Background/Objective: Few studies have been published focusing on the differences between juvenile idiopathic inflammatory myopathy (JIIM) and adult IIM. This study aimed to describe the characteristics of JIIM main subgroups (juvenile dermatomyositis [JDM] and juvenile polymyositis [JPM]) and to compare their differences with adult IIM subgroups (adult DM and adult PM).Methods: This study reviewed the medical records of patients from the REMICAM cohort, a multicentric longitudinal study carried out in patients with IIM, followed up between 1980 and 2014 in 12 hospitals in Madrid, Spain. Patients with definite or probable JPM, JDM, adult DM, and adult PM according to the modified Bohan and Peter criteria were selected. We compared the characteristics between JDM and JPM, and between JIIM and adult IIM.Results: Eighty-six juvenile patients (75 JDMs and 11 JPMs) and 283 adult patients (133 DMs and 150 PMs) were included. Compared with patients with JDM, patients with JPM were older at diagnosis, had more fever and arthritis, and were less frequently treated with disease-modifying antirheumatic drugs (these differences were not statistically significant). Compared with patients with adult DM, those with JDM presented more frequently with calcinosis (33.8% vs 6.9%, p < 0.0001) and had less severe infections (4.3% vs 23.4%, p < 0.0001), malignancies (1.3% vs 25.6%, p < 0.0001), and mortality (3.5% vs 33%, p < 0.0001). Patients with JDM were treated less frequently with azathioprine (10.8% vs 44.7%, p < 0.0001).Conclusions: Our findings confirm that JIIMs are a heterogeneous group of diseases with relevant differences compared with adult IIMs.

BackgroundA collaborative EULAR/ACR Project has developed new criteria for inflammatory myopathies(IM) and their subgoups1 ObjectivesTo analyse agreement between the 2017 IM classification criteria and the Bohan and Peter(BP) criteria in REMICAM cohort2 MethodsAll patients were included. New criteria were applied to obtain classification as: possible(Pos), probable(Pro) and definitive(Def) IM, and subclassification in 6 subgroups: polymyositis(PM), dermatomyositis(DM), juvenile DM(JDM), amiopathic DM(ADM), inclusion body myositis(IBM) and juvenile myositis(JM). The 7 subgroups in REMICAM were harmonised to fit the 6 subgroups of the 2017 criteria. Agreement between 2017 and BP criteria was analysed in classification/subclassification, calculating the weighted kappa value (k). Subanalysis including only patients with available data on the muscle strength items required for the 2017 criteria, and in those having also muscle biopsy data, were conducted.ResultsFrom 479 REMICAM patients, 477 (99.6%), fulfilled BP criteria (5.9%Pos, 26.8%Pro, 67.4%Def) and 431 (89.9%) 2017 criteria (2.5%Pos, 21.8%Pro, 65.7Def). Global agreement between both criteria was 89.5%. Agreement between subtypes (Pos, Pro, Def) was low (k=0.15). When 399 patients with muscle strength data, and 243 with muscle biopsy data were analysed, results were similar (k=0.17). Disagreement was mainly seen in Pos/Pro subtypes with BP criteria, since 60% classified as Def when the 2017 criteria were applied. Agreement in the different subgroups of IM (PM, DM, JDM, ADM, IBM, JM) between both criteria was very high (k=0.94).ConclusionsThe new 2017 EULAR/ACR criteria for IM classification show good agreement with BP criteria in the REMICAM cohort. New criteria classify 60% of Pos/Pro patients by BP criteria, as Def, and show very high agreement between IM subgroups. Validation studies are needed, but our results in this large cohort suggest the 2017 criteria might be useful for clinical trials and research in IM.References[1] Lundberg IE. Ann Rheum Dis2017;76:1955–64.[2] Nuño L. Rheumatol Clin2017;13:331–7.Disclosure of InterestNone declared

Background:Current recommendations for axial Spondyloarthritis (axSpA) include the treat-to-target concept and suggest that the ideal target should be remission or low disease activity (LDA). Also, the importance of a shared decision is highlighted. Unfortunately, the definition of remission is not consensual, and most of the definitions are difficult to evaluate in clinical practiceObjectives:To propose an evaluation of remission by a single question to the patient, by comparing it to the different available definitions. To analyze the metric properties of the current definitions against patient’s perceptionMethods:One-center cross-sectional study in a tertiary care hospital including consecutive patients with a diagnosis of axSpA (and fulfilling the ASAS criteria) were included between February to November 2019. Patient’s perception of remission and LDA was evaluated by a single question. Physician’s perception of remission and LDA was assessed identically. The level of agreement between patients’ perception and the other available definitions was tested by the Prevalence and Bias adjusted Kappa (PABAK). The metric properties Sensitivity (S) and Specificity (Sp) of the available definitions (BASDAI cut-offs, ASDAS disease states, ASAS criteria for partial remission and patient acceptable symptom state), were tested against the patients perspective, as the gold standard.Results:A total of 105 axSpA patients were included. 63,8% were males and 67,6% had radiographic sacroiliitis (Table 1). 21% and 72% of them considered themselves in remission and LDA, respectively. Physician’s perception was 45.7% and 81% for remission and LDA, respectively. The prevalence of the different definitions are shown in Figure 1. The best agreement for patients’s perception of remission was found with a BASDAI <2 + normal CRP (Table 2). This definition was also the most sensitive (S=72,7%) and specific (Sp=83,1%) when taking the patient’s perception as a reference.Table 1.Characteristics of 105 patients with axSpAAll (N:105)Patients in self-defined REM (N:22)Patients in self-defined LDA (N:54)Patients No REM no LDA (N:29)Male, n (%)67 (63,8)18 (81,8)34 (63)15 (51,7)r-axSpA, n (%)71 (67,6)17 (77,3)33 (61,1)21 (72,4)Mean age, years (SD)49 (13)51(15)47 (13)50 (11)Mean AxSpA duration, years (SD)12,2 (13)17,1 (16,2)11,2 (11,7)10,3 (12,3)HLA- B27+, n (%)Data from 10472 (69,2)17/22 (77,3)33/54 (61,1)22/28 (78,6)Periferic arthritis, n (%)34 (32,4)7 (31,8)17 (31,5)10 (45,4)Uveitis, n (%)22 (21)6 (27,3)10 (18,5)6 (20,7)Biological treatment, n (%)43 (41)14 (63,6)19 (35,1)10 (34,5)CRP, mean (SD)3,61 (5,36)2,31 (2,17)2,84 (3,87)6,04 (8,14)ASDAS, mean (SD)1,78 (1,08)0,98 (0,71)1,63 (0,89)2,68 (1,03)BASDAI, mean (SD)3,35 (2,32)1,39 (1,30)3,13 (1,84)5,26 (2,33)BASFI, mean (SD)2,81 (2,45)1,24 (1,37)2,57 (2,00)4,43 (2,92)Table 1.REM: Remission; LDA: Low Disease Activity; SD: Standard Desviation; CRP: C-Reactive Protein IBD: Inflammatory Bowel Disease.Table 2.Agreement between different definitions of remissionASDAS <1,3BASDAI<2+Normal CRPPGA ≤1PhysicianREMPatientREMASAS PR0.53 (0.58)0.59 (0.68)0.76 (0.83)0.22 (0.26)0.39 (0.56)ASDAS <1,30.64 (0.68)0.50 (0.56)0.44 (0.45)0.28 (0.37)BASDAI <2+Normal CRP0.60 (0.69)0.25 (0.28)0.50 (0.62)PGA ≤10.20 (0.24)0.42 (0.62)Physician REM0.20 (0.24)Agreement is presented as Cohen’s Kappa (PABAK: prevalence and bias adjusted kappa).Patient and Physician remission (REM) are based on the single question; ASAS PR:ASAS partial remission; PGA: Patient global assessment.Conclusion:In this real-life population, the evaluation of remission by the patient through a single question was shown to be feasible and to present an acceptable agreement with other definitions.References:[1]Gorlier C, et al. Ann Rheum Dis 2019;78(2):201-8.Fig. 1.REM/LDA: remission/ low disease activity self-defined patient or physician through a simple question. ASDAS <1,3: inactive disease; ASDAS <2,1: low activity; PGA: Patient global assessment; PASS: Patient acceptable symptom state.Acknowledgments:To Ansgar Seyfferth and Alfonso Muriel. To Carlos Sanchez-Piedra, Fernando Alonso and Mercedes Guerra from Sociedad Española de Reumatología, Research Unit.Disclosure of Interests:Marta Valero Grant/research support from: Novartis, Pfizer, Abvie, Speakers bureau: Novartis, Celgene, Javier Bachiller-Corral: None declared, Boris Anthony Blanco Cáceres: None declared, M. Ángeles Blázquez: None declared, Consuelo Díaz-Miguel: None declared, Maria Jesus Garcia Villanueva: None declared, Carmen Larena: None declared, Jose Luis Morell Hita: None declared, Carlos De la Puente Bujidos: None declared, Ana Rodriguez-García: None declared, Mónica Vázquez Díaz: None declared, Anna Moltó Grant/research support from: Pfizer, UCB, Consultant of: Abbvie, BMS, MSD, Novartis, Pfizer, UCB

BackgroundTransition is the process by which a young patient with a chronic disease is able to develop skills and have access to resources to ensure that physical, psychological, educational and vocational needs are covered during the stage from youth to adulthood. According to SERPE and EULAR/PReS recommendations, the goal of transition programs is to improve the support to patients during this process, mainly working in multidisciplinary teams.ObjectivesOur main purpose was to accomplish self-care, illness awareness, stress management and negative emotions workshops focused in improving their life quality.MethodsWorkshops are conducted by a psychologist, a pediatric physiotherapist, two rheumatologists and a rheumatology nurse at our center. It consists of a total of 7 workshops (first six for patients and the last for parents). Five of these workshops are based on HEADSS system (home, education, activities, drugs, sexual activity, and emotions) and the two others are focused on disease and treatment consciousness. Parents signed an informed consent document. Workshops lasted 90 minutes in sessions outside of the consultation hours. At baseline, several questionnaires were completed, VAS pain (parents and patients), VAS general (parents and patients), JAMAR (parents and patients), PedsQL 4.0, those questionnaires were also completed after 3 months together with a satisfaction survey.ResultsA total of 12 patients were included, with ages between 11 to 16 years old, with an average of 14 years old. A total of 10 patients completed 100% of the program (6 girls and 4 boys). 6 patients had AIJ (oligoarticular, enthesitis-related arthritis, psoriatic arthritis), 3 suffered from LES and 1 had Behçet. All patients were under immunosuppressive therapy and 60% of them received biological treatment. 20% of patients had clinical activity data. The average of global VAS was 12 (0–100) and the average of VAS pain was 0.9 (0–100). The average of initial PEDSQL 4.0 was 80.7 (0–100) and the PEDSQL 4.0 at three months was 74.4. At the end of the workshops, 100% of parents and patients would recommend other patients to attend it, more than 50% of patients think that they would be able to go to medical visits without their parents and 90% of them would take responsibility for their treatments. A 90% of patients think that workshops have helped to improve their relationship with their rheumatologist and 60% of them have improved their knowledge about the disease. Regarding to physical activity leves, 30% of the patients have increased it compared to baseline. More than 50% of parents have observed a positive attitude change towards the disease after the program.ConclusionsTransition programs are important for the transfer to be effective throughout the involvement of adolescent, who takes responsibility for his/her disease and also to ensure their psychosocial needs are met. Rheumatologists must be ready to cover these needs with the support of other specialists. Our experience with the program was very positive since most of the pa...

BackgroundMacrophage activation syndrome (MAS) is a severe complication of several rheumatologic diseases, being of special relevance systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA). Its characterized by an excessive activation of the immune system due to various mechanisms, including hyperactivation of macrophages and a failure in downregulation activity by NK and cytotoxic lymphocytes. There are various criteria for its diagnosis, highlighting secondary lymphohistiocytosis syndrome (HLH) criteria from 2004 and provisional secondary MAS criteria for JIA proposed by Ravelli in 20161.ObjectivesTo describe a case series of patients with MAS.MethodsThis is a retrospective case series of 16 patients with MAS secondary to systemic autoimmune diseases diagnosed in Ramón y Cajal Universitary Hospital between April 2009 and September 2018.ResultsThe baseline pathology was sJIA in 8 patients (2 cases with 2 episodes) and SLE in the other 6 patients. Mean age at diagnosis was 17.44 years for sJIA and 37.5 years for SLE. Mean time from diagnosis of the baseline disease to MAS episode was 11.31 years, with 3 cases being the initial manifestation of their systemic disease. 43.8% of patients were treated with corticosteroids previously to MAS episode, and 50% were being treated with DMARDS/biologic agents (SLE: 3 patients with hydroxychloroquine and 1 patient with mycophenolate and hydroxychloroquine; sJIA: 2 patients with Anakinra, 1 patient with tocilizumab and 1 patient with etanercept). Clinical and analytical characteristics of the patients are presented in table 1 and table 2, respectively. In SLE group, only 2 patients (33.3%) had high anti-DNA titers during the MAS episode, 5 patients (83.3%) had increased C3 consumption and 4 patients (66.6%) had increased C4 consumption. As severe manifestations, 4 SLE patients presented neurologic abnormalities and 3 patients presented external hemorrhage. Infection was confirmed as a trigger in 3 patients with SLE (50%) and 4 patients with JIA (40%). Prednisone at high doses was prescribed to all patients, cyclosporine in 4 patients (66.6%) with SLE and 9 patients (90%) with sJIA. Additionally, anakinra was prescribed in 4 patients (40%) with sJIA. 4 patients (66.6%) with SLE met secondary HLH criteria and 9 patients (90%) with sJIA met secondary MAS criteria. Bone marrow biopsy was performed in all patients with SLE and in 9 patients with sJIA, demonstrating hemophagocytosis in 5 patients (83.3%) with LES and 5 (50%) patients with sJIA. Only 2 patients in the SLE group died because of MAS.Table 2 Analytical measures. Hb=haemoglobin(g/dl), Nt=neutrophils (103/mm2), Pla=platelets (103/mm2), Cr=creatinine (mg/dl), Brb=bilirubin (mg/dl), AST (UI/L), ALT (UI/L), Fe=ferritine (ng/ml), Tg=triglycerides (mg/dl), Fb=fibrinogen (mg/dl), INR (International Normalizated Ratio). Hypotension Fever Rash Adenopathy Splenomegaly Hepatomegaly Haemorrhage sJIA 1 (10%)7 (70%)7 (70%)3 (30%)4 (40%)1 (10%)0 SLE 04 (66.7%)1 (16.7%)3 (50%)5 (83.3%)4 (66.7%)3 (50%...

BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disease that is more severe in pediatric population than in adults. Biological therapy with anti-CD20 (rituximab) is an option in patient that do not respond to conventional therapy.ObjectivesThe aim of this study is to determine the clinical and immunological response in 9 patients with childhood-onset systemic lupus erythematosus (cSLE) that received treatment with rituximab in a third level hospitalMethodsThis is a retrospective observational study. 9 patients treated with Rituximab between November 2007 and October 2016 were included and their medical records were reviewed. The response to treatment at 6 months and one year after the first infusion of Rituximab were assessed. Patients with overlap syndromes were excluded. All patients fulfilled four or more of the 1982 revised American College of Rheumatology criteria for the diagnosis of SLE (<16 years).ResultsNine pediatric patients with SLE treated with rituximab were included, all of them were female. The age at diagnosis of SLE was a mean of 15,22 years. The mean time duration of disease was 87,55months (5–255m). 7 patients were caucasians. Rituximab was indicated in 6 patients with class IV of lupus nephritis (LN) 1/9 with class III LN, 1/9 with severe cutaneous lupus, and with severe hematological manifestations in 1 case (haemolytic anemia). In addition, 6/9 patients had mucocutaneous and articular manifestations. The disease activity of all patients was assessed using SELENA-SLEDAI index pre rituximab infusion, the mean was17,11 (8–33). All patients had low level of complement C3 and C4 and 8/9 increased anti-DNA. In 8/9 patients Rituximab was used as a rescue treatment and in a single case as a first line.3/6 patients with renal involvement were previously treated with cyclophosphamide (CF) iv and mycophenolate, 2/6 CF. In case of cutaneous involvement the previous treatment was methotrexate, azathioprine (AZA) and dapsone and in case of hemolytic anemia was AZA.The treatment protocol was 1 gram x 2 (1 cycle) in 7/9 patients, 375mg/m2 x 4 in 1/9 cases and 600mg monthly for 5 months in the case of hemolytic anemia. Five patients received more than 1 cycle. After the administration of Rituximab, the SELENA-SLEDAI activity index was 4.5 points. At 6 months a complete response was obtained in the case of hematological and cutaneous manifestations, in 2 cases of lupus nephritis (proteinuria <0.5 g/day) and partial response was obtained in 2 cases. Data were not analyzed in 2 patients (death and less than 6 months of the first dose of rituximab). Patients with partial response and lack of response achieved complete response at 12 months. 2/9 patients had side effects (Rituximab pneumonitis in 1 case and infections in 2 cases). Mortality was 11.11% (1/9 patients, per infection and lupus activity, SLEDAI pre rituximab =33)ConclusionsIn our study, although it consisted of few patients, it was objected that Rituximab therapy in patients with cSLE is effective, reduces lupus activity index, especiall...

BackgroundAssociations between muscle pathology and clinical features in inflammatory myositis(IM) are not well definedObjectivesto describe the pathological findings in REMICAM1 muscle biopsies, and analyse their associations with clinical featuresMethodsAll patients with available biopsy were included. According to the score proposed by ENMC workshop2, from muscle biopsy reports was extracted: inflammatory cells and location (endomysial, perimysial, perivascular), rimmed vacuola, fibre atrophy, necrosis, regeneration, and HLA expression. Descriptive analysis and association studies between histology and clinical features were performed (t and chi square tests, univariate logistic regression with OR)ResultsFrom 479 patients, 244 (51%) had available biopsy. Most frequent findings were: inflammatory infiltrates(75%): endomysial(44%), perimysial(49%) perivascular(26%); fibre atrophy(54%), perifascicular(27%); necrosis(55%) and regeneration(47%). HLA expression was +in 60/75 cases(80%). Endomysial infiltrate was associated with PM (OR 0.4;p<0.0001), cardiac involvement (OR 2.2;p=0.014), less arthritis (OR 0,5;p=0.01) and older age (51 vs 42 y;p<0.01); perimysial infiltrate and perifascicular atrophy with DM (OR 3.8;p<0.0001) and younger age (40 vs 48 y;p<0.05); necrosis with cardiac involvement (OR 2.1;p=0.035), neoplasia (OR 2.5;p=0.02) and no connective tissue disease (OR 0.4;p=0.01); and HLA with PM (OR 0.1;p=0.003)Conclusionsmost frequent histologic findings in IM muscle are inflammatory infiltrates, necrosis, HLA expression and perifascicular atrophy. Endomysial infiltration and HLA are characteristic of PM, and perimysial infiltration/perifascicular atrophy of DM. Endomysial infiltrates are associated with cardiac involvement. Necrosis is more frequent in neoplasia and less in connective tissue diseases, and is associated with cardiac involvement and older age. Our data suggest that muscle biopsy might help to identify those IM patients at higher risk of severe complications, as neoplasia or cardiac involvementReferences[1] Nuño L. Rheumatol Clin2017.[2] De Bleecker JL. Neuromuscular Disorders2015.Disclosure of InterestNone declared

BackgroundCalcinosis is a cutaneous manifestation that produces disability and complications in patients with idiopathic inflammatory myopathies (IIM). Studies about associated factors are limited.ObjectivesTo investigate cutaneous manifestations and factors associated with calcinosis in patients with IIM from the REMICAM registry.MethodsA multicenter retrospective study (1980–2014) was performed. Patients were classified as primary dermatomyositis (DM), primary polymyositis (PM), cancer associated myositis (CAM), overlap myositis (OM), inclusion body myositis, immune-mediated necrotising myopathy (IMNM) and juvenile myositis (JuM). A description of the cutaneous manifestations in each subgroup of IIM was done. The associations between calcinosis and demographic data, clinical characteristics, antibodies and treatments were analysed independently for JuM and adult IIM.Results479 patients were included, of whom 49 (10%) had calcinosis with the following distribution: 27 JuM (55%), 9 DM (18%), 9 OM (18%), 3 PM (6%) and 1 IMNM (2%). Non- characteristic cutaneous manifestations were more prevalent in DM, OM, CAM and IMNM. At multivariate analysis, disease duration (OR=2; p=0.016), immunoglobulin treatment (OR=5.68; p=0.036) and immunosuppressants (OR=8.94; p=0.01) were associated with calcinosis in JuM. In addition, dysphagia (OR=21.3; p=0.008), positivity for anti-PM-Scl (OR=34.1; p=0.01), baseline ESR (OR=1.05; p=0.04) and interstitial lung disease (OR=10.2; p=0.039) were associated with calcinosis in adult.ConclusionsOur results suggest that factors associated with calcinosis might be different in adult and juvenile myositis, but in both cases, they are related to more severe disease.Disclosure of InterestNone declared