S. Fernandes

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

OBJECTIVESnAssess 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.nnnMETHODSnWe 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.nnnRESULTSnA 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).nnnCONCLUSIONnBiologic therapies seem effective and safe in patients with JIA. In addition, the retention rates for the first biologic agent are high throughout 4 years.

AB0297 Real-Life Effectiveness of Golimumab in Biologic-Naïve Rheumatoid Arthritis Patients – Data from reuma.pt, A Portuguese Registry

Background Registries are becoming an increasingly important source of data, providing additional information on the use of biologics in clinical practice. The real-world clinical data currently available regarding the use of SC anti-TNFs is still limited. Therefore, it is of utmost importance to increase the knowledge of Golimumab (GLM) effectiveness in the clinical practice. Objectives This study was designed to access the effectiveness of SC GLM 50 mg/monthly + MTX through 52 weeks of treatment in biologic-naïve RA patients. The primary objective was to investigate the proportion of patients achieving clinical remission (DAS28ESR<2.6). The secondary objectives were the evaluation of: the treatment persistence rates; the proportion of patients achieving functional response (ΔHAQ>0.22); and the effect of treatment on DAS28 individual components. Methods This was a retrospective non-interventional study based on the Rheumatic Diseases Portuguese Register (Reuma.pt). It was conducted in a cohort of patients aged >18 years with active RA despite previous treatment with conventional DMARDs, biologic-naïve, who started SC GLM+MTX, from March 2011 to August 2015. The cumulative incidence of achieving clinical remission, treatment persistence and functional response/remission were estimated using survival analysis. Cox regression was used to calculate the hazard ratios. Results A total of 109 patients (86.3% female, mean age 55.5±13.2 years; mean age of diagnosis 45.5±13.5 years, rheumatoid factor 78% positive) met the study criteria. Ninety-three had a follow up of at least 52 weeks (i.e. all patients who started treatment before August 2014). At week 52, 38.3% of patients were on clinical remission, 91.9% achieved functional response and 35.2% were on functional remission (HAQ<0.5). The treatment persistence rate was 75.3% for the individuals who were in the study for ≥52 weeks (Figure 1). For functional remission, high CRP levels at baseline seem to be a determining factor (HR=0.54, p=0.026). Conclusions This is the first Golimumab data analysis generated from the Portuguese registry Reuma.pt. Our results are in agreement with data from other national registries and demonstrate the long-term effectiveness and the high treatment persistence rates of GLM through 52 weeks. Disclosure of Interest A. F. Mourão Consultant for: Merck Sharp & Dohme, C. Ribeiro: None declared, J. Borges: None declared, M. J. Gonçalves: None declared, M. Bernardes: None declared, S. Fernandes: None declared, R. Dezerto Employee of: Merck Sharp & Dohme, P. A. Laires Employee of: Merck Sharp & Dohme, P. Machado Employee of: Merck Sharp & Dohme, M. Eusébio: None declared, M. J. Santos: None declared, H. Canhão: None declared

Juvenile Systemic Sclerosis: Review of 17 Portuguese Patients

Introduction Juvenile systemic sclerosis (jSSc) represents about 10% of all systemic sclerosis patients. We aim to describe the clinical characteristics and disease progression of children with jSSc followed in Portuguese pediatric rheumatology centers. Methods Clinical and laboratory features as well as medication and outcomes of jSSc children were reviewed. Results Seventeen patients were included in the analysis, 5 of whom had overlap syndromes. Thirteen girls, with a mean age at diagnosis of 10.6 ± 3.9 years and mean disease duration of 10.5 ± 3.9 years, of these 12 had diffuse cutaneous scleroderma. In 94% cases, the first symptom was Raynauds phenomenon (RP), followed by arthritis and/or puffy hands (59%). Pulmonary involvement was documented in 7 patients at disease diagnosis, despite the paucity of respiratory complaints. Thirteen patients presented periungual capillaropathy. During follow-up, RP and skin thickening were the most frequent clinical manifestations (100%), followed by arthralgia (94%) and arthritis (76%). Pulmonary, as well as gastrointestinal involvement was documented in eight patients. Sixteen children were antinuclear (ANA) positive, eight tested positive for anti-Scl70, and one for anti-fibrillarin antibodies. Immunosuppressants (94%), proton pump inhibitors (76%) and glucocorticoids (65%) were the most common therapeutic options. One child needed autologous bone marrow transplant due to severe refractory disease. An improvement of skin thickening and stabilization of pulmonary involvement was documented in most cases. No deaths were registered in this cohort. Conclusions Raynauds phenomenon as well as capillaroscopic abnormalities were almost universal at disease presentation. Internal organ involvement was common and occurred early during disease course, although asymptomatic in several cases.

AB1011 Vitamin D Status – A Transversal Evaluation in Rheumatic Patients

Background Vitamin D deficiency is a condition reported both in young adults and in elderly and institutionalized patients. It has been increasingly recognized and, although conflicting data subsists, hypovitaminosis D has been related to a variety of rheumatic conditions from osteoporosis (OP) to inflammatory diseases and generalized pain syndromes. Objectives To assess vitamin D status in a rheumatic outpatient setting. Methods Observational, transversal, retrospective study encompassing rheumatic outpatients with at least one 25-hidroxyvitamin D determination since 2014. Data ascertained included gender, age, parathyroid hormone (PTH) and vitamin D levels, rheumatic conditions (<3/patient), co morbidities and therapeutics. Bone mineral density was assessed by densitometry (DXA) and results classified as normal (Tscore >-1), osteopenia (-2.5<Tscore<-1) and osteoporosis (Tscore<-2.5). Statistics: Mann-Whitney, Kruskal-Wallis, ANOVA and Chi-Squared tests and Pearsons correlation; p<0.05. Software: SPSS 17. Results 370 patients included, 87.3% female, mean age 64.9±13.9 years (y), 151 patients <65y. Most prevalent rheumatic conditions: osteoarthritis (OA; 47.8%), OP (43.5% - 33.5% of which with reported fractures), fibromyalgia (FM; 14.6%) and rheumatoid arthritis (RA; 13.2%); 43.9% patients had inflammatory diseases. Most common co morbidities: arterial hypertension (38.6%), dyslipidemia (32.4%) and depression (22.9%). 38.4% were on synthetic Disease Modifying Anti-Rheumatic Drugs (DMARD); 34.1% on corticosteroids (CTC), 49.2% on bisphosphonates (BF) and 69.5% on calcium/vitamin D (Ca/D) supplements. As for patients with available DXA, 41.4% were classified as having osteopenia and 43.6% as having osteoporosis. Mean vitamin D was 29.7±24.5μg/L; 65.4% patients had vitamin D levels <30μg/L. Mean PTH was 56.6±31.3ng/L and alkaline phosphatase (ALP) was 73.8±31.8U/L. In the group >65y, mean PTH was higher than in the <65y group (61.3±33.6 vs 49.7±26.1, p=0.015); 70.9% of the patients <65y and 61.6% of the patients >65y had vitamin D<30 – not significant. We compared vitamin D in OP vs OP with fractures vs OA vs associated OP/OA and found lower vitamin D levels in patients with isolated OA (38.7±32.5 vs 38.1±33.9 vs 25.9±23.3 vs 30.6±20.7, p=0.012); 76.6% with isolated OA had vitamin D<30. 55.7% of the patients on BF >5y, 61.2% on BF <5y and 72.3% without BF had vitamin D<30 (p=0.013). 68.7% in the group with inflammatory conditions and 62.9% in the group with non-inflammatory conditions had vitamin D <30 – not significant. We further compared vitamin D levels in OA vs AR, AR vs FM and OA vs FM, DMARD and CTC use and Ca/D supplementation and found no difference. There was a positive correlation between age and vitamin D (r=0.111, p=0.033), age and PTH (r=0.208, p=0.010) and vitamin D and ALP levels (r=0.311, p<0.0001). Conclusions In this study, vitamin D levels <30μg/L were found in nearly 2/3 of patients, across disease groups. There was a tendency for higher vitamin D levels in elderly patients, in the group with OP comparing with OA and in the group on BF, which may be related to previous supplementation. A potential bias is the fact rheumatologists request vitamin D dosing in patients with risk factors/clinical features of hypovitaminosis. Disclosure of Interest None declared

THU0536 Remission and Re-Treatment of Patients with Paget's Disease of Bone Treated with Zolendronic Acid – A Single Center 10 Year Experience

Background Treatment of Pagets Disease of Bone (PDB) has been revolutionized by the use of zolendronic acid (ZA). Patients usually have a dramatic response to treatment with normalization serum alkaline phosphataise (ALP) levels and a longer period of clinical remission, compared with other class agents. Data from long-term use are scarse. Objectives Evaluate the effectiveness and safety of ZA in PDB patients, as well as remission, re-treatment rates and side effects in our outpatient population since 2005. Methods A retrospective study of PDB patients treated with 5 mg ZA intravenous infusion at our day-care center. Follow up time, demographic and clinical characteristics, previous therapeutic agents, rate of response, number and reasons of re-treatment(s) and rates of adverse events were collected. A descriptive statistic analysis was made. Results 48 patients, 60% female, mean age of 75 years, with a median time since the diagnosis of 12.3 years. The disease was poliostotic in 73% of the patients and pelvis (65%), skull (29%) and spine (27%) were the most common pagetic localizations. Deafness was present in 12.5% and 65% had hip involvement. 44% patients had been treated with another biphosphonate agent previously. Response rates were 97.9% at 1 year, 87.2% after 2 years and 95.1% after 3 years. The mean ALP levels before ZA infusion was 290 UI/L and after 112 UI/L. Sixteen patients needed a re-treatment in the period of follow up, minimum of 1 year after the ZA infusion and maximum of 8 years after. 56.3% due to raised of ALP levels and 43.8% due pain/ hip involvement. Four patients needed a third infusion due to hip involvement, and 2 of them a forth infusion due to the same reason. All of the patients re-treated due to hip involvement had severe hip involvement at time of diagnosis. In our population, 2 patients achieved 10 years remission, 5 patients 9 years remission and 10 patients 8 years remission with a single ZA infusion. Recording adverse effects were: 14.6% Flu like symptoms (7 patients), 2% assintomatic hypocalcemia (1 patient) and no reports of osteonecrosis or fractures. All of these effects were reported after the first ZA infusion. Conclusions In our population, we find high long-term sustained remission rate. Only sixteen patients needed re-treatment. Patients maintained sustained remission up to 10 years of a single ZA infusion. Incidence of adverse events was similar to the reported in the literature. References Reid IR, Miller P, Lyles K et al. Comparison of a Single Infusion of Zolendronic Acid with Risendronate for Pagets Disease. N Eng J Med. 2005 Set:353(9):898–908 Reid IR, Brown JP, Levitt N et al. Re-treatment of relapse Pagets disease of bone with zolendronic acid: results from an open-label study. Natur BoneKEy Report 2. 2013 Nov: 442: 1–3 Reid IR, Lyles K, Brown JP et al. A Single Infusion of Zolendronic Acid Produces Sustained Remissions in Paget Disease: Data from 6.5 years, JBMR. 2011 Sep 26 (9):2261–70 Devogelaer JP, Geusen P, Daci E et al. Remission over 3 years in patients with Paget disease of bone treated with a single intravenous infusion of 5 mg zolendronic acid. Calcif Tissue Int. 2014 Mar:94(3):311–8 Disclosure of Interest None declared

Juvenile idiopathic arthritis: the transition to adulthood

Juvenile Idiopathic Arthritis (JIA) is term used to classify a group of heterogeneous pediatric rheumatic diseases. Many of these conditions remain active until adulthood and when patients start to be followed by adult Rheumatologists there may arise some classification problems once AIA (Adult Idiopatic Arthritis) does not exist! Many published papers regarding the transition of JIA into adulthood miss this point.

Juvenile systemic sclerosis: review of 15 patients

Introduction: Systemic sclerosis, a rare disease in childhood, is characterized by skin fibrosis, internal organ involvement, and vasculopathy. Juvenile systemic sclerosis (JSSc) represents less than 10% of all scleroderma patients.

A7.5 Limb Pain and swelling in children: sometimes it's not inflammation!

Background and Objectives Algodystrophy or Reflex Sympathetic Dystrophy is a localised chronic pain syndrome characterised by extreme and persistent pain, functional disability and refusal of mobilisation of a specific segment. Algodystrophy in children has several specific characteristics that are distinct from the disease seen in adults. Psychological stress and other psychological comorbidities play a prominent role in children, with frequent reports of a previous history of familiar problems, bullying or sexual abuse. Significant preceding trauma is not a common event in most paediatric series, as well as previous severe disease. This condition is more common in adolescent girls and pain typically occurs in a single lower extremity. Radiographs of the affected extremity are usually normal in paediatric patients and technetium bone scintigraphy usually show decrease uptake. Pharmacologic agents have a limited role in the treatment of these children. A multidisciplinary approach should be performed, including psychological, behavioural and occupational therapy. The authors present three case reports of Algodystrophy in children. Material and Methods Three patients from a Portuguese Pediatric Rheumatology centre. Results The first case is a ten year old girl presenting with severe arthralgia, diffuse edema and refusal to use the left foot which was experience a history of bulling in school. The second report of an eight year old girl, with a previous injury reported, presenting with arthralgia, allodynia and refusal to use the right foot, also with a previous history of bullying. And a third case of sixteen year old girl with severe pain, edema, cyanosis and functional impairment of her right hand that progress to addition of her left leg in which a history of previous sexual abuse has been found. One of these patients has been previous and wrongly treated with anti-TNF agents, without any clinical result. Conclusions The diagnosis of Algodystrophy in children must lead to a careful investigation of an underlying psychological cause. Its correction is the only way that allows a correct approach of these children and adolescents and enables to provide the best medical care.

AB1072 Work Disability, Productivity, Presenteeism and Absenteeism in Rheumatic Patients

Background Work capacity is primarily assessed by absenteeism and rheumatic patients may experience decreased productivity as well as presenteeism due to their health problems as well as its costs consequences. Objectives To evaluate the relation between work disability, productivity and disease activity, quality of life and functional disability. Methods 242 rheumatic patients were recruited, 33.3% employed (N=81) completed the questionnaires of work disability and productivity: WALS (Workplace Activity Limitations Scale), SPS 6 (Stanford Presenteeism Scale) and WPAI (Work Productivity and Activity Impairment) 4 scores - absenteeism, presenteeism, work and activity impairment. Patient-reported parameters included pain, fatigue, sleep quality and disease activity (VAS). Functional disability and quality of life outcomes were assessed by HAQ-DI, FACIT and SF-12. Data were collected during a 4-week period. The analysis included descriptive statistics, Mann-Whitney test and Spearman correlation, p <.05. Results 81 gainfully employed patients (85% female) had 48±11 years old, with 10±4 schooling years. Mean VAS were: pain 47±32, fatigue 57±33, sleep quality 46±33 and disease activity 43±30; HAQ-DI: 1.76±0.9, FACIT:17±11 and SF-12: 39±13 in PCS and 43±18 in MCS. Productivity assessment revealed limitations in all measured scores: WALS 8±6 [0-25], SPS 6 12±3 [3-15], WPAI work impairment 29±32%, activity impairment 29±29%, absenteeism 2.1±12.3% and presenteeism 28±32%. WALS was positively correlated to HAQ (r=.657, p<.0001), FACIT (r=.720, p<.0001), both SF12 scores (r>.517, p<.0001), all 4 WPAIs scores (r>.296, p<.01) and inversely with SPS 6 (r= -.341, p=.002). WPAIs scores were all significantly associated to HAQ, FACIT, SF12 PCS and sick leave in the last 12 months (p<.05). SPS 6 was inversely related to HAQ and FACIT scores, WPAI presenteeism, work and activity impairment (p<.05), but not with absenteeism. Pain and fatigue were significantly higher in patients with higher WALS and WPAI scores, in exception to absenteeism, and pain was associated with SPS 6 (p<.05). Sleep quality was decreased when related to higher WALS and WPAI scores (p<.05). When comparing the employed/unemployed patients, WPAI activity impairment, PCS, MCS, HAQ, FACIT, pain and disease activity (p<.01), we verified significantly higher limitations and impairment in the unemployed group, in exception to SPS 6, fatigue and sleep quality. Conclusions These findings suggest the significant impact of rheumatic disease in productivity losses, and that unemployed patients present worst quality of life and higher levels of pain, fatigue and disability. We found good correlations between the productivity assessment and the SF12, HAQ, FACIT, pain, fatigue, sleep quality and disease activity. This provides information about trend of work restrictions, useful in cost-effectiveness analysis for example of new treatment therapies. Moreover, these issues are particularly important because absenteeism and presenteeism have strong links to health related costs. Prevention of work disability and job changes/adaptations to the individual capabilities would be most effective in reducing socioeconomic and work related impact. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5488

AB1071 The Use of Visual Analogue Scale in Rheumatic Disease: Validation of an Electronic Version

Background VAS scales are very useful and easy to perform scales that rheumatologists use on a daily basis. There are several ways to perform this evaluation, on paper through a ruler that includes a slider indicator among others. With the use of more electronic patients records it is useful to determine if the use of a computer assisted VAS could perform the same as the paper. Objectives To evaluate and validate an electronic based VAS in a touch-screen platform. Methods Patients followed in our biologic clinic were evaluated with a paper version of several visual analogue scale (disease activity, pain intensity, back pain in the night, back pain anytime and how the disease disturbs) and after with the electronic version according to their diagnosis. The touch-screen was specially developed for our patients, integrating software that recognized the patient by disease through a bar code and presented the questionnaires according to the disease. Concordance between paper rand touch-screen questionnaire was done through Intraclass Correlation Coefficients. Internal consistency was evaluated by Cronbachs alpha coefficient. Results A total of 88 patients were included in the global disease scale (80.7% rheumatoid arthritis and 19,3% psoriatic arthritis) 85.2% were female, mean age was 54.34±11.05 years and mean disease duration was 11.83±9.32 years. Several other VAS used in spondyloarthropathies was compared in a group of 56 patients the majority were man (58.9%), 30.4% had psoriatic arthritis, 69,6% had ankylosing spondylitis mean age was 46.69±11.78 years and mean disease duration was 10.4±8.77years. Table 1. Results Touch-screen Paper ICC (Touch-screen vs Paper) VAS (last week) How the disease disturbs (n=88) Mean (standard deviation) 36.08 (25.56) 40.28 (27.19) 0.906 Pain intensity (n=87) Mean (standard deviation) 36.15 (25.87) 38.06 (25.63) 0.921 Spondylitis: VAS (last week) (n=56) Back pain during the night Mean (standard deviation) 25.61 (26.23) 25.55 (28.73) 0.943 Back pain at any time (day and night) Mean (standard deviation) 27.96 (25.09) 28.30 (26.37) 0.924 How the disease disturbs Mean (standard deviation) 30.57 (26.34) 29.66 (27.30) 0.867 ICC: Intraclass Correlation Coefficients. Conclusions We found no relevant difference between paper and touch-screen version of all the used VAS scales, with high correlation coefficients validating this platform. This is a useful instrument in our clinical practice, and could be a valid alternative to VAS on paper or rulers. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5384