3. RESULTADOS Y ANALISIS
3.2. EXPECTATIVAS DE LOS JUBILADOS POSTERIOR AL TALLER DE
‘A FIRST-DEGREE RELATIVE WITH PSORIASIS’AS A CRITERION FOR THE ILAR CLASSIFICATION SYSTEM FOR JUVENILE IDIOPATHIC ARTHRITIS
Roselie Achten1, Joost Swart1, Gabriella Giancane1, Tom Wolfs1, Michael Hofer1, Ekaterina Alexeeva1, Violeta Panaviene1, Susan Nielsen1, Jordi Anton1, Florence Uettwiller1, Valda Stanevica1, Maria Trachana1, Denise Pires Marafon1, Constantin Ailioaie1, Elena Tsitsami1, Sylvia Kamphuis1, Troels Herlin1, Pavla Dolezalova1, Gordana Susic1, Berit Flato1,
Flavio Sztajnbok1, Angela Pistorio1, Alberto Martini1, Nicolino Ruperto1, Nico Wulffraat1
1
PRINTO, Istituto Gaslini, Genova, Italy
Correspondence:Roselie Achten
Introduction: Literature showed the importance of classifying pa- tients into the right Juvenile Idiopathic Arthritis (JIA) category to pro- vide them with optimal treatment1. However, a substantial amount
of patients were classified as‘undifferentiated JIA’(UJIA) due to the strict exclusion criteria, harbouring the risk of losing the indication of the preferred treatment.
Objectives:The aim of this study was therefore to investigate the in- fluence of‘a first-degree relative with psoriasis’, which is an inclusion criterion for psoriatic arthritis and an exclusion criterion for the ‘remaining five JIA categories’. This results in more insight in the ac- curacy of the classification system for JIA from the International League of Associations for Rheumatology (ILAR).
Methods:Analyses were made in a retrospective and prospective co- hort of 8,309 patients with JIA. 606 patients were classified as UJIA, 303 patients as‘psoriatic arthritis’(PSA) and 7,400 patients as one of the‘remaining five categories’.
Results: 225 (37.1%) patients with UJIA had ‘a first-degree relative with psoriasis’. No significant difference was found between the PSA and UJIA category in the number of patients with‘a first-degree rela- tive with psoriasis’. None of the abovementioned 225 patients devel- oped psoriasis during the observation period. Only 60 (20.8%) patients were classified as PSA because of the inclusion criterion of having‘a first-degree relative with psoriasis’in the PSA category. Conclusion:The exclusion criterion‘a first-degree relative with psor- iasis’increases the number of patients classified as UJIA. No signifi- cant difference was found between the PSA and UJIA category in the number of patients with‘a first-degree relative with psoriasis’, sug- gesting this criterion is not of added value for the ILAR classification system for JIA. We conclude that‘a first-degree relative with psoriasis’ is not a necessary inclusion criterion for psoriatic arthritis. For these reasons, we suggest removing the inclusion and exclusion criterion‘a first-degree relative with psoriasis’for all the JIA categories.
References
1. Beukelman T, Patkar NM, Saag KG, Et Al. 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Initiation and Safety Monitoring of Therapeutic Agents for the Treatment of Arthritis and Systemic Features. Arthritis Care Res. 2011;63(4):465–82.
Disclosure of Interest None Declared
P070
THE FREQUENCY OF JUVENILE SPONDYLOARTHROPATHIES IN CHILDREN WITH FAMILLIAL MEDITERRANEAN FEVER
Emre Ozer1, Demet Seker1, Emir Taner1, Amra Adrovic1, Sezgin Sahin1, Kenan Barut1, Oya Koker1, Ozgur Kasapcopur1
1
Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey
Correspondence:Amra Adrovic
Pediatric Rheumatology2018,16(Suppl 2):P070
Introduction:Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease characterized with fever, recur- rent episodes of self-limiting polyserositis and arthritis. FMF arthritis is generally acute monoarthritis especially in the larger joints of the lower extremities, healing without a sequelae. However some of the patients develop different type of chronic arthritis, predominantly oligoarticular juvenile idiopathic arthritis (JIA) and juvenile spondyloarthropathies (JSpA). Studies on JSpA among childhood FMF patients are scarce. Objectives:To evaluate the frequency of JSpA in a large childhood FMF cohort. Furthermore, we aimed to define main characteristics of JSpA among childhood FMF patients.
Methods: A total of 320 juvenile FMF patients were blindly ques- tioned according to recently proposed criteria for JSpA by 3 re- searchers (EO, DS, ET). A standardized case report form including demographic data, clinical features, MEFV mutation and treatment was prepared and completed for each patient. Patients fulfilled the JSpA criteria were previously classified as probable JSpA. Afterwards,
an expert in pediatric rheumatology (OK) reevaluated the classified patients and some of them were confirmed to be a definite while some of them were accepted as potential JSpA patients.
Results:37 patients (11.5%) were initially classified as potential JSpA: 32 (10%) were accepted as definite and 5 (1.5%) as probable JSpA. Demographic, clinical and treatment data of definitive JSPA patients are shown in Table 1.
Conclusion: Articular involvement compatible with JSpA could be seen in childhood FMF patients. Spondyloarthropathies were detected in 10% of childhood FMF cases. The M694V mutation is the most common MEFV mutation among JSpA patients with FMF. JSpA should be considered in childhood FMF patients, especially in those chronic arthritis, axial involvement and enthesopathy.
Disclosure of Interest None Declared
P071
DIFFERENCES IN THE PERCEPTION OF DISEASE IMPACT BETWEEN US AND ITALIAN CHILDREN WITH JUVENILE IDIOPATHIC ARTHRITIS: A NETWORK ANALYSIS OF VIRTUAL FOCUS GROUPS Alessandra Alongi1, Serena Calandra1, Susan Thornhill2, Jennifer Stinson3, Stephanie Luca3, Jennifer Horonjeff4, Angelo Ravelli5, Jane E. Munro6, Esi M. Morgan7, Alessandro Consolaro5, OMERACT JIA Core Set Working group
1
UNIVERSITÀ DEGLI STUDI DI GENOVA, Genoa, Italy;2Thornhill Associates, Hermosa Beach, USA;3The Hospital for Sick Children, Toronto, Canada; 4
Columbia University Medical Center, New York;5Istituto Giannina Gaslini, Genoa, Italy;6Murdoch Children’s Research Institute, Melbourne, Australia;7Cincinnati Children’s Hospital Medical Center, Cincinnati, USA
Correspondence:Alessandra Alongi
Pediatric Rheumatology2018,16(Suppl 2):P071 Table 1 (abstract P070).See text for description
FMF + Definite JSPA FMF + Probable JSPA FMF patients without JIA and JSpA FMF + JIA (except ERA or JSpA) Patients, n 32 5 268 15 Female, n (%) 10 (31.25%) 1 (20%) 148 (55.22%) 10 (66.66%)
Age of disease onset, mean ±SD years
7.19 ± 3.68 5.60 ± 4.93 4.91 ± 3.40 4.93 ± 3.32
Age at study, mean ±SD years 14.84 ± 3.70 13.40 ± 1.67 12.51 ± 4.43 10.73 ± 3.57 M694V mutation n(%) 19 / 30 (63.33%) 3 (60%) 148 / 245 (60.40%) 11 (73.33%)
Disease onset >6 yrs 26 (81.25%) 5 (100%) 6 (40%)
Oligorthritis 21 (65.62%) 1 (20%) 14 (93.33%)
Inflammatory back pain 17 / 32 (53.1%) 3 / 5(60%) 0 (0%)
Enthesopathy 3 / 5(60%) 0 (0%) Sacroiliitis 22 /32(68.7%) 0 / 1 (0%) 0 /5 (0%) Coxofemoral Arthritis 14 /21(66.7%) 19/32(59.37%) 2 / 5(40%) 0 / 15 (0%) Tarsometatarsal sensivity 1 / 5(20%) 1 / 7(14.3%) HLA-B 27 Positivity 12 / 32 (37.5%) 0 / 1(0%) 0 / 3(0%) Male Gender 4 / 5(80%) 5 /15(33.3%) Response to NSAID 9 / 22(40.9%) 4 / 5(80%) 2 / 7(28.6%) Limitation in Schober test (< 4 cm) 22 /32(68.7%) 2 / 5(40%) 0 / 7(0) 21 / 26(80.7%)
Family history of SpA group of disease, dactylitis, psoriasis or presence of IBD
7 / 32(21.87%) 1 / 5(20%) 1 /8(12.5%)
Introduction:The OMERACT JIA Core Set Working Group formed in 2015 as an international initiative to revise the existing Core Set with relevant patient/caregiver input. In efforts to develop an updated, patient-centered Core Outcome, virtual focus groups (VFGs) to iden- tify main patient/caregivers-valued themes regarding the physical, mental, and social impact of JIA disease activity states were conducted.
Objectives:To identify and prioritize key features defining patient- perceived inactive disease state and to examine possible cross- cultural differences in the main domains
Methods:Two sets of paired VFGs were conducted with JIA patients (adolescents and young adults) and parents (split by ages of their children) in two clinics, in the US and in Italy respectively. Eligibility included prior or current experience of JIA disease inactivity (>3 months). A 3-day facilitated online board was held per group, focusing on the impact of JIA on physical, mental and social health, and the per- ceived differences between active and inactive JIA. Participants were asked to identify and rank their five top priority features defining in- active disease. Content analysis of transcripts was conducted independ- ently in the two centers and compared. Coded transcripts were further analyzed using network analysis, an approach that allows to study the structure of the connections between domains and the impact of spe- cific items, measured by centrality indexes.
Results:86 subjects were included. Caregiver were split by ages of their children (under and over 15 years old in the US sample; under and over 10 years old in the Italian sample). Patients were split in ad- olescents (15-17 years old in the US sample, 15-18 years old in the Italian sample) and young adults (18-24 years old in the US sample, 19-25 years old in the Italian sample). Qualitative analysis revealed psychosocial impact and limitations in daily activities emerged as main domains in both samples; fear of relapses and burden of medi- cations were indicated as concerns mostly by Italian patients and caregivers, while the impact on children’s activities and family life ap- peared relevant in US groups. Network models of ranked“top five” remission-defining items identified absence of pain as the single item with the highest degree, closeness and between centrality, indicating high relevance; others central themes in both samples were absence of functional limitations, improved engagement and autonomy. When comparing network models by the two populations, reduction of anxiety and absence of uveitis showed the greatest differences in centrality across the samples, respectively showing higher centrality in the Italian and in the US groups.
Conclusion:Data analysis revealed several domains that need to be evaluated for the development of patient/parent-centered outcomes assessment instruments. Network models revealed the central role of pain, functional limitation and restricted participation in patient- perceived remission in both populations. Main differences in the two populations include the role of relapses, treatments and secondary demands in the impact of disease. Further analysis and cross-cultural validation of the results is planned to inform the development of pa- tient/parent-centered outcomes measures.
Disclosure of Interest None Declared
P072
POLYARTICULAR JUVENILE IDIOPATHIC ARTHRITIS WITH RHEUMATOID FACTOR: A RETROSPECTIVE MONOCENTRIC STUDY OF 46 PATIENTS
Charlotte Boussard1, Ambre Hittinger1, Pierre Quartier-Dit-Maire1,2, Wouters Carine1, Brigitte Bader-Meunier1,2
1IMAGINE Institute and Pediatric Immunology, Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Assistance Publique Hôpitaux de Paris;2Paris-Descartes University, Paris, France
Correspondence:Charlotte Boussard
Pediatric Rheumatology2018,16(Suppl 2):P072
Introduction: Rheumatoid factor (RF) positive polyarticular juvenile idiopathic arthritis (JIA) with is a rare subtype of JIA (2-5%) of pa- tients. No series have been published on this topic.
Objectives:Our objective was to describe the demographics charac- teristics and the course of RF positive polyarticular JIA.
Methods: We performed a monocentric retrospective study of pa- tients with RF positive polyarticular JIA included between 2008 and February 2018 in the database CEMARA of the French reference cen- ter for juvenile arthritis and rare pediatric systemic autoimmune diseases (RAISE). This data base has been approved by the French Commission Nationale Informatique et Libertés. We recorded pa- tients age at onset, gender, familiy history, comorbities, growth-height charts, acute phase reactants, positivity of antinuclear antibodies (ANA) and anti-citrullinated protein antibodies (ACPA), treatments, side effects and course. Clinical remission was defined according to Wallace criteria1.
Results:Fourty-six patients (40 girls, 6 boys) were included. The me- dian age at onset was 13 years (5-23) and the median follow-up 3 years (1-15). Sixteen patients (35%) had a familial history of auto- immunity or juvenile inflammatory rheumatism. Two patients were siblings from the same non-consanguineous parents. Fourteen pa- tients (30%, 4/6 boys, 10/40 girls) had associated conditions: 4 pa- tients had auto-immune diseases (thyroiditis, vitiligo, Raynaud syndrome, insulin dependent diabetes), 3 (6,5%) had bronchiectasis, 5 (10,8%) a growth retardation. Finally two patients had a Mendelian diseases: Marfan syndrome and hereditary multiple osteochondromas resulting from heterozygote mutation of EXT1. ACPA and ANA were positive in 27/33 (82%) and 19/24 (79%) patients respectively. Twenty-one patients (45,6%) had an elevation of acute phase reac- tants at disease onset. Eight patients (17%) had erosive arthritis within the first year of evolution. The first line treatment was METHO- TREXATE for 34 patients (74%) alone in 11 patients (32%)) or in asso- ciation with anti-TNF in 23 patients (67%)). Twenty-four/27 patients (88%) were treated by anti-TNF: ETANERCEPT (74%), ADALIMUMAB (11%), and INFLIXIMAB (3.7%). Other biologic agents were RITUXIMAB (n=2), TOCILIZUMAB (n=4). ABATACEPT (n=6). Sixteen patients (35%) received short or sustained treatment with corticosteroids. Primary or secondary resistance for the first biological agent occurred in 3 (6.5%) and 4 patients (8.6%) respectively. Seventeen out of 35 pa- tients (48%) were on remission on treatment or off treatment (mean follow-up without relapse: 7 and 8 years respectively). Four patients (15%) who were treated by at least one biological agent presented side effects, which required to stop the treatment.
Conclusion:Our study confirms the severity of RF positive polyarticu- lar JIA and emphasizes its demographic and immunological heterogen- eity. This first report of bronchiectasis in this population emphasizes the need for a careful pulmonary evaluation. The frequency of family auto-immunity history and the occurrence in two siblings suggest that some FR positive polyarticular JIA might be monogenic diseases. References
1. Wallace CA, Giannini EH, Huang B, Itert L, Ruperto N, Null N, et al. American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis. Arthritis Care & Research. 1 juill 2011;63(7):929‑36.
Disclosure of Interest None Declared
P073
FREQUENCY OF THORACOLUMBAR SPINE INVOLVEMENT IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS
Selcan Demir, Fatma B. Ergen2, Hafize E. Sönmez1, Erdal Sag1, Yelda Bilginer1, Ustun Aydıngöz2, Seza Ozen1
1Department of Pediatric Rheumatology;2Department of Radiology, HACETTEPE MEDICAL FACULTY, Ankara, Turkey
Correspondence:Selcan Demir
Pediatric Rheumatology2018,16(Suppl 2):P073
Introduction:Juvenile idiopathic arthritis (JIA), is the most common chronic rheumatic disease among the world and the diagnosis is based on the exclusion of other causes in the presence of arthritis, lasting at least 6 weeks in children under 16 years of age.
Objectives:We aim to assess the frequency of thoracolumbar spine (TLS) involvement and Magnetic Resonance Imaging (MRI) findings in the patients with JIA.
Methods:Patients who underwent for thoracolumbar MRI, known or suspected JIA between January 2015–2017, were retrospect- ively re-examined by a musculoskeletal radiologist for the pres- ence of inflammatory (central/corner lesion, costovertebral inflammation, supraspinous or interspinous enthesitis) and erosive (wedging, central erosion) lesions. At the time of spinal MRI we also investigated the presence of sacroiliac joint (SIJ) involvement, peripheral joint (PJ) involvement and symptoms for spine involvement.
Results:Totally 108 patients were re-examined. Finally, 52 patients (mean age: 14.5±3.4) with a definitive diagnosis of JIA were en- rolled in the study. Among them 41 had enthesitis related arth- ritis (ERA), 4 had oligoarticular (OA), 5 had polyarticular (PA) JIA, one had psoriatic arthritis (PsA) and one had systemic JIA (sJIA). Among ERA patients there were at least one inflammatory lesion in 23 (56%), one erosive lesion in 3 (%7), and both inflammatory and erosive lesions in 3 (7%) patients. In ERA patients with posi- tive findings at TLS MRI, 19 (82%) had sacroiliitis and 14 (60%) had peripheral arthritis concurrently. 15 (65 %) were clinically asymptomatic in terms of TLS involvement (none of them had in- flammatory back pain or morning stiffness, the Schober’s test [>6 cm] and chest expansion [>10 cm] were normal). Among the pa- tients with other subtypes of JIA, there were at least one inflam- matory lesion in 5 (45%) and at least 1 erosive lesion in 1 (9%). In non-ERA patients with positive findings at TLS MRI, 6 (83%) were clinically asymptomatic for TLS involvement. Of note, one patient had sacroiliitis and 5 patients had peripheral arthritis in addition to TLS involvement.
Conclusion: Although current literature indicates that spinal MRI shows insufficient evidence for detecting early JIA; with this study we demonstrated that, Inflammatory and/or erosive lesions of thoracolumbar spine can be seen in asymptomatic patients of ERA and even in non-ERA JIA patients and the majority of spinal lesions detected in asymptomatic ERA and non-ERA JIA patients were inflammatory.
Disclosure of Interest None Declared
P074
DAILY PHYSICAL ACTIVITY AND SLEEP IN CHILDREN WITH JUVENILE IDIOPATHIC ARTHRITIS AND HEALTHY CHILDREN IN RELATION TO PAIN
Simon Esbersen1, Troels Herlin1, Birgitte T. Mahler1
1Pediatrics and Adolescents Medicine, Pediatric Rheumatology, Aarhus University Hospital, Skejby, Denmark
Correspondence:Simon Esbersen
Pediatric Rheumatology2018,16(Suppl 2):P074
Introduction: Around 30% of children with juvenile idiopathic arthritis (JIA) suffer from daily pain regardless of medication. Pain in JIA causes disturbances in the circadian rhythm compared to healthy children.
Objectives:To study if pain is related to disturbed sleep, physical ac- tivity, and higher JIA disease activity compared to healthy age- matched controls.
Methods:A prospective observational study was conducted between 2016 and 2018. We wanted to include 100 JIA patients and 200 healthy age-matched controls between 6-16 years old. JIA patients were consecutively asked during their routinely visits to our out- patient clinic. Controls were invited by notice on the parents IT- based communication platform in schools. They were asked to complete a sleeping schedule and a pain app daily for one week. One time they answered retrospective surveys about pain, sleep, and activity.
Results:93 with JIA and 188 controls were included. 76% of JIA pa- tients and 43% of controls reported pain in retrospective
questionnaires. In one week's observation, 84% with JIA and 70% of controls had pain minimum once. 32% with JIA and 6 % of controls reported daily pain. Pain intensity (VAS score 0-10) reported as me- dian [min;max] was: 1.00 [0;8.25] for JIA patients and 0.29 [0;6.29] for controls. Pain prevalence and pain intensity were significantly higher in JIA patients compared to controls (p<0.05). 55% with JIA and 90% of controls always participated in physical education, thus JIA pa- tients were significantly less active than controls. Pain did not reduce physical activity in JIA, but pain significantly lowered the level of ac- tivity in controls (p<0.001). No significantly relation between neither sleep disturbances and study groups nor sleep disturbances and pain was found. Disease activity (JADAS-27) in JIA was significantly posi- tively correlated to pain intensity, and significantly related to reduced physical activity (p<0.05).
Conclusion:Pain is common in JIA patients and controls. JIA patients are significantly less active compared to controls. Low physical activ- ity in JIA is not related to pain, but to increased disease activity. Higher pain intensity is related to higher disease activity in JIA. In controls, pain is related to reduced activity.
Disclosure of Interest None Declared
P075
MUSCULOSKELETAL CONDITIONS AMONG PERINATALLY HIV- INFECTED ADOLESCENTS IN THE CAPE TOWN ADOLESCENT ANTIRETROVIRAL COHORT
Sana Mahtab1,2, Chris Scott1, Takwanisa Machemedze1,2, Susan Joubert1,2, Nana Akua Asafu-Agyei1,2, Landon Myer3, Heather J. Zar1,2
1Department of Pediatrics & Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town;2SA MRC Unit on Child & Adolescent Health;3Epidemiology & Biostatistics, School of Public Health & Family Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa
Correspondence:Sana Mahtab
Pediatric Rheumatology2018,16(Suppl 2):P075
Introduction: Perinatally HIV-infected adolescents (PHIVA) have been shown to have a higher risk of developing musculoskeletal diseases. Increasing access to antiretroviral therapy (ART) has dra- matically improved life expectancy but little is known about mus- culoskeletal disease in PHIVA who are on ART in Africa.
Objectives:To study the prevalence and spectrum of musculoskel- etal abnormalities in PHIVA on ART using the pediatric Gait, Arms, Legs, Spine (pGALS) screening tool.
Methods: HIV-infected and matched HIV-uninfected adolescents (HIV-) enrolled in the Cape Town adolescent antiretroviral cohort (CTAAC) had pGALS examination and screening musculoskeletal questionnaires completed by trained study clinicians. Childhood Health Assessment Questionnaires (CHAQ) were used to assess participants’ musculoskeletal health status; participants with ab- normal pGALS examinations were referred to rheumatology. Results: pGALS was performed on 473 PHIVA (median age: 13.1 years; 49% female; median age at ART initiation: 4.3 years) and 101 HIV negative adolescents (median age: 12.8 years; 54% fe- male). Median duration on ART was 8.8 years (IQR 5.8-10.5) with 38% initiating ART at ≤2 years of age. Twenty-three percent of PHIVA were WHO HIV stage 4 at time of HIV diagnosis. At the time of pGALS screening, 60% were on two nucleoside reverse transcriptase inhibitors and a non-nucleoside reverse transcriptase inhibitor and 36% were on two nucleoside reverse transcriptase inhibitors and a protease inhibitor. Eighty percent of the PHIVA had viral load ≤100 copies/ml and the median CD4 count was 711cells/uL.
Of the 23 (4%) participants with abnormal pGALS screening, 21 (4.4%) were PHIVA and 2 (2%) were HIV negative adolescents,