Objectives:Clinical trial data are lacking for treatment of patients with juvenile systemic sclerosis (jSSc). Three published recommendations exist for jSSc but real-world data on treatment patterns are lacking. The aim of this study was to analyse treatments used in the jSSc inception cohort (jSSci) and compare to published recommendations on the treatment of jSSc. Methods:Data was extracted for patients with 24 months follow-up visits in the jSSci up until June 2023. Medications used and their association with clinical characteristics were analysed. Logistic regression analyses were performed to compare treatments between limited and diffuse cutaneous jSSc subtypes, organ involvement and time of initiation of treatment. Multilevel mixed effects logistic regression analyses were used to evaluate the change in medication use in follow-up. Treatment patterns were compared against published recommendations. Results:93 patients had 24 months follow-up data. 77% of patients were receiving disease-modifying treatment (DMARD) at enrolment, which increased to 91% at 24 months (p<0.001). Patients with diffuse cutaneous jSSc subtype had significantly more frequently active ulcerations, skin involvement and received any kind of treatment more often compared with limited (97% vs 91%, p=0.047). Methotrexate was used in 52% of patients at enrolment which decreased to 37% at 24 months (p=0.001). Mycophenolate mofetil use increased from 24% to 46% (p<0.001). Biological DMARDs increased from 5% to 22% (p<0.001). The treatment pattern strongly overlapped with the published paediatric guidance. Conclusion:This is the first report regarding the pattern of medication use in real life in the currently largest patient cohort of patients with jSSc. The observed pattern overlaps with the published recommendations.

The aim of this study was to describe the clinical and molecular genetic findings in seven individuals from three unrelated families with Blau syndrome. A complex ophthalmic and general health examination including diagnostic imaging was performed. The NOD2 mutational hot spot located in exon 4 was Sanger sequenced in all three probands. Two individuals also underwent autoinflammatory disorder gene panel screening, and in one subject, exome sequencing was performed. Blau syndrome presenting as uveitis, skin rush or arthritis was diagnosed in four cases from three families. In two individuals from one family, only camptodactyly was noted, while another member had camptodactyly in combination with non-active uveitis and angioid streaks. One proband developed two attacks of meningoencephalitis attributed to presumed neurosarcoidosis, which is a rare finding in Blau syndrome. The probands from families 1 and 2 carried pathogenic variants in NOD2 (NM_022162.3): c.1001G>A p.(Arg334Gln) and c.1000C>T p.(Arg334Trp), respectively. In family 3, two variants of unknown significance in a heterozygous state were found: c.1412G>T p.(Arg471Leu) in NOD2 and c.928C>T p.(Arg310*) in NLRC4 (NM_001199139.1). In conclusion, Blau syndrome is a phenotypically highly variable, and there is a need to raise awareness about all clinical manifestations, including neurosarcoidosis. Variants of unknown significance pose a significant challenge regarding their contribution to etiopathogenesis of autoinflammatory diseases.

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 Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects data prospectively in jSSc, allowing the evaluation of the development of organ involvement and patient and physician reported outcomes in jSSc over time. Objectives To review the changes in the clinical characteristics, and patient and physician reported outcomes, over a 24 month observation period from the time of inclusion into the cohort. Methods The jSScC cohort enrolls 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. We reviewed jSScC patient clinical data and patient and physician reported outcomes, who had 24 months follow up from the time of inclusion until 1st of December 2022. Results We extracted data from 90 patients, 77% of them had the diffuse subtype. The female/male ratio was 3.5:1. Median age of onset of Raynaud´s was 9.4 years and the median age of onset of non-Raynaud´s was 10.0 years. Eighty-nine percent of the patients were treated with disease modifying anti-rheumatic drugs (DMARDs) at time of inclusion in the cohort (T0) and 96% after 24 months (T24). Median disease duration was 2.4 years at T0. No patient died during the follow up. Antibody profile stayed unchanged. Only 3 clinical parameters changed and all improved significantly, the median modified Rodnan skin score improved from 11 to 8 (p=0.021), number of patients with joints with pain on motion decreased from 21% to 10% (p=0.04) and the number of patients with muscle weakness decreased from 13% to 4% (p=0.03). All other organ involvement did not show any statistically significant change from T0 to T24. All collected patient reported outcomes improved significantly from T0 to T24: the patient reported disease activity by VAS 0-100 from 40 to 20 (p=0.001), the patient reported disease damage by VAS 0-100 from 35 to 20 (p=0.027), patient reported ulceration activity by VAS 0-100) from 8 to 0 (p=0.001) and the patient reported Raynaud activity by VAS 0-100 from 20 to 10 (p=0.002). Two of the three physician reported outcomes improved significantly, the physician global disease activity by VAS 0-100 from 30 to 20 (p=0.001) and physician reported ulceration activity by VAS 0-100 from 5 to 0 (p=0.017). (Table 1.) Conclusion Skin and musculoskeletal clinical features improved over 24 months, with almost all patients on DMARDs, supporting the response of these features to therapy. It was promising that internal organ involvement, like cardiac, lung and gastrointestinal, did not significantly worsen or increase. The most striking observation is the positive direction and improvement all patients and two of the three physician reported outcome measures over 24 months in this large international cohort. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests None Declared.

After 24 Months Observation Period the Patients Related Outcomes Improve Significantly in the Juvenile Scleroderma Inceptions Cohorte

Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.Methods: The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians' and the patients' global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models.Results: Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity.Conclusion: Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.

OBJECTIVES:Assessment of active synovitis is crucial for the management of juvenile idiopathic arthritis (JIA). We aimed to investigate the correlation of musculoskeletal ultrasound (MSUS) and clinical examination results and relate them to arthritis relapse rate.METHODS:JIA patients with questionable presence of active arthritis (Q-joints) and controls (JIA and healthy children) were recruited. MSUS of Q-joints, active joints and their inactive counterparts was performed at study entry. Standard disease activity parameters were prospectively recorded.RESULTS:Of 481 joints of 138 JIA patients, 99 joints (20.6%) of 58 patients had one or more Q-joints with 54/99 (54.5%) having MSUS features of active disease. Clinically inactive joints had lower proportion of MSUS synovitis (78/253, 30.8%) while MSUS activity was present in 114/129 (88.4%) of clinically active joints and in 2/105 (1.9%) joints of 36 healthy controls. Within the 15-month follow-up 23/99 (22%) Q-joints and 31/253 (12%) clinically inactive joints relapsed. Joints with subclinical synovitis relapsed more frequently than MSUS inactive ones (p<0.001). The relapse rate was higher in MSUS-active Q-joints (19/23, 82%) than in clinically inactive ones (16/31, 52%) with MSUS synovial hypertrophy as the main relapse predictor in multivariate analysis. Ankle and knee joints relapsed most frequently.CONCLUSIONS:Acknowledgement of joints with questionable synovitis may contribute to the assessment of disease activity in JIA. Presence of MSUS synovitis carries a clinically meaningful risk of disease recurrence in these joints. In clinical practice, our findings encourage timely MSUS assessment of the joints in question, especially in patients without any other features of active disease.

Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. Conclusion: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.

Background 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. Objectives 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. Methods 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). Results 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). Conclusion 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. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests 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

OBJECTIVE:Juvenile systemic sclerosis (SSc) is an orphan disease, associated with high morbidity and mortality. New treatment strategies are much needed, but clearly defining appropriate outcomes is necessary if successful therapies are to be developed. Our objective here was to propose such outcomes.METHODS:This proposal is the result of 4 face-to-face consensus meetings with a 27-member multidisciplinary team of pediatric rheumatologists, adult rheumatologists, dermatologists, pediatric cardiologists, pulmonologists, gastroenterologists, a statistician, and patients. Throughout the process, we reviewed the existing adult data in this field, the more limited pediatric literature for juvenile SSc outcomes, and data from 2 juvenile SSc patient cohorts to assist in making informed, data-driven decisions. The use of items for each domain as an outcome measure in an open label 12-month clinical trial of juvenile SSc was voted and agreed upon using a nominal group technique.RESULTS:After voting, the domains agreed on were global disease activity, skin, Raynaud's phenomenon, digital ulcers, musculoskeletal, cardiac, pulmonary, renal, and gastrointestinal involvement, and quality of life. Fourteen outcome measures had 100% agreement, 1 item had 91% agreement, and 1 item had 86% agreement. The domains of biomarkers and growth/development were moved to the research agenda.CONCLUSION:We reached consensus on multiple domains and items that should be assessed in an open label, 12-month clinical juvenile SSc trial as well as a research agenda for future development.

BackgroundJuvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently no medications are licensed for the treatment of jSSc. Due to its rarity, only recently have the first management and treatment guidelines been published, the jSSc SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) recommendations, reflecting consensus opinion upon pediatric rheumatologists (1).ObjectivesTo better understand treatment practices internationally for jSSc, both at baseline and over 24 months observation period and to compare if real world therapies are congruent with the recent SHARE recommendations.MethodsThe juvenile systemic sclerosis inceptions cohort (jSScC) is a multinational cohort that prospectively collects clinical data, including medications at baseline and subsequent visits. The jSScC enrollment criteria include age of onset of the first non-Raynaud symptom younger than 16 years and age younger than 18 years at cohort entrance. The frequency of medications (general category and specific medication) was calculated across the cohort at timepoint 0 (enrollment), 12 months and 24 months.ResultsWe extracted data from the jSScC of patients who were followed for 12 or 24 months. 109 patients were followed at time point 0 (T0) and 12 months (T12), and data was available for 77 of them up at 24 months (T24). The mean age of the patients was 13.2 years at the timepoint 0. 77% were female and 75% had diffuse subtype. Disease duration at baseline visit was 3.1 years. The medications the patients were on recorded by the physician were captured at T0, T12 and T24 listed in Table 1.Table 1.MEDICATIONSTime point 0N=109T12 monthsN=109T24 months N=77Any Medication92% (100)97% (106)97% (75)Vascular medications Endothelial receptor antagonist16% (17)24% (26)21% (16) PDE-5-Blocker5% (5)8% (9)9% (7)ImmunomodulatorsCorticosteroids52% (57)44% (48)44% (21)All csDMARDs:81% (88)93% (101)92% (71) csDMARDs monotherapy61% (67)66% (72)60% (46) csDMARDs combination therapy17% (18)15% (16)14% (11) Methotrexate51% (56)50% (55)39% (30) Mycophenolate Mofetil26% (28)44% (48)47% (36) Hydroxychloriquine11% (12)15% (16)21% (16) Cyclophosphamide12% (13)2% (2)1% (1) Azathioprine2% (2)2% (2)3% (2)All bDMARDs:5% (5)14% (15)18% (14) bDMARDs monotherapy2%(2)2%(2)1% (1) bDMARDs combined with csDMARDs3% (3)12% (13)17% (13) Tocilizumab2% (2)10% (11)14% (11) Rituximab2% (2)4% (4)4% (3) Adalimumab1% (1)0% (0)0% (0)Autologous Stem cell transplantation0% (0)1% (1)0% (0)csDMARDs: Conventional synthetic disease-modifying antirheumatic drugsb DMARDs: Biological disease-modifying antirheumatic drugsConclusionAt baseline half of the patients were on corticosteroids. This is more frequent than typical adult SSc practice but coincides with jSSc SHARE treatment recommendations (#1). After 12 months observation in the cohort over 90% of patients received a DMARD therapy. Methotrexate and mycophenolate mofetil were the most commonly prescribed DMARDs, which also reflects the SHARE treatment recommendations (#2, #3). At 12 months the use of glucocorticoid decreased and the use of bDMARDs increased. In general, biological DMARDs are typically considered in severe or refractory (SHARE recommendation #7), reflecting the lower percentage compared to csDMARDs. Autologous stem cell transplantation was observed in one patient at 12 months, reflecting an option in jSSc with progressive and refractory disease (SHARE recommendation #8). Endothelial receptor antagonists, such as bosentan, were used over time in approximately 20% of the patients, reflecting SHARE recommendation #6 for pulmonary hypertension and/or digital tip ulcers. This is the first evaluation looking at clinical medication practice pattern in jSSc, and its comparison to recently published consensus guidelines.References[1]Foeldvari I, Culpo R, Sperotto F et al. Consensus-based recommendations for the management of juvenile systemic sclerosis. Rheumatology (Oxford). 2021;60(4):1651-8.Disclosure of InterestsNone declared

Abstract Introduction Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients there are significant differences between the clinical presentation of diffuse and limited subtypes. We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort (jSSc). Objectives To study the clinical presentation of jSSc patients with diffuse (djSSc) and limited (ljSSc) subtypes. Methods We reviewed the baseline clinical characteristics of the patients, who were recruited to the jSScC till December 2022. 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. Results The JSScC included 232 patients, 68% (n = 159) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3–12.9) and the median age at the first non-Raynaud symptom was 10.9 years (7.3–13.0). Median disease duration was 2.5 years (1.0–4.6). The female/male ratio was significantly lower in the djSSc subtype (3:1 vs 5:1, P < 0.001). Antibody profile was similar, with the exception of a significantly higher number of anticentromere positive patients in the ljSSc (10% vs 2%, P = 0.02). Decreased FVC < 80% was found in ∼30% and decreased DLCO < 80% was found in around 40% in both subtypes. Abnormal HRCT findings were found in 44% of patients. Pulmonary hypertension assessed by ultrasound was identified in ∼5% in both groups. Gastrointestinal involvement occurred in 43% of djSSc and 36% in ljSSc (P = 0.303). Patients with diffuse subtype had significantly higher modified Rodnan Skin Score (16 vs 4, P = 0.001), more frequently sclerodactyly (85% vs 55%, P < 0.001), a history of digital ulceration (62% vs 30%, P < 0.001), active ulceration (21% vs 8%, P = 0.021), telangiectasia (44% vs 22%, P = 0.002), a decreased Body Mass Index z scores ≤ -2 (20% vs 6%, P = 0.008) and decreased joint range of motion (64% vs 48%, P = 0.022). Patients with ljSSc had significantly higher rate of cardiac involvement (11% vs 3%, P = 0.007). Regarding patient related outcomes djSSc patients had more severe disease with respect to patient reported global disease activity by VAS 0–100 (40 vs 30, P = 0.024), patient reported global disease damage by VAS 0–100 (40 vs 25, P = 0.001), patient reported assessment of ulceration activity by VAS 0–100 (10 vs 0, P = 0.001) and patient reported Raynaud activity by VAS 0–100 (30 vs 15, P = 0.001). Regarding physician related outcomes the physician reported global disease activity by VAS 0–100 (30 vs 20, P = 0.001), physician reported global disease damage by VAS 0–100 (30 vs 20, P = 0.004), and physician assessed ulceration activity by VAS 0–100 (5 vs 0, P = 0.018) were significantly higher in djSSc. Conclusion In the largest jSSc cohort in the world, djSSc patients have a significantly more severe disease. Patients and physician related outcomes were significantly more severe in djSSc group. Interestingly, we found no differences regarding interstitial lung disease, pulmonary hypertension or gastrointestinal involvement, although the number of patients with decreased BMI < -2 z scores was significantly higher in the djSSc patients. Ethics Our work is approved by the IRB of the Ärztekammer Hamburg