C. Furtado

Using the Juvenile Arthritis Disease Activity Score Based on Erythrocyte Sedimentation Rate or C-Reactive Protein Level: Results From the Portuguese Register

Our aims were to evaluate the correlation between Juvenile Arthritis Disease Activity Score 27‐joint reduced count (JADAS27) with erythrocyte sedimentation rate (ESR) and JADAS27 with C‐reactive protein (CRP) scores and to test the agreement of both scores on classifying each disease activity state. We also aimed at verifying the correlation of the 2 scores across juvenile idiopathic arthritis (JIA) categories and to check the correlation between JADAS27‐ESR and clinical JADAS27 (JADAS27 without ESR).

Effectiveness and long-term retention of anti-tumour necrosis factor treatment in juvenile and adult patients with juvenile idiopathic arthritis: data from Reuma.pt

OBJECTIVES Assess the effectiveness and safety of biologic therapy as well as predictors of response at 1 year of therapy, retention rate in biologic treatment and predictors of drug discontinuation in JIA patients in the Portuguese register of rheumatic diseases. METHODS We prospectively collected patient and disease characteristics from patients with JIA who started biological therapy. Adverse events were collected during the follow-up period. Predictors of response at 1 year and drug retention rates were assessed at 4 years of treatment for the first biologic agent. RESULTS A total of 812 JIA patients [65% females, mean age at JIA onset 6.9 years (s.d. 4.7)], 227 received biologic therapy; 205 patients (90.3%) were treated with an anti-TNF as the first biologic. All the parameters used to evaluate disease activity, namely number of active joints, ESR and Childhood HAQ/HAQ, decreased significantly at 6 months and 1 year of treatment. The mean reduction in Juvenile Disease Activity Score 10 (JADAS10) after 1 year of treatment was 10.4 (s.d. 7.4). According to the definition of improvement using the JADAS10 score, 83.3% respond to biologic therapy after 1 year. Fourteen patients discontinued biologic therapies due to adverse events. Retention rates were 92.9% at 1 year, 85.5% at 2 years, 78.4% at 3 years and 68.1% at 4 years of treatment. Among all JIA subtypes, only concomitant therapy with corticosteroids was found to be univariately associated with withdrawal of biologic treatment (P = 0.016). CONCLUSION Biologic therapies seem effective and safe in patients with JIA. In addition, the retention rates for the first biologic agent are high throughout 4 years.

Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage

Objectives To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Methods Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index—articular (JADI-A) and Juvenile Arthritis Damage Index—extra-articular (JADI-E) damage index and disease activity were analysed. Results 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Stills disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Conclusions Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive of higher disability and decreased the chance of inactive disease.

Genetic Predictors of Poor Prognosis in Portuguese Patients with Juvenile Idiopathic Arthritis: Data from Reuma.pt

Introduction. This study aimed to assess the genetic determinants of poor outcome in Portuguese patients with juvenile idiopathic arthritis (JIA). Methods. Our study was conducted in Reuma.pt, the Rheumatic Diseases Portuguese Register, which includes patients with JIA. We collected prospectively patient and disease characteristics and a blood sample for DNA analysis. Poor prognosis was defined as CHAQ/HAQ >0.75 at the last visit and/or the treatment with biological therapy. A selected panel of single nucleotide polymorphisms (SNPs) associated with susceptibility was studied to verify if there was association with poor prognosis. Results. Of the 812 patients with JIA registered in Reuma.pt, 267 had a blood sample and registered information used to define “poor prognosis.” In univariate analysis, we found significant associations with poor prognosis for allele A of TNFA1P3/20 rs6920220, allele G of TRAF1/C5 rs3761847, and allele G of PTPN2 rs7234029. In multivariate models, the associations with TRAF1/C5 (1.96 [1.17–3.3]) remained significant at the 5% level, while TNFA1P3/20 and PTPN2 were no longer significant. Nevertheless, none of associations found was significant after the Bonferroni correction was applied. Conclusion. Our study does not confirm the association between a panel of selected SNP and poor prognosis in Portuguese patients with JIA.

AB1364 Adaptation and validation of the rheumatoid arthritis quality of life (RAQOL) scale for portugal

Background Rheumatoid Arthritis (RA) is a chronic inflammatory disease that has a major impact on patients’ quality of life. The Rheumatoid Arthritis Quality of Life Questionnaire (RAQoL) is a patient-centric outcome measure, specific to RA. The measure has not previously been available for use with Portuguese RA patients. Objectives To produce a Portuguese version of the RAQoL that is acceptable to Portuguese patients and demonstrates sound psychometric properties. Methods The dual panel methodology was used to translate the UK RAQoL into Portuguese. This involved conducting a bilingual panel (providing the initial translation into Portuguese) followed by a lay panel (where items are assessed for comprehension and acceptability). Cognitive debriefing interviews were conducted with Portuguese RA patients to determine the face and content validity of the translated scale. A large-scale postal validation survey was carried out to establish the psychometric properties of the Portuguese RAQoL. The measure was administered on two occasions to RA patients, alongside a comparator instrument – the Nottingham Health Profile (NHP). Internal consistency was assessed using Cronbach’s alpha coefficient. Spearman’s Rank correlation coefficient was employed to assess test-retest reliability. Convergent validity was tested by correlating RAQoL scores with those on the NHP sections. Known group validity was assessed using non-parametric tests for independent samples. This involved determining the ability of the RAQoL to distinguish between patients that differed according to their self-perceived severity of RA and general health. Results The translation panels produced a Portuguese version of the RAQoL that was easily understood and considered natural by native speakers. Interviewees considered the new language version to be relevant and appropriate. One hundred and seventy-eight RA patients (82% female) took part in the postal validation survey with a mean age of 56.6 (range 25 to 79) years. The Portuguese RAQoL demonstrated excellent internal consistency (Cronbach’s α=0.95) and test-retest reliability (r=0.92), indicating that the measure produces low levels of random measurement error. RAQoL scores correlated most strongly with scores on the NHP Physical mobility scale (r=0.77) and showed moderately strong correlations with the Emotional reactions, Pain and Energy level section scores. Non-parametric tests for independent samples demonstrated significant differences in RAQoL scores between patients who differed according to their self-perceived RA severity (p<0.001) and general health (p<0.001). Conclusions The Portuguese version of the RAQoL was found to be a comprehensive, reliable and valid questionnaire. The new language version is recommended for use in routine clinical practice and for research purposes, to assess quality of life in Portuguese RA patients. Disclosure of Interest None declared

AB0965 Single Nucleotide Polymorphisms in TNFA1P3 and PTPN2 Are Associated with a Poor Outcome in Juvenile Idiopathic Arthritis. Data From Reuma.PT

Background Genetic variants have been identified associated with the susceptibility to Juvenile idiopathic arthritis (JIA). Little is known about the genetic determinants of disease severity and long-term outcomes. Objectives This study aimed to assess the genetic determinants of poor outcome in Portuguese patients with JIA. Methods Our study relied on Reuma.pt, the Rheumatic Diseases Portuguese Register, which includes patients with JIA receiving biological therapies and synthetic DMARDs. Subject and disease characteristics of patients with the diagnosis of JIA were collected prospectively and a blood sample for DNA analysis stored. Poor prognosis was defined as CHAQ/HAQ >0.75 at the last visit and/or the need for biological therapy. A previously selected panel of 31 single nucleotide polymorphisms (SNPs) was studied to verify if there was any association with poor prognosis. Results 291 patients out of the 812 JIA patients registered in Reuma.pt, had a blood sample to perform the genetic analysis. From those, 267 had CHAQ/HAQ registered. The mean age was 19.9±11.3 years, 65% females. The mean age at JIA onset was 6.9±4.7 years. Of the 267 patients analyzed, 85 match our criteria of poor prognosis. In univariate analysis, including all disease categories of JIA, we found significant associations with poor prognosis for allele A of TNFA1P3/20 rs6920220, allele G of TRAF1/C5 rs3761847 and allele G of PTPN2 rs7234029. In multivariate models, the associations for TNFA1P3/20 rs6920220 (odds ratio =1.88 [95% CI 1.13-3.14]) and PTPN2 rs7234029 (OR 1.75 [1.03-3.00]) remained significant at the 5% level, while TRAF1/C5 rs3761847 (OR 1.53 [0.95-2.47]) was no longer significant. Conclusions TNFA1P3/A20 and PTPN2 were associated with poor prognosis in Portuguese patients with JIA. These genes were also associated with susceptibility to JIA. Further studies in independent populations are needed to strengthen the knowledge in this field. Disclosure of Interest None declared

AB0413 Hepatitis B Serologic Profile and Reactivation in Rheumatic Patients Treated with Biological Therapies – Single Centre Experience

Background Biologic therapy for rheumatic diseases has been associated with increased risk of latent infections reactivation such as tuberculosis or hepatitis B (HB), due to severe immunosuppression. However, the actual risk of HB reactivation is still unclear regarding the several biologics available, especially in low-incidence countries such as Portugal. Objectives To evaluate the serologic HB profile of biologic-treated rheumatic patients in a single centre and assess the incidence of HB reactivation. Methods We retrospectively collected electronically available HB serology of rheumatic patients that ever started biological therapy at our department and we reviewed the clinical course to identify reactivation cases, defined as raising viral load and transaminases. Results We included 288 patients with available electronic data on HB serologies. Mean age was 43.9±15.7 years (3.1 to 82.3 years), disease duration was 10.9±9.5 years, 63.9% of patients were female and the most common rheumatic disease was rheumatoid arthritis (112, 38.9%), followed by ankylosing spondylitis (76, 26.4%) and psoriatic arthritis (54, 18.8%). As first biologic, 254 patients (88.2%) started anti-TNF agents, 14 (4.9%) rituximab, 11 (3.8%) tocilizumab and 9 patients (3.1%) another biologic. 30 patients (10.4%) eventually stopped biologic treatment. 185 patients (64.2%) were treated with concomitant methotrexate (mean dosage 15.7±5.4mg), 81 (28.1%) with other DMARDs and 169 (58.7%) were treated with corticosteroids (58.7%). All of the 288 patients were HBsAg negative and 23 were anti-HBc positive (9%). The 23 anti-HBc positive patients did not differ significantly from the overall population in terms of age, gender distribution, disease duration, follow-up time, biologic discontinuation or biologic started. No patient received prophylactic antiviral therapy and there were no cases of reactivation or isolated rise in viral load during a cumulative biologic exposure of 1005.6 patient-years (Fig. 1). Figure 1. Hepatits B serologic pattern of rheumatic patients starting biological therapy Conclusions In our cohort, there were no cases of HB reactivation on the 288 patients treated with biologic therapy. Anti-HBc positivity was infrequent, viral load was undetectable in all cases and no chronic HB cases were detected. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4716

PReS-FINAL-2095: Older age predicts poor response to 6-months methotrexate therapy in a juvenile idiopathic arthritis cohort of patients

The identification of predictive factors of poor response to methotrexate (MTX) in juvenile idiopathic arthritis (JIA) patients could contribute to optimize the treatment strategy, namely by the earlier introduction of biological treatments.

PReS-FINAL-2021: JADAS-CRP instead of JADAS-ESR...results from REUMA.PT

Recently, Juvenile Arthritis Disease Activity Score (JADAS) was found to be a valid instrument for assessment of disease activity. JADAS was developed with erythrocyte sedimentation rate (ESR) because C-reactive protein (CRP) values were not available in all databases used to validate the tool. Nordal et al compared recently in a Nordic population the JADAS based on CRP with JADAS based on ESR and concluded that these instruments correlated closely, indicating that both scores can be recommended for assessing disease activity in JIA.