BackgroundJuvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies assessment of the organ involvement is not standardized. Our project is the first one, where prospectively and with a standardized assessment data of jSSc patients are collected.Objectivesto learn about the characteristics and evolvement of jSScMethodsPatients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol.Results26 centers from 17 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 74 patients were enrolled. Sixty (81%) of the 74 patients were female. The mean age of the onset of Raynaud symptomatic was 9.2 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,7 years (0.3 -15.9). 56 (76%) of the 74 have diffuse subtype, 10 (14%) of them have an overlap symptomatic. At the time of the inclusion the mean modified Rodnan Skin Score was 16.0. ANA positive were 55/71 (77%), 24/70 (34%) of them were anti-Scl 70 positive and 3/42 (7%) was anticentromere positive. 43/74 (58%) had already capillary changes and 36/72 (50%) inactive ulcerations, 13/72 (18%) had active ulceration at the time of the inclusion. 38/74 (51%) had cardiopulmonary involvement, 19/38 (50%) of had signs of interstitial lung disease on imaging, 18/42 (43%) had FVC <80% and 12/21 (57%) had DLCO <80%. 6/38 (16%) patients had pulmonary hypertension. 5/74 (7%) had renal involvement. 26/74 (35%) had gastrointestinal involvement, and 23/26 (88%) of them esophageal involvement. 46/73 (63%) had musculoskeletal involvement. 2/74 (3%) showed neurologic involvement. The mean CHAQ score was 0.4 (0–2.5). Patient global disease activity on VAS (0–100) was 44.9 and disease damage 41.6. Physician global of disease activity on VAS (0–100) was 39.7 and physician global of disease damage was 34.6.ConclusionsThe current recruitment data confirms that pediatric patients are different from the adult patients, there is a significantly higher proportion of diffuse subset patients with 81%. 14% of the patients have overlap features.Disclosure of InterestNone declared

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Background The temporomandibular joint (TMJ), like any other synovial joint, may be involved in Juvenile Idiopathic Arthritis (JIA). According to the literature, the frequency of TMJ involvement varies from 17-87%, depending on the population under study, on the subtypes of the disease and on the method used to evaluate joint involvement. Condylar damage may be present early in the disease course and progress even in absence of clinically detectable symptoms or signs. Gadolinium-enhanced magnetic resonance imaging (MRI) is considered to be the gold standard in diagnosing early inflammatory changes of the TMJ in patients with JIA. Objectives The aims of our longitudinal study were to perform a comprehensive clinical evaluation of temporomandibular joint (TMJ) and to investigate the association between the magnetic resonance imaging (MRI) and clinical findings in TMJs and the disease activity of patients with JIA. Methods Seventy five patients with JIA participated in this study and were divided into 3 groups: patients with active disease, patients with clinical remission on medication and patients with clinical remission off medication. Thirty-nine patients had oligoarticular course and 36 had poliarticular/ systemic course. All patients underwent a rheumatologic examination performed by a pediatric rheumatologist, a TMJ examination performed by a dentist and MRI of the TMJs. These examinations were scheduled at the same date. The patients were examined again with one-year interval. Results The mean age at diagnosis was 6.0 years and the mean age at the first examination was 12.4 years. According to the degree of activity at the first examination, 33 patients were characterized as active, 21 were in remission on medication and 21 were in remission off medication. At the second examination, 21 patients were active, 28 were in remission on medication and 26 in remission off medication. At the first examination no symptoms were reported in 47 (62.7%) of patients and at the second evaluation in 64 (85.3%). The most common symptoms were pain in function, TMJ sounds and pain at rest. Synovial enhancement was present in 70 (93.3%) patients at the first phase exams compared with 65 (86.7%) at the second phase, one year apart. Intense contrast enhancement was significantly associated with active disease only at the first evaluation (p=0.0008), with poly/systemic subtypes at both evaluations (p=0.028 and p=0.049 respectively), with the presence of erosions at both evaluations (p=0.0001 and p<0.0001 respectively) and with altered condylar shape at the second evaluation (p=0.0005). Conclusions The TMJ should always be evaluated in JIA patients even in the absence of signs and symptoms; accordingly to the current established concepts TMJ synovial enhancement could be present even in patients in remission; although synovial enhancement is considered the gold standard in assessing TMJ involvement, we should be cautious in interpreting this finding. Disclosure of Interest None Declared

Background Juvenile dermatomyositis (JDM) is a rare autoimmune disease in childhood and the only available diagnostic criteria currently used are those defined by Bohan and Peter in 1975 1-3. Potential pitfalls occur in the laboratory diagnosis of JDM because serum levels of muscle enzymes are not elevated in every case, electromyography (EMG)presents with sampling errors and even muscle biopsy specimens may be normal due to patchy muscle involvement. Changes in clinical practice have resulted in the use of non-invasive techniques, such as magnetic resonance (MR) imaging, in place of EMG and muscle biopsy in the diagnosis of JDM 4-6. Objectives Our goal was to demonstrate the benefit of whole-body MR imaging as a diagnostic tool in the detection of muscle inflammatory activity in JDM and to correlate these findings with clinical evaluation including muscle strength tests, laboratorial exams, nailfold capillaroscopy and muscle biopsy. Methods Twenty-three patients aged 6-19 years, 17 girls and 6 boys with a diagnostic of JDM were prospectively evaluated at any point during their illness course. All patients were evaluated using clinical examination, muscle enzymes determination, muscle strength tests such as Childhood Myositis Assessment Scale (CMAS) and Manual Muscle Testing (MMT), nailfold capillaroscopy and short tau inversion recovery (STIR) whole-body MR imaging. JDM activity was evaluated by Disease Activity Score (DAS). An open muscle biopsy was performed in biceps braquialis if muscle disease activity was detected on MR exam. Results Whole-body scanning gave a complete assessment of all muscles groups and disease activity was detected in STIR MR imaging in three (13%) patients. Muscle biopsy confirmed inflammatory myositis in these three patients. All three patients had elevation of at least one muscle enzyme and the nailfold capillaroscopy showed scleroderma (SD) pattern. CMAS was <52 in all three patients; MMT was <80 and DAS <20 in two patients (not done in one patient). Twenty patients had inactive disease. Nailfold capillaroscopy was normal in twelve patients and with SD pattern in six patients (not done in two patients). Muscle strength tests (CMAS and MMT) were normal in twelve out of twenty patients (MMT not done in four patients). Conclusions Whole-body MR allows us to evaluate the extent and symmetry of muscle disease in a single exam by revealing muscles groups not seen with standard protocols. Muscle strength tests do not evaluate the accumulated effects in muscles over time in patients with JDM. References Reed AM, Lopez M. Juvenile dermatomyositis: recognition and treatment. Paediatr Drugs 2002; 4(5):315-21. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med1975;292:344-7. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med1975;292:403-7. Yosipovitch G, Beniaminov O, Rousso I, David M. STIR magnetic resonance imaging: a noninvasive method for detection and follow-up of dermatomyositis. Arch Dermatol 1999; 135:721-3....