E. Bonfa

Prevalence of sarcopenia and associated risk factors by two diagnostic criteria in community-dwelling older men: the São Paulo Ageing & Health Study (SPAH)

SummarySarcopenia is an aging syndrome that can be characterized by many criteria adjusted or not by fat mass. This study suggested that the optimal criteria should be selected according to body mass index (BMI) in older men and identified age, BMI, race, smoking, physical activity, hip bone mineral density (BMD) as risk factors for this syndrome.IntroductionThis study aims to analyze the prevalence of sarcopenia and associated risk factors using appendicular skeletal mass (ASM)/height2 and ASM adjusted for total fat mass criteria in older men from community.MethodsThree hundred ninety-nine men were included and answered a questionnaire about lifestyle and medical history. Individuals were classified by their BMI using the classification adjusted by age. Body composition and bone mineral density were measured by dual X-ray absorptiometry. Sarcopenia was classified according to both criteria. Logistic regression models were used to analyze risk factors associated with sarcopenia.ResultsThe mean BMI was 26.46xa0kg/m2: 12.5xa0% underweight, 43.6xa0% normal, and 43.9xa0% overweight/obese. Fifty-four (13.5xa0%) were considered sarcopenic by ASM/height2 and 79 (19.8xa0%) by ASM adjusted for fat (pu2009=u20090.001). Fifty-one (12.8xa0%) individuals had discordant sarcopenia classification: 13 were classified only by ASM/height2 and 38 only by ASM adjusted for fat. Of the 13 subjects classified as sarcopenic only by ASM/height2, 84.6xa0% (11/13) were underweight and solely one (7.7xa0%) was considered overweight/obese. In contrast, of those 38 older men classified as sarcopenic only by ASM adjusted for fat, none were underweight and 53xa0% (20/38) were overweight/obese. Subjects classified as sarcopenic according to both criteria had the same risk factors in the final model analyses (age, BMI, race, smoking, physical activity, hip BMD; pu2009<u20090.05).ConclusionThis study suggested that the optimal criteria for sarcopenia should be selected according to BMI in community-dwelling older men.

128 Outcomes of 847 childhood-onset systemic lupus erythematousus patients in three age groups

Background and aims To assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease(<6 years), group B school age(≥6and<12 years) and group C adolescent(≥12and<18 years). Methods Observational cohort study of 10 Paediatric Rheumatology centres, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was higher in group A compared to groups B and C[8.3 (0.1–23.4)u2009vs. 6.2 (0–17)u2009vs. 3.3 (0–14.6) years, p<0.0001]. The median SLICC/ACR-DI[0 (0–9)u2009vs. 0 (0–6)u2009vs. 0 (0–7), p=0.065] was comparable in all groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric(21%u2009vs. 10%u2009vs. 7%, p=0.007), skin (10%u2009vs. 1%u2009vs. 3%, p=0.002) and peripheral vascular involvements(5%u2009vs. 3%u2009vs. 0.3%, p=0.008) were more frequent in group A compared to B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia and autoimmune hemolytic anaemia were similar in all groups(p>0.05). Mortality rate was higher in group A compared to groups B and C(15%u2009vs. 10%u2009vs. 6%, p=0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes, with higher mortality rate and neuropsychiatric/vascular/skin organ damages in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

129 Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

Background and Aims To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus(cSLE) patients with and without panniculitis. Methods Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p<0.004). Results Panniculitis was observed in 6/847 (0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalised distribution was evidenced in 3/6 and localised in upper limbs in 2/6 and face in 1/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75 (0–11.4)u2009vs. 2.83 (0–11.8) years, p=0.297], showed higher frequencies of constitutional involvement (67%u2009vs. 10%,p=0.003), leukopenia (67%u2009vs. 7%, p=0.002) and median C-reactive protein (10.5u2009vs. 0.5u2009mg/L, p=0.001). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.

SAT0253 Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study

Background Evans syndrome (ES) is an uncommon manifestation characterized by autoimmune destruction of red cells and platelets and concomitant or sequential appearance of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). This involvement has been associated to severe disease activity in adult patients with systemic lupus erythematosus (SLE), particularly at disease onset. However, ES studies in childhood-onset SLE (cSLE) patients have been rarely reported and limited to small populations. Objectives The objective of the present multicenter study was to assess ES in a large cSLE cohort at diagnosis evaluating prevalence, clinical features, laboratory findings and outcomes. Methods A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE (ACR criteria). None of them had secondary etiologies of autoimmune cytopenias, such as infections, primary immunodeficiencies and malignancies. Patients were divided in two groups for the assessment of lupus manifestations, laboratory exams and treatment at cSLE diagnosis: patients with ES and patients without ES. Results ES was observed in 11/850 (1.3%) cSLE patients at diagnosis. The majority of them had hemorrhagic manifestations (58%) and active disease (82%). All patients with ES were hospitalized and none of them died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multi-organ involvement, autoantibody profile and low complement levels (p>0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, p=0.003) and musculoskeletal involvement (18% vs. 69%, p=0.001) than those without this complication. The median of hemoglobin [7.4 (5.4–9.4) vs. 10.3 (3.5–16.4)g/dL, p<0.001] and platelets [27 (15–54) vs. 231 (2–761)x103/mm3, p=0.005] were significantly lower in ES compared to non-ES patients, whereas lymphocytes were significantly higher in ES patients [1.8 (1–2.38) vs. 1.16 (0.07–7)x103/mm3, p<0.001]. The frequencies of intravenous methylprednisolone (82% vs. 43%, p=0.013) and intravenous immunoglobulin use (64% vs. 3%, p<0.0001) were significantly higher in the former group. Current prednisone dose between the two groups was similar [1.1 (0.76–1.5) vs. 1.0 mg/kg/day (0–30), 0.195]. Conclusions Our large multicenter study identified that ES was a rare and severe cSLE manifestation with a difficult diagnosis due to the absence of typical lupus manifestations, often requiring hospitalization and intravenous treatment. Disclosure of Interest G. Lube: None declared, M. Ferriani: None declared, L. Campos: None declared, M. Terreri: None declared, E. Bonfá: None declared, C. Magalhães: None declared, N. Aikawa: None declared, D. Piotto: None declared, O. Peracchi: None declared, M. C. Santos: None declared, S. Appenzeller: None declared, V. Ferriani: None declared, R. Pereira: None declared, C. Silva Grant/research support from: Conselho Nacional de Desenvolvimento Científico e Tecnolόgico (CNPq 302724/2011–7 to CAS), Federico Foundation (to CAS) and by Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS

THU0517 Invasive Fungal Infections Survey in 852 Childhood-Onset Systemic Lupus Erythematosus: A Multicenter Cohort

Background The majority of infections in childhood-onset systemic lupus erythematosus (cSLE) patients are caused by virus and bacteria, and less frequently by opportunistic agents, such as fungi.1 However, studies evaluating solely invasive fungal infections (IFI) in cSLE patients are restricted to case reports or case series1 without any systematic evaluation of the possible associated risk factors and outcomes in pediatric lupus population. Objectives To study the prevalence, risk factors and mortality of IFI in cSLE patients. Methods A retrospective multicenter cohort study was performed in 852 cSLE patients from 10 Pediatric Rheumatology services of São Paulo state, Brazil. An investigator meeting was held and all participants received database training. IFI were diagnosed according to EORTC/MSG Consensus Group (proven, probable and possible).2 Demographic data, clinical, laboratorial, SLEDAI-2K, SLICC/ACR-DI, treatment and outcome were also evaluated. Results IFI were recorded in 33/852 (3.9%) cSLE patients. Proven IFI was evidenced in 22 cSLE patients, probable IFI in 5 and possible IFI in 6. The most frequent types of IFI were candidiasis (n=20) and aspergillosis (n=9). The median of disease duration was lower (1.0 vs. 4.7 years, p<0.0001), with a higher current SLEDAI-2K [19.5 (0-44) vs. 2 (0-45), p<0.0001] and current prednisone dose [50 (10-60) vs. 10 (2-90) mg/day, p<0.0001] in patients with IFI compared to those without IFI. The frequency of death was higher in the former group (51% vs. 6%, p<0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR=1.108; 95%CI=1.057-1.163; p<0.0001), current prednisone dose (OR=1.046; 95%CI=1.021-1.071; p<0.0001) and disease duration (OR=0.984; 95%CI=0.969-0.998; p=0.030) were independent risk factors for IFI (R2 Nagelkerke 0.425). Conclusions This was the first study that characterized IFI in a large cSLE population. We identified that disease activity and glucocorticoid use were the main risk factors for these life-threatening infections, mainly in early disease course and with a high rate of fatal outcome. References Silva MF, Ribeiro AS, Fiorot FJ, et al. Invasive aspergillosis: a severe infection in juvenile systemic lupus erythematosus patients. Lupus 2012;21:1011-6. De Pauw B, Walsh TJ, Donnelly JP, et al. Revised definitions of invasive fungal disease from the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group. Clin Infect Dis 2008;46:1813-21. Disclosure of Interest None declared

SAT0245 In VIVO Evidence of IL-17/IL-23 Axis Activation in Ankylosing Spondylitis Patients with Long-Term TNF Blockade: The Missing Therapy Target?

Background In spite of recent evidences regarding the relevance of IL-23/IL-17 axis in Ankylosing Spondylitis (AS) pathogenesis, there are no data on the long-term effect of anti-TNF therapy in this pathway and their possible correlation with treatment, clinical, laboratory and radiographic parameters. Objectives Investigate the influence of TNF-blockage in IL-23/IL-17 axis of AS patients. Methods Eighty-six AS patients, 47 with refractory active disease (BASDAI≥4) referred to anti-TNF therapy and 39 AS patients with BASDAI<4 (AS control group) were included. The AS active group was evaluated at baseline, 12 months and 24 months after TNF blockage and compared at baseline to the AS control group and to 47 healthy age- and gender-matched controls. Plasma levels of IL-17A, IL-22, TNFα, IL-23, PGE2 were measured. Radiographic severity and progression was assessed by mSASSS. Results At baseline, active AS patients presented higher IL-23 and PGE2 plasma levels compared to AS control group (p<0.001 and p=0.008) and to healthy controls (p<0.001 and p=0.02). After 24 months of TNF blockage, IL-23 and PGE2 remained elevated with higher levels compared to healthy group (p<0.001 and p=0.03) in spite of significant improvement in all clinical (ASDAS-CRP, BASFI, BASMI and ASQol, p<0.001) and inflammatory parameters (C-reactive protein and ESR, p<0.001). Further analysis of 27 anti-TNF treated patients that achieved good-response (ASDAS-CRP<2.1 with Δ≥1.1) at 24 months revealed that their IL-23 plasma levels were higher than healthy controls (p<0.001) and higher than 21 AS control group with similar disease activity (ASDAS-CRP<2.1),(p=0.01). No predictor for anti-TNF response or to radiographic progression was identified. In AS active group (n=47), there was a strong correlation between IL-23 and IL-17A at baseline (r=0.64, p<0.001), 12 months (r=0.47, p=0.001) and 24 months (r=0.61, p<0.001). IL-23 was also correlated with PGE2 at 12 months (r=0.45, p=0.001) and 24 months (r=0.33, p<0.05). No correlation was observed between NSAID, cytokines levels and radiographic progression (p>0.05). Conclusions This study provides novel data demonstrating that IL-23/IL-17 axis is not influenced by TNF blockage in AS patients despite clinical and inflammation improvement and NSAID intake. In this context, IL-23/IL-17 blockage emerges as potential additional target in AS patients. References Hreggvidsdottir HS, Noordenbos T, Baeten DL. Inflammatory pathways in spondyloarthritis. Mol Immunol 2014;57:28-37. Yeremenko N, Paramarta JE, Baeten D. The interleukin-23/interleukin-17 immune axis as a promising new target in the treatment of spondyloarthritis. Curr Opin Rheumatol 2014;26:361-370. Disclosure of Interest None declared

FRI0493 Clinical and Serological Comparative Analysis of Systemic Sclerosis with or without Overlap Syndromes in A Large Brazilian Cohort

Background There are scarce data comparing clinical and serological features in patients with systemic sclerosis (SSc) and SSc overlap syndromes (SSc-OS) due to the rarity of these associations. Objectives To analyze clinical and SSc-associated serological profiles including a panel of novel described antinucleolar antibodies (ANoA) in a large cohort of Brazilian patients with SSc and SSc overlap syndromes. Methods Three-hundred twenty-eight SSc patients classified according to the ACR/EULAR 2013 SSc criteria, attended at the Scleroderma Outpatient Clinic of a tertiary referral university hospital from 2000 to 2011 were enrolled in the study; 32% had diffuse cutaneous SSc and 68% limited cutaneous SSc. Clinical and demographic data were obtained from an electronic register database. Serum samples were analyzed for the presence of antinuclear antibodies (indirect immunofluorescence on HEp-2 cells), antibodies to Scl-70, PM-Scl, RNA-Pol III, CENP-A/CENP-B, and Ro/SS-A (52 and 60 kDa) (ELISA), and antinucleolar antibodies (ANoA) fibrillarin, Ku, Th/To and NOR90 (line blot immunoassay) using commercial available standardized kits. Results Two-hundred sixty eigth patients were classified as SSc and sixty patients as SSc-OS (14 with systemic lupus erythematosus, 11 with polymyositis, 7 with rheumatoid arthritis, and 35 with Sjogren Syndrome, isolated or combined). Both groups (SSc-O and SSc) were similar regarding mean age at onset, female predominance, disease duration and ethnicity (p>0.05). Concerning clinical features, there were no significant differences related to the occurrence of pitting scars, digital amputation, calcinosis, telangiectasia, interstitial lung disease, pulmonary hypertension, cardiovascular disease, renal crisis (p>0.05). Conversely, SSc-OS patients had lower mean modified Rodnan skin score (5.6±4.24 vs. 10±11.7, p<0,001),but higher percentage of joint (33 vs. 21%, p=0.04) and muscle involvement (22 vs. 8%, p=0.003) compared to the SSc group. There was no difference regarding the frequency of all autoantibodies tested in the two groups (p>0.05), except for the negative association among anti-RNA-Pol III with SSc-OS (p=0.011). Conclusions This study identified a comparable occurrence of major organ involvement and ischemic lesions in SSc-OS and SSc. This finding is strengthened by the observation of a similar frequency of all scleroderma autoantibodies in both groups and suggests that SSc determines the clinical manifestation in overlap syndromes in spite of a less extensive skin involvement in these patients. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5863

FRI0461 Subclinical Ovarian Dysfunction in Takayasu Arteritis Patients

Background Takayasu arteritis (TA) is a rare idiopathic systemic chronic vasculitis that involves large arteries. This disease occurs mainly in female gender during the reproductive age and ovarian reserve and future fertility are major topic of interest. Aging, surgery and hypothalamic-pituitary-gonad axis dysfunction have been found to influence the quantity and quality of primordial follicles in ovaries and, ultimately, the ovarian reserve. Other conditions such as autoimmune oophoritis and immunosuppressive drugs, particularly cyclophosphamide, may also result in diminished ovarian reserve. Female TA patients are susceptible to these factors but there is no systematic study assessing this ovary abnormality in these patients. Objectives To assess ovarian reserve markers and anti-corpus luteum antibodies (anti-CoL) in TA patients. Methods We have screened 52 consecutive female patients with TA. All patients aged between 18 and 45 years and fulfilled the American College of Rheumatology classification criteria. Exclusion criteria were: current pregnancy, hypothalamus-pituitary-gonadal axis dysfunction, use of hormonal contraceptive in the last six months, gynecological surgery, gynecological cancer, presence of an additional autoimmune disease, did not agree to participate in this study and incomplete gonadal evaluation. Thirty-two were excluded: use of hormonal contraceptive (n=15), did not agree to participate (n=7), incomplete assessment (n=4), presence of granulomatosis with polyangiitis (n=2), current pregnancy (n=2), hyperprolactinemia (n=1) and current treatment for gynecological cancer (n=1). Therefore, a cross sectional study was conducted in 20 patients with TA and 24 healthy controls according to the same exclusion criteria. Anti-CoL (immunoblot) and ovarian reserve were assessed by: follicle stimulating hormone (FSH), luteinizing hormone (LH), estradiol, anti-Müllerian hormone (AMH), and antral follicle count (AFC). Demographical data, menstrual abnormalities, disease parameters and treatment were also analyzed. Results The median current age was similar in TA patients and controls (31.2±6.1 vs. 30.4±6.9 years, p=0.69). The frequency of decreased levels of AMH (50% vs. 17%, p=0.02) and the median of AMH (0.7 vs. 2.7ng/mL, p=0.008) were significantly reduced in TA patients compared to controls without significant menstrual abnormalities with regard to the median of flow duration (p=0.25) and cycle length (p=0.85). The other hormones and AFC were similar to controls (p>0.05). Anti-CoL was solely observed in TA patients (5% vs. 0%, p=0.45). Further evaluation of TA patients with low AMH levels (<1.0 ng/mL) versus normal AMH levels (>1.0 ng/mL) revealed that the frequency of disease activity (p=1.0) and the median of ESR (p=0.6), CRP (p=0.4), prednisone cumulative dose (p=0.8) and methotrexate cumulative dose (p=0.8) were comparable in both groups. Cyclophosphamide use was reported in only one patient with reduced ovarian reserve, whereas none of the remaining patients received gonadotoxic drug. Conclusions Therefore, the present study demonstrated for the first time a high prevalence of diminished ovarian reserve in TA patients reinforcing fertility counseling. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1484

THU0056 Acute Aerobic Exercise Induces Increases on Plasma Levels of α-Melanocyte Stimulating Hormone in Patients with Active Systemic Lupus Erythematosus

Background Systemic lupus erythematosus (SLE) is an autoimmune disease associated with chronic inflammation. Pro-opiomelanocortin (POMC) axis activation leads to the production of several bioactive hormones, such as adrenocorticotropic hormone (ACTH) and the neuropeptide α-melanocyte stimulating hormone (α-MSH). Both hormones are closely related to the inflammatory process with pro- or anti-inflammatory effects. Although physical exercise has been described as a promoter of alterations in the neuroendocrine profile, no studies have addressed the participation of melanocortins in SLE patients in response to acute aerobic exercise. Objectives To evaluate α-MSH production in SLE patients under acute aerobic exercise. Methods Ten active SLE patients [age: 29.9±4.2yrs; body mass index (BMI): 26.0±4.8 kg/m2; SLEDAI: 5.9±2.1; glucocorticoid: 16.0±6.0 mg] and 10 age- and BMI-matched healthy controls performed 30 minutes of aerobic exercise (∼70% of VO2 peak) in a treadmill. The a-MSH and ACTH serum levels were assessed at baseline and immediately after exercise session by a multiplex system (HNP-35K kit, Millipore, Billerica, MA, USA). Results Similar levels of a-MSH were observed at baseline between active SLE patients and healthy controls (P=0.69). However, after exercise, a distinct pattern of response was noted (Fig. 1). The serum levels of a-MSH was not altered in healthy controls, but a significant increase in mean levels was observed in active SLE patients after exercise (92.39±16.12 vs. 146.23±25.01 pg/mL, P=0.04). ACTH levels did not differ between groups at baseline and after exercise (P>0.05). Figure 1. α-MSH response to acute session of aerobic exercise in active SLE patients ad healthy controls. Conclusions The present study provides novel evidence that acute aerobic exercise induces a hypothalamic-pituitary-adrenal axis response with an increase in the levels of neuropeptide α-MSH in active SLE patients. We speculate that this is an exacerbated adaptive response to exercise stress to avoid further stimulation of proinflammatory cytokines in these patients. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.5206

AB0637 Systemic Sclerosis-Related Auto-Antibodies Are Markers of New Clinical Associations in A Cohort of 328 Brazilian Patients

Background Systemic Sclerosis (SSc) shows a heterogeneous clinical presentation, characterized by marked skin and internal organ fibrosis and vascular dysfunction, associated with immunological abnormalities. A varied panel of SSc-related auto-antibodies has been described, and there is a growing interest to establish their prevalence and clinical associations in populations of different ethnicities. Objectives To evaluate the frequency and the putative associations of a panel of SSc-related auto-antibodies with demographic and clinical features in a large SSc cohort. Methods We analyzed serum of 328 consecutive SSc adult patients attended at the Scleroderma Outpatient Clinic of a tertiary referral university hospital in Brazil, classified according to the ACR/EULAR 2013 SSc criteria: 32% had diffuse cutaneous SSc and 68% limited cutaneous SSc. Clinical and demographic data were obtained through a review of the electronic register database. Serum samples were analyzed for the presence of autoantibodies using commercially available standardized kits. Results ANA positivity was 88%, in the following patterns: centromeric (26%), nuclear homogeneous/nucleolar (18%), nucleolar (16%), nuclear homogeneous (12%), nuclear speckled (12%), dense fine speckled (2%), nucleolar speckled (<1%), and cytoplasmatic (1%). Anti-Scl70 was present in 92 (28%), anticentromere (ACA) in 83 (25%), anti-fibrillarin in 39 (12%), anti-RNA Pol III in 23 (7%), anti-Th/To in 17 (5%), anti-PM-Scl in 16 (5%), anti-Ku in 12 (4%), and anti-NOR in 6 (2%), and anti-Ro/SSA was positive in 96 patients (29%). Anti-Scl70 was associated with the nuclear homogeneous/nucleolar and nuclear homogeneous patterns, while symptomatic ILD was associated to both ANA patterns (p<0.001 and 0.005), digital ulcers were associated with the nuclear homogeneous/nucleolar patterns (p=0,042). ACA was associated with female gender (p<0.001), white ethnicity (trend; p=0.076), limited cutaneous SSc (p<0.001), calcinosis (p=0.002), asymptomatic ILD (p<0.001), and isolated PAH (p<0.001). Otherwise, anti-Scl70 was associated with male gender (p=0.034), diffuse cutaneous SSc (p<0.001), digital ulcers (p<0.001), symptomatic ILD (p<0.001), and use of immunosuppressive drugs (cyclophosphamide, azathioprine, mycophenolate mofetil (p<0.001). Three patients were diagnosed as scleroderma renal crisis and two of then presented anti-RNA pol III, that was associated with diffuse cutaneous SSc (p=0.011). Anti-fibrillarin was associated with symptomatic ILD (trend; p=0.072) and heart involvement (p=0,043). Anti-Ku was associated with finger amputation (p=0.019). Anti-PM-Scl, anti-Th/To and anti-NOR90 did not show any specific association. Conclusions Among the different SSc-related auto-antibodies, anti-Scl70 and ACA clearly characterize distinct demographic and clinical presentations. Our study also identified new clinical associations, among anti-fibrillarin and heart involvement and anti-Ku with digital amputation, as well as the concomitance of anti-Ro/SSA with anti-Scl70 being associated with poor prognosis. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5641