferred option. GPs were considered not to be a viable support option.
However, participants were interested in transitional care arrangements
Table 27 (abstract P70). See text for description between paediatric and adult care.
For many, the development of new treatments was a low priority, be-
West-Europe East-Europe Asia
cause they were currently happy with their treatment regime, or be-
Netherlands 100 Germany 28 Latvia 21 Israel 29 cause they felt uncomfortable with the risk of trying an experimental
Belgium 16 Austria 1 Lithuania 2 treatment.
Finally, for all groups Public Health was ranked low. There was a
Denmark 34 Switzerland 1 Czech Republic 20 need identified to provide education to a range of audiences about
Sweden 1 Italy 26 Serbia 5 their conditions, e.g. GPs, schools, workplaces, but scepticism existed
as to whether or not such educational materials would be utilised.
United Kingdom 33 Portugal 5 Slovakia 33 Conclusion: Understanding the research priorities of young people
Ireland 26 Spain 20 Slovenia 17 with rheumatic conditions is important to ensure that research strat-
egies are developed that are in tune with their needs and wants. The
France 59 Albania 1
findings from this work will feed into the research strategy of the
Greece 30 BANNAR.
Disclosure of Interest
TOTAL = 350 TOTAL = 129 TOTAL = 29
None Declared
Pediatric Rheumatology 2017, 15(Suppl 1):37 Page 57 of 259

P72 P73
Development of the first video game aimed at children suffering JIA patients have a similar quality of life as healthy peers after
from Juvenile Idiopathic Arthritis three years of specialised care
Jean-David Cohen1, Damien Bentayou2, Marc-Antoine Bernard Brunel2, Jens Klotsche1, Miriam Listing1, Martina Niewerth1, Gerd Horneff2,
Sonia Trope3 Angelika Thon3, Hans-Iko Huppertz4, Kirsten Mönkemöller5, Ivan
1
Public Hospital, Montpellier, France; 2CG Artists, Bordeaux, France; Foeldvari6, Dirk Föll7, Kirsten Minden1, and ICON study group
3 1
ANDAR patient organization, Paris, France Epidemiology unit, German Rheumatism Research Centre, Berlin,
Presenting author: Jean-David Cohen Germany; 2Kinderklinik, Asklepios, Sankt Augustin, Germany; 3Kinderklinik,
Pediatric Rheumatology 2017, 15(Suppl 1):P72 Medizinischen Hochschule Hannover, Hannover, Germany; 4Prof.-Hess-
Kinderklinik, Klinikum Bremen-Mitte, Bremen, Germany; 5Klinik für Kinder-
und Jugendmedizin, Kinderkrankenhauses der Stadt Köln, Köln,
Introduction: In France, Juvenile Idiopathic Arthritis (JIA) affects be- Germany; 6Hamburger Zentrum für Kinder- und Jugendrheumatologie,
tween 2 000 and 4 000 children. The physical and psychological im- am Klinikum Eilbek, Hamburg, Germany; 7Klinik für pädiatrische
pact of this chronic condition can be significant, including difficulties Rheumatologie und Immunologie, Universitätsklinikum Münster,
in the family environment, as well as at school. Whilst the arrival of Münster, Germany
biotherapies has revolutionised the care of these children, educa- Presenting author: Kirsten Minden
tional support remains necessary to increase their independence and Pediatric Rheumatology 2017, 15(Suppl 1):P73
improve their quality of life.
Objectives: The development of e-health, in the context of the dem- Introduction: The treatment of patients with juvenile idiopathic arth-
ocratisation of communication tools, has led us to create a Serious ritis (JIA) currently aims at achieving an inactive disease state and
Game as educational support for children. the best possible quality of life. Whether these treatment goals are
Methods: The game has been developed following the ADDIE peda- achievable in a routine clinical setting can be assessed by data from
gogical engineering model. The game will be translatable in all lan- the Inception Cohort Of Newly-diagnosed patients with JIA (ICON).
guages and made available to foreign associations free of charge. A Herein, patients with recent-onset of JIA have been prospectively
steering committee was formed, composed of the parents associ- observed.
ation that initiated the project, a paediatric rheumatologist, and a Objectives: To study changes in disease activity state and quality of
video game agency. life of patients during and after the first three years of paediatric
The National Association for the Defence against rheumatoid arthritis rheumatology care.
manages the logistics and monitoring. The rheumatologist creates Methods: Data of 597 patients with JIA and 306 healthy peers who
the scientific context, in collaboration with the association. The de- were included in ICON and followed prospectively for at least three
velopers make artistic and gaming suggestions. The committee sets years were considered for this analysis. JIA disease activity was
the direction: format, themes, key messages, graphics, animations assessed by the cJADAS-10 (clinical Juvenile Arthritis Disease Activity
and game sequences. Learning is validated through multiple choice Score). Inactive disease was defined as cJADAS-10 ≤ 1. The PedsQL
quizzes. The association KOURIR (association for parents of children 4.0 (Pediatric Quality of Life Inventory) Generic Core Scales were ap-
with JIA) will test the beta version. Adaptations will then be made plied to measure health-related quality of life (HRQoL) in patients
before the game’s release. and controls and the PedsQL 3.0 Rheumatology module to assess
Results: The committee decided to create an adventure game disease-specific QoL.
with interactive activities. The following options were selected: Results: Patients (69% females) and controls (61% females) had a
The game is developed using the Unity® engine for computers, mean age of 9.8 and 10.1 years at the 3-year-follow-up (3-y-FU).
smartphones and tablets, allowing for use anywhere (Apple, An- Approximately half of the patients (48%) had oligoarthritis, 27%
droid). The engine is designed to support all languages. The rheumatoid factor (RF) negative polyarthritis. During the 3rd year
topics of the hospital process, treatment, complimentary examina- of observation, 56% of the patients were on MTX, 26% on bio-
tions and rehabilitation are all discussed with corresponding key logics and 10% had been treated with systemic glucocorticoids.
messages. Patients’ disease activity significantly decreased from a mean cJA-
Many themes are discussed in a single location, the hospital. Two DAS of 9.5 at first assessment to 2.6 at the 3-y-FU. A total of 271
avatars (a boy, a girl) have been created in order to facilitate (45%) patients had an inactive disease, ranging from 47% for per-
identification for young players. Skill games and 16 multiple sistent oligoarthritis and 56% for RF negative polyarthritis to 79%
choice quizzes (used to assess knowledge and deliver peda- for systemic JIA. Another 10% reached a minimal active disease
gogical messages) are spread throughout the game. The test at FU. Eighty percent of the patients had received DMARDs up to
phase brought together 7 children of various ages (5 to 16) and the 3-y-FU. At the 3-y-FU, 27% did not receive any anti-rheumatic
parents members of the KOURIR association and led to some ad- drug. However, only 8% were in remission off drug, another 37%
justments: enhanced interactivity in the rehabilitation part, short- had attained remission on drug.
ening and improved ergonomics of the swimming-pool game, At inclusion in ICON, patients had a significantly lower HRQoL than
and simplification of some of the vocabulary. controls (mean total scale score 71.3 (SD 18.4) versus 90 (SD 70.5),
Conclusion: To the best of our knowledge, this is the first serious proxy reports). After three years of rheumatology care, the patients’
game dedicated to JIA. Other locations (home, school) could HRQoL had clinically meaningful improved and did no longer differ
complete this version. An English version is already under develop- significantly in any health domain from that of controls (total score
ment. The warm reception it has received from a number of paediat- 86.8 (SD 14.5) versus 89.5 (SD 8.6)). Patients with inactive disease had
ric rheumatologists means we are hopeful many children will be able a higher HRQoL of 92.8 (SD 8.8) at 3-y-FU. Fourteen percent of pa-
to benefit from this initiative, at home or in a structured educational tients still had high active disease (c-Jadas-10 > 4/8.5 for oligoarthri-
context. tis/polyarthritis) and a significantly lower mean PedsQL score of 74.0
Disclosure of Interest (SD 17.8). The patients’ disease-specific QoL also significantly im-
None Declared proved from ICON inclusion to the 3-y-FU.
Pediatric Rheumatology 2017, 15(Suppl 1):37 Page 58 of 259

Conclusion: Current treatment options in paediatric rheumatology opposed to controls whose BMI increased after a similar follow-up
care allow achieving an inactive or minimal active disease state and period (0.23 ± 0.34 vs. 0.45 ± 0.51, p < 0.0001).
a normalisation of HRQoL in the majority of patients. Conclusion: Long-term biologic treatments are associated with a sig-
Trial registration identifying number: ICON is supported by a grant nificant increase of height SDS and decrease of BMI SDS in JIA patients.
from the Federal Ministry of Research and Education (FKZ: 01ER0812) Disclosure of Interest
Disclosure of Interest None Declared
J. Klotsche: None Declared, M. Listing: None Declared, M. Niewerth:
None Declared, G. Horneff Grant / Research Support from: Pfizer,
Abbvie, Roche, A. Thon: None Declared, H.-I. Huppertz: None De-
clared, K. Mönkemöller: None Declared, I. Foeldvari: None Declared, P75
D. Föll: None Declared, K. Minden Grant / Research Support from: Pfi- Response to conventional DMARD treatment in a cohort of
zer, Abbvie children with Juvenile Idiopathic Arthritis in Bogota, Colombia
Adriana S. Díaz-Maldonado1, Sally Pino2, Pilar Guarnizo3
1
Pediatric Rheumatology, Care for Kids - Hospital La Misericordia -
P74 Instituto Roosevelt, Bogotá, Colombia; 2Pediatric Rheumatology, Care for
Auxological features in patients with Juvenile Idiopathic Arthritis Kids - Hospital Infantil de San José, Bogotá, Colombia; 3Pediatric
(JIA) treated with biologic drugs Rheumatology, Care for Kids - Fundación Cardio Infantil - CAYRE, Bogotá,
Achille Marino1, Stefano Stagi2, Niccolò Carli1, Federico Bertini1, Teresa Colombia
Giani1, Gabriele Simonini1, Rolando Cimaz1 Presenting author: Adriana S. Díaz-Maldonado
1
Department of Paediatrics, Rheumatology Unit, Anna Meyer Children’s Pediatric Rheumatology 2017, 15(Suppl 1):P75
Hospital, University of Florence, Florence, Italy; 2Department of
Paediatrics, Endocrinology Unit, Anna Meyer Children’s Hospital, Introduction: Different treatment options are available for juvenile
University of Florence, Florence, Italy idiopathic arthritis (JIA) increasing with the time. There are multiple
Presenting author: Achille Marino factors, such as JIA subtype that impact in the management of medi-
Pediatric Rheumatology 2017, 15(Suppl 1):P74 cation and the escalation of the therapy (1).
Objectives: To describe the treatment with conventional and bio-
Introduction: Juvenile idiopathic arthritis (JIA) is an heterogeneous logical DMARDs and their response on a cohort of children with JIA
group of diseases associated with an increase of inflammatory cyto- in 6 health care institutions in Bogotá, Colombia.
kines that may influence child growth. Methods: Medical records were retrospectively reviewed derived
Objectives: To evaluate the auxological features in a cohort of pa- from 568 patients diagnosed with JIA and attended in 6 health care
tients with JIA treated with biologic drugs. institutions in Bogotá, Colombia since 2010. Demographic and clin-
Methods: This single-center retrospective study evaluated 86 chil- ical characteristics were collected as well as the current and previ-
dren with JIA (7 systemic, 32 oligoarticular, 31 polyarticular, 6 psori- ously suspended treatments due to no response. The no response
atic, 10 enthesitis-related arthritis) on long-term (mean 38.5 months; was considered when a patient persists with clinical and laboratories
range 6-134) treatment with biologics, and compared them (in a 1:2 criteria after 6 months of treatment.
ratio) with 172 age and sex-matched healthy controls for height and The description of the current and previous treatments of patients
body mass index (BMI). Patients with endocrinological or other with JIA was performed according to JIA subtype and Disease-
chronic comorbid conditions or on long-term steroid (>3 months) modifying antirheumatic drugs (DMARDs). A descriptive analysis was
treatment were excluded. performed of different treatments and those who required to sus-
Results: All patients received anti-TNFα treatment except 4 patients pend any biological (bDMARDs) or conventional DMARDs
with systemic JIA; 21 patients received > 1 biologic (2 biologics in 15 (cDMARDs).
patients, 3 biologics in 4 patients and 4 biologics in 2 patients); 13 Results: 471 patients (83%) received immunosuppressive therapy
patients received CTLA-4Ig; one patient received anti-IL6; anti-IL 1 with DMARDs and 97 (17%) with NSAID or prednisolone. Of these
was used in 5 patients. 43% (244) have been received bDMARD.
The target height SDS was not statistically different (0.02 ± 0.66 vs. Among those treated with DMARD, 51% patients not responding to
0.05 ± 0.79 SDS) between JIA patients and controls. The median cDMARDs and need bDMARDs. TNF inhibitors are the most frequent
height SDS at JIA diagnosis was -0.29 ± 0.66 vs. 0.09 ± 0.84 SDS of (72,2%) followed by interleukin - 6 receptor antagonist (IL-6 RA)
controls (p < 0.005) (-0.24 ± 0.66 SDS for oligoarticular (p < 0.05), (16,8%), co-stimulary (4%), IL-1RA (0,4%) and anti CD20 monoclonal
-0,17 ± 0.79 SDS for polyarticular (p = NS), -1.33 ± 0.49 SDS for sys- antibodies (5,2%).
temic (p < 0.001), -0.71 ± 1.13 SDS for psoriatic (p < 0.05), and 0.30 ± 42% of patients with Polyarticular JIA rheumatoid factor (RF) posi-
0.11 SDS for enthesitis-related arthritis (p = NS)). tive, Polyarticular JIA rheumatoid factor (RF) negative, psoriatic
During disease modifying antirheumatic drugs (DMARDs) treatment, arthritis and systemic arthritis have been treated with any bio-
before biologic therapy beginning, the height was: in all JIA patients logics. Additionally, the 50% of patients with enthesitis related
-0.31 ± 0.66 SDS vs. 0.14 ± 0.86 in controls (p < 0.0001); -0,39 ± 0.93 arthritis (ERA) HLA-27B positive have been treated with biological
SDS (p < 0.005) for oligoarticular and -0.23 ± 0.79 SDS (p < 0.05) for therapy.
polyarticular, -1,00 ± 0.13 SDS (p < 0.005) for systemic, -0.65 ± 0.61 Regarding to previous biological therapy, 73,4% had received one
SDS for psoriatic (p < 0.05), and 0.10 ± 0.64 SDS for enthesitis-related biologic, 22,2% two biologics, 2% three biologics and 2% four bio-
arthritis (p = NS). logics. All patients mainly belong to polyarthritis and systemic
After a follow-up of 6.4 ± 2.3 yrs height SDS in all JIA patients was arthritis.
-0.16 ± 0.89 vs. 0.08 ± 0.81 SDS in controls (p < 0.05). Results were In current treatment, 30,7% of the patients use bDMARDs in
-0,08 ± 0.12, SDS (p = NS) for oligoarticular, -0,13 ± 0.89 SDS (p = NS) monotherapy while the other 69.3% use bDMARDs in combin-
for polyarticular, -0,58 ± 0.14 SDS (p < 0.05) for systemic, -0.58 ± 0.33 ation with one cDMARDs. Conventional DMARDs are used in
SDS for psoriatic (p < 0.05), and 0.01 ± 0.60 SDS for enthesitis-related 58,99% of patients with JIA. Methotrexate is used in 88,9% pa-
arthritis (P = NS). tients with cDMARDs, followed by sulfasalazine (6,2%) and leflu-
The Δheight during biologic treatment was statistically significant nomide(4,9%). Combination of two cDMARDs is used in 2,77% of
considering all JIA patients, oligoarticular, systemic and psoriatic (p patients with cDMARDs.
<0.0001) but not polyarticular and enthesitis-related arthritis. Interest- Ninety one patients achieved remission: 77 patients (84,6%) achieved
ingly, JIA patients showed a significative reduction of BMI SDS after remission off medications, which 28 patients presented oligoarticular
biologic treatment (all patients -0.19 ± 0.47 at diagnosis vs. 0.07 ± JIA (36,3%), 21 polyarticular JIA (27,2%) and 20 systemic arthritis
0.31 at last follow-up visit, p < 0.005; systemic -0.11 ± 0.31 vs. 0.40 ± (25,9%). The highest percentages of remission was oligoarthritis. 13
0.49, p < 0.05; polyarticular 0.13 ± 0.30 vs. -0.06 ± 0.32, p < 0.05), as patients (14,3%) are in remission on medications.
Pediatric Rheumatology 2017, 15(Suppl 1):37 Page 59 of 259

Thirty four patients dropout the treatment where administrative bar- Table 28 (abstract P76). Initial clinical and laboratory findings in
riers were the mainly cause. Children with oligoarticular and polyarti- patients with leukemic arthritis vs JIA
cular JIA were the most frequent in drop outing. Leukemic arthritis JIA (N = 25) P value RR (IC 95%)
Conclusion: Conventional DMARDs are the most frequent drugs used (n = 9)
in the treatment of JIA represented mainly by methotrexate. In the Pain intensity (0–10) 10 6.6 (4–10) *0.0001
case of bDMARDs, the anti TNF inhibitors are the principal drugs. The
bDMARDs showed a high frequency of use in positive HLA–B27 ERA Night pain 66% 4% *0.0001 7.7 (2.5–23)
patients compared to other subtypes. Polyartticular JIA patients pre- Fever 66% 12% *0.001 5 (1.7–17.6)
sented the most frequent changes of bDMARDs compared to other
Weight loss 78% 16% *0.001 7.3 (1.8–29.6)
JIA subtype
Disclosure of Interest Lymphadenopathy 88% 4% *0.0001 22 (3.2–153.7)
None Declared Anemia 56% 8% *0.002 4.8 (1.7–13.3)
Hemoglobin g/dL
10.9 (8.3–12.9) 13.1 (10.2–16.4)
P76 Leukopenia 67% 0% *0.0001 9.3 (3–27)
Discrimination between Juvenile Idiopathic Arthritis and leukemic Leukocytes mm3
3555 (1610–5530) 9750 (5190–24630)
arthritis
Alfonso Ragnar Torres-Jimenez1, Berenice Sanchez-Jara2, Eunice Solis- Neutropenia 88% 0% *0.0001 25 (3.3–170)
Vallejo1, Adriana Ivonne Cespedes-Cruz1, Maritza Zeferino-Cruz1, Julia Neutrophil mm3
473 (0–1820) 6456 (2270–22130)
Veronica Ramirez-Miramontes1
1 Thrombocytopenia 44% 0% *0.0001 6 (2.6–13)
Reumatologia Pediatrica, Imss Hospital General Centro Medico Nacional
Platelets ×103/mm3
La Raza, Azcapotzalco, Mexico; 2Hematologia Pediatrica, Imss Hospital 171 (53–296) 375 (207–661)
General Centro Medico Nacional La Raza, Azcapotzalco, Mexico
Pediatric Rheumatology 2017, 15(Suppl 1):P76

Introduction: P77
Acute lymphoblastic leukemia may occur with arthritis before the ap- Rheumatoid factor positive polyarticular JIA with pulmonary langerhans
pearance of blasts in peripheral blood and can be confused with ju- cell histiocytosis- a chance association or a causal relationship?
venile idiopathic arthritis. In this study, we identified the clinical and Ankur Kumar1, Anju Gupta1, Deepti Suri1, Amit Rawat1, Nandita Kakkar2,
laboratory differences between the two entities. Surjit Singh1
1
Objectives: To determine the clinical and laboratory differences be- Pediatrics, PGIMER, Chandigarh, India; 2Histopathology, PGIMER,
tween leukemic arthritis and juvenile idiopathic arthritis at onset of Chandigarh, India
symptoms Presenting author: Ankur Kumar
Methods: Pediatric Rheumatology 2017, 15(Suppl 1):P77
Patients under 16 years old, both genders, initially presenting to the
Pediatric Rheumatology unit, General Hospital, National Medical Center Introduction: Langerhans cell histiocytosis is a multisystem disease of
La Raza, with diagnosis of probable JIA, without blast cells in peripheral an unknown etiology characterized by infiltration and accumulation of
blood, where the final diagnosis was acute lymphoblastic leukemia or CD1a+/ CD 207+ histiocytes in various organ systems. Association of LCH
juvenile idiopathic arthritis. The clinical and laboratory manifestations with autoimmune or inflammatory disorders has rarely been reported.
were evaluated. Data were expressed as percentages for each analysis Objectives: To describe the case of a young girl with juvenile idio-
area. Chi square and Relative Risk (qualitative variables) and Mann- pathic arthritis and pulmonary LCH and to discuss the possible com-
Whitney and T test for comparison of means between groups The data mon pathogenetic mechanism for both diseases.
were analyzed by software using SPSS version 15.0. Methods: A 10 year old girl was symptomatic for past 1 year. Her ill-
Results: We analyzed data on 34 patients, 9 with leukemic arthritis and ness started with left ankle swelling with pain, redness, restriction of
25 with JIA. The mean age at diagnosis was significantly lower in the movement and early morning stiffness. Over the next 5 months she
group of leukemic arthritis 7.56 vs 10.76 years (p = 0.045), female gen- had progressive involvement of right ankle joint, small joints of bilateral
der prevailed in both groups (66% and 76%, respectively, p = 0.586), foot and bilateral wrist joints. On examination, she had swelling in bilat-
time of onset of symptoms it was lower in the group of leukemic arth- eral ankle and wrist joints. There was redness and increased
ritis. Pain intensity, night sweats, weight loss, fever, lymphadenopathy, temperature over the surface associated with synovial thickening and
hepatosplenomegaly and night pain was higher in the group of restriction of passive range of movement. Her investigations revealed
leukemic arthritis. Remission of pain with analgesics was significantly (Table 29) thrombocytosis; elevated ESR and C- reactive protein; posi-
lower in patients with leukemic arthritis As for laboratory parameters, tive antinuclear antibody (ANA) and positive rheumatoid factor (RF).
rheumatoid factor positivity was higher in the group of AIJ without Chest X-ray revealed cystic changes in bilateral lung fields which were
showing statistical significance. The hemoglobin level, the leukocyte confirmed on CT chest which showed multiple well defined cysts with
and neutrophil count was lower in patients with leukemic arthritis, the variable sizes distributed throughout the parenchyma in bilateral lung
lymphocyte count was similar in both groups. The platelet count was fields. An open lung biopsy was performed to confirm the diagnosis.
higher in JIA patients, the VSG was similar in both, the difference be- Histopathology examination of the resected lung tissue revealed lym-
tween the value of the PCR was not the value of the DHL was higher in phomononuclear inflammatory infiltrate in the interstitium with collec-
the group of leukemic arthritis without showing statistical significance. tion of large atypical cells around the peribronchiolar region which
Conclusion: Leukemia is an important diferential diagnosis in chil- were showing oval to round nuclei and moderate to abundant vacuo-
dren presenting with arthritis. lated cytoplasm, these cells were positive for CD1a. Skeletal x-ray re-
In this study it was found that the presence of pain disproportionate to vealed no lytic lesions and bone marrow examination was also normal.
the degree of arthritis, fever, night pain, which does not yield to the There was no evidence of any other organ involvement with LCH.
use of analgesics, weight loss and lymphadenopathy, help to discrimin- Results:
ate clinically between leukemic arthritis and juvenile idiopathic arthritis. For arthritis, she was initiated on oral naproxen, a short course of oral
Useful laboratory parameters are leukopenia, neutropenia, and prednisolone and subcutaneous methotrexate; which led to marked
anemia, the mean platelet count was in low normal value, but lower improvement in her joint symptoms. For management of LCH, as she
than in juvenile idiopathic arthritis. The laboratory parameter with was asymptomatic with predominantly cystic lung changes and no
most higher relative risk was neutropenia. nodular changes on CT chest; hence a possibility of chronic fibrotic
Disclosure of Interest disease was considered and a decision not to give any specific ther-
None Declared apy for LCH was taken
Pediatric Rheumatology 2017, 15(Suppl 1):37 Page 60 of 259

Conclusion: Our case highlights a rare and previously unrecognized Conclusion: This is the first study to investigate the prevalence of over-
association of juvenile idiopathic arthritis with pulmonary LCH. It also weight in children and adolescents with JIA at the time of diagnosis with-
supports the inflammatory origin of Langerhans cells out any treatment. We could not find any difference between the
Disclosure of Interest overweight and obesity rates of patients with JIA and children from the
None Declared general population. This study revealed that 17.3% of JIA patients had in-
creased weight. A similar overweight/obese rate of 16-21% for children
Table 29 (abstract P77). Investigations in the index case and adolescents in the general population in Turkey was found previously.
In a recent study of Schenck et al, systemic JIA and ERA patients showed
Investigation Result
significantly higher overweight rates compared to other subgroups. We
Haemoglobin (gm/L) 119 also found a significantly higher rate of overweight in ERA when compared
WBC counts (×109 cells/L) 11 to other subgroups. However; when it was adjusted for age and gender,
Differential counts N63/L23/M7/E7
increased BMI was not significantly associated with any certain subgroup.
Disclosure of Interest
Platelet counts (×109 /L) 467 None Declared
ESR (mm/hr) 49

CRP (mg/dL) 18
Table 30 (abstract P78). See text for description
AST (U/L) 49
Healthy weight Increased weight
ALT (U/L) 30
Persistent oligoarthritis 95 (90.5) 10 (9.5)
ALP (U/L) 190
ERA 55 (68) 26 (32)
Anti nuclear antibody 3+ homogenous
Poliarthritis RF (+) 46 (78) 13 (22)
Rheumatoid factor (U/ml)
Systemic 28 (90.5) 1 (9.5)
At diagnosis 181 (normal range: 0–19)
Undifferentiated 17 (81) 4 (19)
4 months later 199
Psoriatic 16 (100) 0 (0)
Extended oligoarthritis 8 (89) 1 (11)
Poliarthritis RF (-) 4 (80) 1 (20)
P78
Data were presented as n (% within subgroup)
The rate of obesity and overweight among Turkish children with
Juvenile Idiopathic Arthritis
Balahan Makay, Özge A. Gücenmez, Erbil Ünsal
Pediatric Rheumatology, Dokuz Eylül University Hospital, İzmir, Turkey P79
Presenting author: Balahan Makay Patient partnership and disabkids in the Swedish Pediatric
Pediatric Rheumatology 2017, 15(Suppl 1):P78 Rheumatology Registry
Bo Magnusson1, Karina Mördrup1, Anna Vermé1, Christina Peterson2, and
Introduction: The increasing rate of overweight among children and Board of the Swedish Pediatric Rheumatology Registry
1
adolescents is a serious public health concern. Children with physical Paediatric Rheumatology Unit, Astrid Lindgren Children’s Hospital,
disabilities may have an increased risk for overweight and overweight Karolinska University hospital, Stockholm, Sweden; 2Hälsohögskolan,
may be a risk factor for inflammatory arthritis, as it is in adults with Jönköping, Sweden
psoriatic and rheumatoid arthritis. However; there are only a few stud- Presenting author: Bo Magnusson
ies about obesity and juvenile idiopathic arthritis (JIA) in the literature. Pediatric Rheumatology 2017, 15(Suppl 1):P79
Objectives: To investigate the rate of being overweight/obese among
Turkish children with JIA at the time of diagnosis and to assess whether Introduction: The healthcare of today is facing higher demands on
increased BMI is associated with different subgroups of JIA. coproduction of it’s service with partnership between patients and
Methods: The hospital charts of 325 patients diagnosed as JIA accord- healthcare providers. Good outcomes, most recognise, are more
ing to the International League of Association of Rheumatology (ILAR) likely if the clinician and patient communicate effectively. Real time
criteria in a 10-year period in a tertiary hospital were retrospectively patient feedback facilitated by Patient Reported Measures (PRMs) can
reviewed. Demographic and clinical findings and JIA subgroups were be used to enable true co-production of care. DISABKIDS is a Euro-
recorded. Center for Disease Control BMI-for-age percentile charts were pean project that has developed with focus on health related quality
used. Patients were classified as having “increased weight” (BMI ≥ 85th of life (HRQoL). The Swedish Pedatric Rheumatology Registry started
percentile), “healthy weight” (5th ≤ BMI < 85th percentile) or “under- in 2009. Today over 2700 patients with JIA are included. The cover-
weight” (BMI < 5th percentile) according to their BMI at baseline. age is high. 65% of all Swedish patients with JIA (0-17 years of age)
Results: The mean age at diagnosis was 9.1 ± 4.9 years. There were 182 are included and above 90% of all patients with JIA on biological
girls and 143 boys. The median delay time to dignosis was 5 months treatment. The registry is a tool for both patients and care providers
(interquartile range 2-12 months). 105 patients (32.3%) had persistent oli- in promoting valuabel care in many ways. National and local data are
goarthritis, 81 (24.9%) had enthesitis-related arthritis (ERA), 59 (18.2%) had provided promoting good and equal care for all patients. DISABKIDS
RF (-) poliarthritis, 29 (8.9%) had systemic arthritis, 21 (6.5%) had undiffer- is selected in the registry as instrument for HRQoL.
entiated arthritis, 16 (4.9%) had psoriatic arthritis, 9 (2.8%) had extended Objectives: To describe the Swedish experience of the process of co-
oligoarthritis and 5 (1.5%) had RF (+) poliarthritis. 244 patients (75%) had production with patients, families and patient representativs. Evaluate
healthy weight, 56 (17.3%) had increased weight and 25 (7.7%) were and describe the differences in the assessments of DISABKIDS as
underweight. The 46.4 percent of the patients with increased weight HRQoL and how to use the data to reveal individual problems and con-
belonged to ERA subgroup. The rates of increased BMI according to JIA nect to the planning of further care in partnership with the patient.
subgroups were given in Table 30. In univariate analyses; male gender, Methods: During the project, an improvement-coach led group semi-
age at diagnosis and ERA subgroup were found to be significantly associ- nars and the discussions which constituted a base for prioritized
ated with increased BMI (p = 0.013, p < 0.001, and p < 0.001, respectively). areas. The sessions resulted in two defined improvement areas.
However; multivariate backward stepwise regression analyses revealed Results:
that only age at diagnosis was significantly associated with increased BMI. To develop a visualization platform for patient reported data as well
Increased BMI was not significantly associated with diagnostic delay time, as other biomedical aspects. Further, to describe the needs in care
number of involved joints and erythrocyte sedimentation rate. for children and adolescents with pediatric rheumatic diseases.