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Purpose To assess the impact of hexaminolevulinate (HAL) on the long-term recurrence rate of NMIBC. Methods A total of 130 patients with bladder tumour were randomized into two groups. The patients in one group had a HAL instillation before surgery, and they first had a white-light and after that a blue-light cystoscopy (BL group) and resection. The second group had only white-light cystoscopy (WL group) and resection. They have been followed up with cystoscopy every 3 months for a period of up to 40 months. Results The recurrence-free period was not significantly different between the two groups (BL and WL groups) (long-rank test p  = 0.202). The use of HAL helped detect four flat lesions and 28 papillary lesions with cancer that would have been missed under WL only, on 16 out of the 54 patients (29.6 % CI 95 % 11.1–33.3). The use of HAL changed the proposed postoperative treatment and follow-up for one out of the five patients. Conclusions Although the use of HAL cystoscopy identified at least one cancer lesion more than WL cystoscopy on one out of the three patients, the recurrence-free period was not significantly different.

Organizations collect log data for various reasons, including security related ones. The multitude and diversity of the devices that generate log records increases, resulting to dispersed networks and large volumes of data. The design of a log management infrastructure is usually led by decisions that are commonly based on industry best practices and experience, but fail to adapt to the evolving threat landscape. In this work a novel methodology for the design of a dynamic log management infrastructure is proposed. The proposed methodology leverages social network analysis to relate the infrastructure with the threat landscape, thus enabling it to evolve as threats evolve. The workings of the methodology are demonstrated by means of its application for the design of the log management infrastructure of a real organization.

BackgroundSystemic juvenile idiopathic arthritis (sJIA) accounts up to 15 % of all patients with JIA and is distinct from the other disease categories due to the association of articular and extraarticular manifestations. The systemic Juvenile Arthritis Disease Activity Score (sJADAS) is a composite disease activity score validated specifically for use in sJIA that includes, beside the four components of the original JADAS, a fifth item aimed to quantify the burden of systemic features. The interpretation of scores on sJADAS requires criteria that identify the states of disease activity. These criteria can be used to monitor the disease course over time and to define therapeutic targets.ObjectivesTo compare the clinical and laboratory data of each disease activity state in patients enrolled in the multinational study aimed to define the sJADAS cutoffs.MethodsData were extracted from a multinational cross-sectional dataset that included patients diagnosed as sJIA by ILAR criteria, recruited between February 2022 and November 2022. At study visit, each patient was categorized subjectively by the caring physician into one of the following disease activity states: inactive disease (ID), low (or minimal) disease activity (LDA), moderate disease activity (MDA), or high disease activity (HDA). Study data was collected through a standard case report form and entered into an electronic database.ResultsA total of 231 patients were enrolled in 29 centers in 12 countries. The mean age at diagnosis was 5.63 years. 87 patients (37.7%) were judged as having ID, 39 (16.9%) LDA, 46 (19.9%) MDA and 59 (25.5%) HDA. The comparison of the main clinical and laboratory features across patients with the four disease activity states is shown in the Table 1. Overall, the presence of extraarticular manifestations was more common in patients with MDA and HDA (P<0.00001), whereas fever, rash, hepatosplenomegaly, and lymphadenopathy were more frequent in HDA patients (<0.00001). The count of active joints increased progressively from ID to HDA (p<0.00001). The mean values of physician global assessment of disease activity and systemic manifestations, as well as the mean values of acute phase reactants, were highest in patients with HDA, with gradual decrease from MDA to LDA to ID.ConclusionThis preliminary analysis of the study data indicates that the subjective assessment of disease state by the caring physicians led to discriminate reliably patients with different level of disease activity. This evaluation will, then, serve well as reference for the subsequent analyses aimed to identify the cutoffs for the main disease activity states in sJIA.Reference[1]Tibaldi J, Pistorio A, Aldera E, et al. Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis. Rheumatology (Oxford). 2020 Nov 1;59(11):3505-3514.Table 1.Comparison of clinical and laboratory features across disease activity states (n = 231). ID=inactive disease; LDA= low (or minimal) disease activity; MDA=moderate disease activity; HDA=high disease activity; MD global VAS=physician global assessment of disease activity; MD systemic VAS= physician global assessment of systemic disease activity; ESR= erythrocyte sedimentation rate; CRP= C-reactive protein; NAJ=Number of active joints.IDLDAMDAHDANumber of patients (n, %)87 (37.7)39 (16.9)46 (19.9)59 (25.5)Age at onset, years (mean, SD)6.17 (4.24)5.44 (3.06)5.11 (3.42)5.36 (4.06)MD global VAS (mean, SD)1.08 (2.64)2.5 (2.59)5.03 (2.59)6.61 (2.91)MD systemic VAS (mean, SD)0.06 (0.23)0.88 (1.13)3.08 (2.69)7.52 (2.17)Systemic features (n,%)1 (1.1)7 (17.9)22 (47.8)56 (94.9)• Fever0 (0)0 (0)19 (41.3)55 (93.2)• Rash0 (0)4 (10.3)9 (19.6)29 (49.2)• Hepatomegaly0 (00 (0)2 (4.3)21 (35.6)• Splenomegaly0 (0)1 (2.6)4 (8.7)14 (23.7)• Lymphadenopathy1 (1.1)2 (5.1)5 (10.9)20 (33.9)• Serositis0 (0)0 (0)4 (8.7)9 (15.3)ESR, mm/h (mean, SD)8.46 (7.63)16.26 (14.43)46.11 (34.53)66.85 (32.4)CRP, mg/dl (mean, SD)0.39 (0.85)0.88 (1.93)5.17 (7.03)7.89 (6.06)NAJ > 1 (%)1 (1.1)13 (33.3)34 (73.9)53 (89.8)AcknowledgementsThis work was partially supported by the Fundación Española de Reumatología (FER).Disclosure of InterestsAna Isabel Rebollo Giménez: None declared, Yulia Vyzhga: None declared, Luca Carlini: None declared, Silvia Rosina: None declared, Elisa Patrone: None declared, Maria Katsikas: None declared, Claudia Saad-Magalhaes: None declared, Dalia El-Ghoneimy: None declared, Yasser El Miedany: None declared, Raju Khubchandani: None declared, Priyankar Pal: None declared, Gabriele Simonini Grant/research support from: educational grants from Abbvie, Sobi, Novartis, Giovanni Filocamo Consultant of: consultancies from Sobi and Novartis, Maurizio Gattinara: None declared, Fabrizio De Benedetti: None declared, Davide Montin: None declared, Adele Civino: None declared, Motasem O. Alsuweiti: None declared, Valda Stanevicha: None declared, Vyacheslav Chasnyk: None declared, Ekaterina Alexeeva Speakers bureau: speakers bureaus for Roche, Novartis and Pfizer., Grant/research support from: research grants from Roche, Pfizer, Centocor, Eli Lilly, AbbVie, Bristol-Myers Squibb, MSD, Sanofi, Amgen and Novartis., SULAIMAN AL-MAYOUF Speakers bureau: speaker fees from Sobi and Novartis, Soamarat Vilaiyuk: None declared, Angelo Ravelli Speakers bureau: from:Abbvie, Angelini, Pfizer, Novartis, BMS, Reckitt Benckiser, Sobi, Alexion, Roche.

Background:Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients diffuse subtype is associated with higher number of organ systems involvement. In a CARRA North American study, it was noted that 38% of jSSc patients had four or more organ systems involved. This topic has not yet been assessed in a large juvenile scleroderma inception cohort (jSSc) cohort.Objectives:To assess the number of organ systems involved in the diffuse and limited jSSc patients at the time of inclusion in the cohort.Methods:The jSSc is a prospective cohort including patients, who fulfill the adult SSc criteria[1], with first non-Raynaud symptom before the age of 16 years and under 18 years of age at the time of inclusion. We reviewed the number of organ systems involved at the time of inclusion into the cohort. The categorization of the organ system involvement was skin, vascular, muscular, articular, pulmonary, cardiac, gastrointestinal, renal and nervous system. We compared the number of involved systems between diffuse and limited jSSc subtype.Results:Until 1st of December 2023, 253 patients were enrolled and 177 of them had diffuse subtype. The median age at the onset of Raynaud´s was 10.4 years. The median age of the first non-Raynaud organ involvement was 10.9 years. The median disease duration was 2.5 years. The distribution of the cumulative organ involvement in the whole group and in the diffuse and limited subtype can be seen in Table 1. Similar to the CARRA study, a large number of jSSc patient had 4 or more organ systems involved, almost half of the cohort (46%). There was no significant difference between the cumulative number of organ systems involved between the SSc subtypes in the Inception cohort.Comparison of diffuse/limited jSSc at time of inclusion in the cohortWhole GroupN=253Diffuse SubtypeN=177Limited SubtypeN=76P valueNumber of organs involved0.22915% (13/253)3% (5/177)11% (8/76)221% (52/253)20% (35/177)22% (17/76)328% (72/253)29% (51/177)28% (21/76)423% (59/253)24% (43/177)21% (16/76)516% (40/253)18% (31/177)12% (9/76)66% (15/253)6% (11/177)5% (4/76)71% (2/253)1% (1/177)1% (1/76)80% (0/253)0% (0/177)0% (0/76)90% (0/253)0% (0/177)0% (0/76)Conclusion:In this largest jSSc cohort in the world, approximately half the enrolled children have 4 or more organ systems involved, which highlights the overall severity of the disease. There was no significant difference in jSSc children skin subtypes, lcSSc or dcSSc regarding, the cumulative number of organ systems involved, although as shown in our publications[2] the diffuse subtype presented more severe disease.This project was supported by an unrestricted grant from „Joachim Herz Stiftung“REFERENCES:[1] van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.[2] Foeldvari I, Klotsche J, Kasapcopur O, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence in 3 in 1 000 000 children. Positive nailfold capillaroscopy(NF+) finding correlate with more severe disease in adult systemic scleroderma[1]. There is currently no data, if this correlation does exist in jSSc.Objectives:To assess the difference in patients with jSSc with normal (NF-) and pathologic NF(NF+) findings at the time of inclusion in the cohort.Methods:Baseline data was extracted from patients enrolled in the juvenile scleroderma inception cohort that had nailfold capillaroscopy performed at inclusion [2] until 1st of December 2023. NF was performed by dermatoscope and/or high-resolution video nailfold capillaroscopy. We compared patients with NF+ and NF- findings from the baseline visit using chi-square test.Results:237 patients were included in the analysis, 185 (78%) of them were female. 126 (70%) had diffuse subtype. 183/237 patients (77%) were in the NF+ group. 71% in the NF+ group were Caucasian compared to 85% in the NF- group (p=0.051). Median disease duration was 2.3 years in the NF+ and 3.2 years in the NF- patients. Median age at onset of the first non-Raynaud´s was around 11 years in both groups. More patients in the NF+ group were ANA positive (95% compared to 79%, p <0.001). There was no difference in the anti-Scl70 or anti-centromere distribution.NF+ patients had significantly more frequent Raynaud phenomenon (96% compared to 78%, p<0.001); history of digital ulcerations (59% compared to 27%, p<0.001); abnormal high resolution CT findings of the lung (49% compared to 30%, p=0.034); overall gastrointestinal involvement (49% compared to 20%, p<0.001); oesophageal involvement (47% compared to 19%, p<0.001); musculoskeletal involvement (71% compared to 41%, p=0.003); presence of joints with decreased range (63% versus 45%, p=0.022) and presence of muscle weakness (25% compared to 3%, p=0.002). No significant differences were demonstrated in involvement of other organ systems such as skin, cardiac or renal. (see Table 1)Conclusion:In a jSSc cohort there were significantly more patients affected within various organ systems in those with nailfold capillary changes at enrollment compared to those without. Future studies should assess whether these differences persist over time.This project was supported by an unrestricted grant from “Joachim Herz Stiftung”REFERENCES:[1] Vanhaecke A, Cutolo M, Distler O, et al. Nailfold capillaroscopy in SSc: innocent bystander or promising biomarker for novel severe organ involvement/progression? Rheumatology (Oxford). 2022 Nov 2;61(11):4384-4396.[2] Foeldvari I, Klotsche J, Kasapcopur O, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:In adult systemic sclerosis they are significant differences in clinical presentation of diffuse and limited subtype. In juvenile systemic sclerosis (jSSc) are the differences less prominent as we reviewed last time in a publication for the first 150 patients [1] of the juvenile scleroderma inception cohort (jSScC). The differences can change as the included number of patients is growing in the cohort.Objectives:To review the differences of clinical characteristics of patients and patient (PRO) and physician (PhRO) reported outcomes with limited jSSc (ljSSc) and diffuse (djSSc) subtype in the jSScC.Methods:We extracted date from the jSScC including patients who were enrolled till 1st f December 2023 into the cohort [1]. We compared the clinical characteristics, PRO and PhRO of the two subtypes and calculated statistical significance using chi-square test.Results:253 patients were included in the study. 70% (n=177) of the patients had diffuse subtype. Around 70% of the patients were Caucasian in both groups. The median age of onset of Raynaud’s were 10.1 in the djSSc and 11.8 years in the ljSSc. The median age at the time of the first non-Raynaud was 10.5 years in the djSSc and 12.0 years in the ljSSc. Looking at antibody profile the anti-centromere antibodies were significantly more frequent in ljSSc (11% versus 3%, p=0.025). djSSc patients had significantly higher (Table 1). Modified Rodnan skin score (16 versus 4, p=0.002), more frequently Gottron papules (32% versus 15%, p=0.004), with sclerodactyly (85% versus 54%, p <0.001), with telangiectasia (42% versus 21%, p=0.003), with history of ulceration (61% versus 30%, p<0.001)), with decreased Body mass Index <2 standard deviation(19% versus 6%, p=0.008), and presence of joints with decreased range of motion (65% versus 46%, p=0.004). None of the patients had renal crisis. There was no significant difference in cardiopulmonary and gastrointestinal involvement. Looking at PRO and PhRO in all categories djSSc patients had significantly more severe disease (Table 2).Conclusion:This results present a different organ involvement pattern form adults. Despite more severe disease according to patient and physician reported outcomes, we found no significant differences in the cardiopulmonary and gastrointestinal involvement between the subtypes. The antibody profile anti-Scl70 and anti-PMscl was not different between subtypes either.This project was supported by an unrestricted grant from „Joachim Herz Stiftung“REFERENCES:[1] Foeldvari I, Klotsche J, Kasapcopur O, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022 Oct;74(10):1575-1584.Acknowledgements:NIL.Disclosure of Interests:None declared.

BackgroundJuvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients decreased body mass index (BMI) correlates with higher mortality. We hypothesized that jSSc patients with lower BMI at presentation have more severe organ involvement.ObjectivesTo compare the organ involvement pattern at inclusion into the cohort of jSSc patients with BMI ≤ -2 z score with the patients with higher BMI.MethodsWe reviewed the clinical characteristics of patients who were recruited to the juvenile jSScC till 1st of December 2022. We compared patients with BMI ≤ -2 z score with patients (lwgroup) with higher BMI (nlwgroup). jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion.ResultsAt the time of the evaluation, we had 232 patients in the cohort and 217 of them had BMI data to include in the evaluation. Thirty-three patients were in the lwgroup (15%) and 88% (n=29/33) of them diffuse subtype and in the nlwgroup 64% (113/177). The median age at onset of Raynaud phenomenon in the whole group was 10.6 years and the median age at the first non-Raynaud symptom in the whole group was 11.0 years. Median disease duration in the whole group was 2.4 years at the time of inclusion. Approximately 95% of the patients were treated with a DMARD. There were no statistically significant differences between the lwgroup compared to nlwgroup regarding antibody pattern, inflammatory marker or organ involvement pattern, except higher number of patients with Gottron papules (41% lwgroup vs 25% nlwgroup; p=0.01) and sclerodactylia (84% lwgroup vs 73 % nlwgroup; p=0.049). Regarding the patient related outcomes at inclusion in the cohort, the global disease activity by VAS 0-100 was 40 in both groups (p=0.032), but the patient global disease damage by VAS 0-100 was 50 in the lwgroup which was significantly higher compared to 30 nlwgroup (p=0.014).Table 1.Comparison of patients with different BMI z-scores at time of inclusion in the cohortZ-Score ≤ -2N=33Z-Score > -2 to < 2N=177P valueGottron Papules41%(13/32)25%(44/175)0.001Puffy Fingers13%(4/30)38%(61/159)0.049Sclerodactylie84%(27/32)73%(121/166)0.049Patient globaldisease damage50 (30 – 75)n=2730 (10 – 55)n=1330.014ConclusionIn our jSSc cohort, currently the largest of the world, we could not find any differences regarding major internal organ involvement in patients with lower BMI at time of inclusion in the cohort. Nevertheless, there is a significant difference in patient related outcomes regarding global organ damage between the two groups. The long-term prognosis of these patients should be addressed in future studies.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Juvenile systemic sclerosis (jSSc) is a rare disease in childhood. To date, no composite response index exists to assess treatment effect in jSSc patients. ACR CRISS score (probability of improvement ranging from 0 to 1 based on mRSS, FVC%, PtGA, MDGA and HAQ-DI) and revised ACR CRISS (rCRISS, proportion of patients who improve in ≥ 3/5 ACR CRISS core items by a certain percentage, e.g. 30%, except 5% for FVC) were developed by experts in the field as outcome measures in adult patients with SSc. In addition, the Ranked Composite Important Difference (RCID) score was recently introduced as anchor to the ACR CRISS. We aimed to study the applicability and performance of the ACR CRISS, rCRISS and RCID in a prospectively followed cohort of patients with diffuse cutaneous jSSc. Data from the international jSSc inceptions cohort were used for this analysis. The ACR CRISS, rCRISS and RCID were calculated between baseline and 12-months follow-up according to the scoring algorithms. Missing values in the core items were estimated by multiple imputation by chained equations. Here we aimed to determine the value of the response measures to detect clinically change defined by the anchor questions about change (much better or little better versus almost the same, little worse or much worse) in patients overall health due to scleroderma since the last visit provided by the treating physicians and parents or patients (aged > 12 years). We included 95 jSSc patients with diffuse cutaneous subtype with available baseline and 12-months visit. Seventy-nine percent were female, the mean age at enrollment was 13.0 (3.8) and the mean disease duration was 3.1 (2.8) years. Among 95 patients, 57% were treated with steroids, 47% with methotrexate, 27% with MMF and 3% with a biological at baseline. ACR CRISS showed a ceiling effect (>.998) in 51% and a floor effect (<0.005) in 26% of patients. Patients who reported at least moderate improvement had a median ACR CRISS of 0.99 and in mean 2.6 (1.3) core items that improved by ≥20% from baseline to 12-months follow-up. The rCRISS 20/30/50 responses were 59%/49%/33% in patients who reported improvement (table 1) and 25%/25%/8% in patients with worsening. The RCID was approximately normal distributed (mean 20.7, SD 43.4). Mean (SD) RCID for patients who reported worsening was -10.5 (38.6) vs RCID of 20.7 (45.2) for patients who reported improvement. RCID scores for physician reported anchors of worsening or improvement were 6.5 (44.2) and 18 (45.4), respectively. The concordance between a positive RCID score and rCRISS 20/30 was moderate (rCRISS 20 and RCID, 43%, kappa=0.43; rCRISS 30 and RCID, 38%, kappa=0.36). Our data confirmed the presence of a ceiling and floor effect of ACR CRISS as shown in studies of adult SSc patients. The CRISS, rCRISS and RCID response distinguished between patients who rated their disease course since last visit as worsened or improved. Future studies should focus on the determination of specific pediatric weights for the CRISS and RCID components rather than extrapolation from adult SSc. In general, the RCID offers a meaningful tool in order to determine response to therapy in future clinical trials in jSSc patients. NIL. NIL. None Declared. Table 1ACR CRISS, rCRISS and RCID score by patients and physicians ratings about scleroderma disease courseWorsening/ no improvement reported by patients(n=12)Improvement reported by patients(n=49)P valueWorsening/ no improvement reported by physicians(n=14)Improvement reported by physicians(n=50)P valueMedian ACR CRISS score (IQR)0.0 (0 to 0.75)0.99 (0 to 1.0)0.0070.35 (0 to 0.99)0.99 (0 to 1.0)0.037rCRISS response 20%3(25%)29 (59%)0.0345 (36%)29 (58%)0.140rCRISS response 30%3 (25%)24 (49%)0.1342 (14%)27 (54%)0.008rCRISS response 50%1 (8%)16 (33%)0.0920 (0%)18 (36%)0.008Mean RCID score (SD)-10.5 (38.6)20.7 (45.2)0.0316.5 (44.2)18 (45.4)0.411CRISS = Composite Response Index in Systemic Sclerosis; RCID=Ranked Compsoite Important Difference; rCRISS = revised Composite Response Index in Systemic Sclerosis; SD = standard deviation

The aim of this study was to determine the risk factors, genotype-specific prevalence, and concordance of human papillomavirus (HPV) infections at three anatomical sites in a cohort of high-risk Greek men. Patients were recruited from sexually transmitted infection and HIV clinics in Athens. Samples were obtained from oral, penile, and anal sites of 294 study participants and HPV testing was performed on 882 samples using next-generation sequencing. Patients also completed a questionnaire assessing risk factors for infection. The mean age of the participants was 33.1, 30% identified as men who have sex with men (MSM), and 21% were HIV positive. The prevalence of HPV was 49%; it was the highest at anal sites (33%) compared with 23% at penile sites ( P =0.008) and 4% at oral sites ( P <0.001). The most common HPV types in order of frequency were 6, 44, 16, 53, and 89. The genotype concordance rate was the highest between the penile and anal sites (7%), followed by 2% for anal–oral concordance. Identifying as MSM [adjusted odds ratios (aOR)=6.75, P <0.001] and being HIV positive (aOR=2.89, P =0.026) were significant risk factors for anal HPV infection, whereas alcohol use (aOR=0.45, P =0.002) was associated negatively with infection. The only significant risk factor for oral infection was an older age of sexual debut (aOR=1.32, P =0.038). Nearly half of our study participants tested positive in at least one of three anatomical sites. Using next-generation sequencing, we could identify high-risk types that are not covered by the current vaccine and would be missed by traditional HPV testing kits.

BackgroundJuvenile systemic scleroderma (jSSc) is an orphan disease with an estimated prevalence of around 3 per 1 000 000 children. There are no studies which evaluated prospectively the patient related outcomes in these patients. We report the data from juvenile scleroderma inception cohort (jSSc) regarding organ involvement and patient related outcomes.MethodsThe jSSc is a prospective cohort of jSSc patients. Patients were enrolled who were diagnosed with jSSc, had a jSSc onset age under 16 years and were younger as age of 18 years at the time of inclusion. The patients are prospectively assessed every 6 months according to a standardised protocol. Patients with available 12 months follow up data were included in the analyses.ResultsCurrently 100 patients are followed in the jSSc cohort. 51 of them had available 12 months follow up data. Among those patients 37 (72.5%) had diffuse and 14 (27.5%) limited subtype. Mean age of onset of disease was 9.5 (±4.1) years and the mean disease duration at time of inclusion was 3.1 years (±3.2). The proportion of patients treated with DMARD increased from 74.5% to 88% at 12 months follow up. 86% were ANA positive at both assessments. Anti-scl70 positivity increased from 38% to 42%. Anticentromere antibody positivity was 2.4% at both assessments. Mean modified skin score decreased from 17.7 to 14.3 (p=0.151) Raynaud phenomenon occurred in 86% at enrolment and increased up to 88% at 12 months follow up. Nailfold capillary changes occurred around 70% at both assessments, but number of patients with active ulceration decreased from 28% to 16% (p=0.148). The number of patients with decreased FVC (FVC under 80%) decreased from 40.5% to 32% (p=0.497). The number of patients with pulmonary hypertension remained around 10%. No renal crisis or hypertension were reported. The gastrointestinal involvement was around 40% at both assessments. The number of patients with swollen joints decreased from 24% to 10% (p=0.06). The number of patients with muscle weakness decreased significantly from 33% to 9% (p=0.016), parallel to the number of patients with elevated CK values which decreased from 27% to 12% (p=0.074). All patient related outcomes, like global disease activity (p=0.048), global disease damage (p=0.05), Raynaud activity (p=0.003) and ulceration activity (p=0.001) improved significantly over 12 months. Physician assessed global disease activity (p=0.003) and ulceration activity (p=0.001) also improved significantly.ConclusionsOur data show, that jSSc patients over a 12 months disease course stayed quite stable or improved regarding organ involvement. But patient and physician related outcomes regarding activity assessment improved significantly.Disclosure of InterestNone declared