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Background: Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status. Objectives: To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc). Methods: The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed. Results: Among 3240 registered patients, 10% were diagnosed as SSc-overlap syndrome. Of these, 82.5% were female. SSc-overlap patients had a mean age of 48±1.2 years and carried significantly more often ‘other antibodies’ (68.0%; p

Background To improve detection and follow-up of patients with systemic sclerosis, the German Network for Systemic Scleroderma (DNSS) was founded 2003 and comprise rheumatologists, dermatologists, pulmonologists and nephrologists from more than 40 medical centers. Renal crisis is rare but still a medical emergency in patients with SSc. Objectives Up to date, more than 3000 patients have been grouped into four descriptive disease subsets, i.e. limited cutaneous disease (lcSSc), diffuse cutaneous disease (dcSSc), overlap-syndrome and undifferentiated connective tissue disease (UCTD) with scleroderma features. Methods In this analysis, we have focused on renal crisis within the three main subsets, e.g. lcSSc, dcSSc and overlap-syndromes to identify baseline aspects, which are predictive for future SSc associated renal crisis. Results Recent analyses of up to now 3180 patients revealed that 56% of patients suffer from limited SSc (lcSSc), 34% from diffuse SSc (dcSSc) and 11% of patients were diagnosed with an overlap-syndrome. Eighteen patients developed a renal crisis (1.4%, 18/3180), while 10% (315/3180) were classified with kidney involvement and 8% (257/3180) with proteinuria. Of these, 66.7% (12/18) were diagnosed with the diffuse form of SSc, while just 27.8% (5/18) were diagnosed with lcSSc and 5.6% (1/18) with SSc-overlap syndromes. Predictive factors for renal crisis in our patient cohort included the diffuse form of SSc (odds ratio (OR) 4.6, p=0.005, 95%>confidence interval (CI) 1.6-13.5), a modified Rodnan skin score (mRSS) of more than 15 (OR 4.7; p=0.002, 95%>CI 1.7-13), positive anti-RNA polymerase (RNAP) autoantibodies (OR 24.6, p CI 6.1-99.5), tendon friction rubs (OR 5.4, p=0.004, 95%>CI 1.7-16.9), hypertension (OR 6.1, p CI 2.3-16.5), proteinuria (OR 11.8, p CI 4.3-32.1) and elevated CK-levels (OR 5.1, p=0.01, 95%>CI 1.4-18.9). Interestingly, positive anti-topoisomerase autoantibodies did not predict a higher risk for renal crisis. Patients diagnosed with renal crisis were significantly more frequent on ACE-inhibitors (61.1%, 11/18, p=0.001). Of these, 5 patients also suffered from proteinuria and 7 patients from hypertension. Patients on systemic glucocorticoids had also an increased risk to develop a renal crisis (OR 5.1, p=0.002, 95%>CI 1.8-14.3), independent of the dosage (> or Conclusions Renal crisis has become a rare complication in SSc. The highest risk was associated with the detection of RNAP antibodies, followed by proteinuria, hypertension, tendon friction rubs, elevated CK-levels and a modified Rodnan skin Score above 15. Close monitoring of patients at high risk for SSc associated renal crisis is mandatory. Disclosure of Interest None declared

Background Vasculopathy is a major pathophysiological problem in patients with systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcerations (DU), pulmonary arterial hypertension (PAH). It is not known, how SSc patients are treated with vasoactive agents in daily practice. Objectives To determine, to which extent SSc patients are treated with vasoactive drugs. Methods Data of 3248 patients of the registry of German Network for Systemic Scleroderma were analyzed. Results Patients were treated with vasoactive drugs in 54.9% (1784) cases. Of these, 53% received calcium channel inhibitors (CCI), followed by 23.5% treated with intravenous Prostanoids (Iloprost, Prostavasin), 11.8% with beta-blockers, 11.3% with Pentoxifylline, 9.7% with Endothelin 1 receptor antagonists (ETRA), 4.6% with PDE5 inhibitors, 2.7% with topical vasodilative agents and 3.2% with others. Patients with RP received vasoactive therapy in 47,4%, with DU in 66,8% and with PAH in 71,6% of cases. Logistic regression analysis revealed, that patients who suffered from PAH were significantly more often treated with PDE5 inhibitors (odds ratio (OR) 7.2; p When patients, registered after 2009, were compared to patients registered prior to 2004, it was found that after 2009 significantly more patients received ETRA (12% vs 5.5%; p Conclusions These data clearly indicate that many SSc patients with RP, DU, and PAH do not yet receive sufficient vasoactive therapy. Furthermore, in recent years a marked change of treatment regimens can be observed. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4946

Background Digital ulcers (DU) constitute an important component of disease burden in SSc. Among patients with SSc it has been reported that 15 to 25 % have active digital ulcers and 35 % have or have had DUs in the past. The natural history, including risk as well as protective factors for the development of DU are only partially understood. Objectives To identify new clinical parameters as risk factors for the development of DU. Methods In a prospective study, patients with definite SSc were included in three groups: i) group 1 for patients with active DU; ii) group 2 for patients, who have no active DU at baseline, but had reliably documented SSc-related DU in the past; iii) group 3 for patients, who neither have active DU at inclusion nor had DU in their past. Patients in group 3 are followed on months 6, 12, 24 for the development of DU. Results 87 patients were included in group 1, 148 patients in group 2. 420 patients were recruited for group 3. Mean disease duration at enrolment as determined by first non-Raynaud organ involvement was 10.6 years in group 1, 2 and 8.8 in group 3. Patients in group 3 with no history of DU were significantly (p KI (0,014;0,952)) and >6 years ((OR = 0,337, 95%>KI (0,121;0,943)). Conclusions In addition to well known risk factors for DU, including diffuse disease, high mRSS, Scl-70 antibody, this study indicates that a) a substantial number of SSc patients do not develop DU and b) disease duration is inversely correlated with the risk to develop DU for the first time. Disclosure of Interest P. Moinzadeh Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., N. Hunzelmann Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., U. Mueller-Ladner Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., F. Meier Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., G. Riemekasten Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., M. Becker Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., A. Kreuter Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., G. Wozel Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., I. Melchers Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., M. Sardy Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., I. Herrgott Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., K. Graefenstein Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., G. Fierlbeck Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., C. Pfeiffer Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., M. Worm Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., H. Burkhardt Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., H. Mensing Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., K. Kuhr Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., C. Sunderkoetter Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany., T. Krieg Grant/research support from: This study was financially supported by Actelion Pharmaceuticals Germany.

Background To improve detection and follow-up of patients with systemic sclerosis, the German Network for Systemic Scleroderma (DNSS) was founded and initiated a registry gathering information on diagnosis, clinical symptoms and therapy of SSc patients. Methods Up to date, more than 3000 patients have been grouped into four descriptive disease subsets, i.e. limited cutaneous disease (lcSSc), diffuse cutaneous disease (dcSSc), overlap-syndrome and undifferentiated connective tissue disease (UCTD) with scleroderma features. Disease progress between initial patient registration (year 0) and fourth follow-up (year 4) was measured using organ involvement and present symptoms as indicators. Results Recent analyses revealed that 49% of patients suffer from limited SSc (lSSc), 31% from diffuse SSc (dSSc) and 10% of patients were diagnosed with an overlap-syndrome. 8% had an undifferentiated form while Scleroderma sine scleroderma was present in 0.7% of patients. Follow-up data are available from 1595 patients after one year, from 901 patients followed for two years, 573 patients followed for three years and 386 followed for at least four years. After four years a significant increase was detected in the frequency of pulmonary hypertension (PAH) (14.4% to 24.2%, p=0.001), lung fibrosis (38.8% to 47.6%, p=0.006), oesophagus involvement (58.5% to 74.8%, p=0.0001), bowel involvement (13.6% to 19.9%, p=0.03), Kidney (10.7% to 15.8%, p=0.03) and Heart (13.4% to 24.0%, p Disease subsets were associated with different organs being more frequently involved, i.e. lcSSc with PAH (13.5% to 25.0%, p=0.007), oesophagus (60.7% to 77.0%, p=0.0001) and colon involvement (7.3% to 11.6%, p=0.002); dcSSc with PAH (19.0% vs. 25.9%, p=0.035), lung fibrosis (63.5% vs. 71.4%, p=0.02) and heart involvement (19.1% vs. 27.7%, p=0.007). Interestingly, patients with overlap syndrome showed no significant change in organ involvement over time. Conclusions After four years of follow-up in a large well defined SSc cohort, a significant increase in frequency of organ involvement was observed, particularly with respect to the lung, GI-tract and heart. Patients with SSc should be followed at least annually for organ involvement, the frequency of follow-ups being increased depending on the disease activity. Disclosure of Interest None Declared