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1. Longitudinal analysis of quality of life in patients with undifferentiated connective tissue diseases

3. Clinical significance of the anti-Nucleolar Organizer Region 90 antibodies (NOR90) in systemic sclerosis: Analysis of the European Scleroderma Trials and Research (EUSTAR) cohort and a systematic literature review

4. AB1176 SCOPING REVIEW TO GUIDE THE DEVELOPMENT OF AN ARTICULAR SCORE IN SYSTEMIC SCLEROSIS (ASSESS SCORE)

5. POS0832 IMMUNOSUPPRESSION VERSUS COMBINATION OF IMMUNOSUPPRESSION AND ORAL GLUCOCORTICOIDS FOR SKIN FIBROSIS IN EARLY DIFFUSE SYSTEMIC SCLEROSIS PATIENTS. A TARGET TRIAL EMULATION STUDY FROM THE EUSTAR DATABASE

9. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

10. OP0238 IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS

11. POS0182 RISK-BENEFIT ASSESSMENT OF THE USE OF RITUXIMAB FOR RHEUMATOID ARTHRITIS IN REAL LIFE: FINDINGS FROM 1984 PATIENTS FROM THE FRENCH AUTOIMMUNITY AND RITUXIMAB REGISTRY

13. POS1309 ASSOCIATION BETWEEN RED CELL DISTRIBUTION WIDTH AND INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS

14. POS1255 CLINICAL SIGNIFICANCE OF THE ANTI-NUCLEOLAR ORGANIZER REGION 90 ANTIBODIES (NOR90) IN SYSTEMIC SCLEROSIS: A EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) ANALYSIS

15. Rituximab versus cyclophosphamide en traitement d’induction de la granulomatose avec polyangéite : essai thérapeutique émulé

16. Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort

17. Étude des granulomatoses avec polyangéites réfractaires au traitement d’induction

18. POS0874 GLUCOCORTICOIDS PRESCRIBING PRACTICES IN SYSTEMIC SCLEROSIS: AN ANALYSIS OF THE EUSTAR DATABASE.

19. AB0736 ASSOCIATION BETWEEN ERYTHROCYTE DISTRIBUTION WIDTH AND SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE

20. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

22. POS0316 MODELLING SHORT-TERM FVC CHANGES FROM SENSCIS TO LONG-TERM FVC COURSE IN SSc-ILD DEMONSTRATES CLINICALLY MEANINGFUL REDUCTION OF FVC DECLINE AND SURVIVAL BENEFITS

25. Les granulomatoses avec polyangéite (GPA) sans ANCA ou avec ANCA anti-myéloperoxydase représentent des entités distinctes au sein des GPA. Analyse de 727 GPA du registre du Groupe Français d’Etude des Vascularites

26. Granulomatose avec polyangéite : analyse des 795 patients de la base de données du groupe français d’étude des vascularites

30. Rémission à distance de tout traitement au cours de la granulomatose avec polyangéite (Wegener) : données du registre du Groupe français d’étude des vascularites

32. Update of EULAR recommendations for the treatment of systemic sclerosis

33. Update of EULAR recommendations for the treatment of systemic sclerosis

35. Les granulomatoses avec polyangéite (GPA) sans ANCA ou avec ANCA anti-myéloperoxydase représentent des entités distinctes au sein des GPA : analyse de 727 GPA du registre du Groupe français d’étude des vascularites

37. CLINICAL SIGNIFICANCE OF THE ANTI-NUCLEOLAR ORGANIZER REGION 90 ANTIBODIES (NOR90) IN SYSTEMIC SCLEROSIS: A EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) ANALYSIS.

39. Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity

42. Brief report: Successful pregnancies but a higher risk of preterm births in systemic sclerosis: An Italian multicentric study

45. [OP0219] EUROPEAN SCLERODERMA STUDY GROUP (ESCSG) ACTIVITY INDEX IS CORRELATED TO QUALITY OF LIFE MEASURES BOTH AT ADMISSION AND OVERTIME M. Iudici, G. Cuomo, G. Abignano, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Objectives: EScSG activity index has been validated for construct validity [1]. Its discriminant validity awaits to be investigated. In order to address this aspect, we investigated the relationship between the activity index and Health Assessment Questionnaire-Disability Index, physical and mental component scores (PCS and MCS) of Short Form-36 (SF36). Methods: 140 SSc patients consecutively admitted at tertiary center were investigated for EScSG activity index, HAQ-DI and PCS and MCS of SF36 at enrolment and after 1 year. The change (Δ) for each of the all measures was calculated. Results: EScSG activity index was found to be correlated to HAQ-DI, PCS and MCS both at admission (Rho=0.49, p=0.0003; Rho= -0.40, p<0.0001; Rho= -0.29, p=0.001, respectively) and after 1 year (Rho=0,64, p<0,0001; Rho= -0.29, p=0.001; Rho=-0.18, p=0.046 respectively). Moreover, the change between the value of the activity index at 1 year and that at admission (Δ activity index) was significantly correlated to ΔHAQ-DI, ΔPCS, ΔMCS (Rho=0,43, p=0.002; Rho=0,56, p<0.0001; Rho=0.48, p=0.002, respectively). Conclusion: HAQ-DI and SF36 (PCS and MCS) are considered to be validated outcome measure in SSc. Our results further support the construct validity of EScSG activity index and are for the first time underline its discriminant validity. References: • Valentini G. et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 2003. Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):128 Session: Abstract Session: Modern prespective in systemic sclerosis

46. [THU0309] CLINICAL MANIFESTATIONS IN LATE VS EARLY –ONSET SYSTEMIC SCLEROSIS (SSC) G. Cuomo, M. Iudici, G. Abignano, G. Valentini. Second Universty of Naples, Rheumatology Unit, Naples, Italy Background: Differences have been so far pointed out in the clinical manifestations and disease course in patients with a number of autoimmune rheumatic diseases and different age at onset (1-3). Objectives: To investigate the occurrence of differences between early and late onset SSc. Methods: 260 SSc patients (233 females) with a median age at the onset of the disease of 40.5 years (range 8-74) were consecutively admitted to a tertiary centre from November 1st 2000 to October 31st 2008. They were divided into 2 groups: early onset (<40.5 years) and late onset (≥40.5 years). Differences in each of the epidemiological, clinical and serological aspects collected in all the patients at admission were analysed. Results: Table 1 lists the significant differences detected between the 2 groups. Early onset patients were found to have a longer disease duration at presentation, a higher prevalence of anti-Scl70 positivity and a higher prevalence of scleroderma renal crisis; a lower prevalence of antinucleolar antibody positivity; a lower percentage of pulmonary hypertension; a lower HAQ-DI and a lower prevalence of heart involvement. No difference was detected in clinical subset distribution. Late onset (138) Early onset (122) p Disease duration mean (sd) 6 (6) 10 (10) 0.003 Range (median) 0.5-26 (5) 0.5-45 (6) Serological subset Scl70 43 54 0.04 Nucleolar 9 23 0.004 PAPs ≥40mmHG (echocardiography) 14/124 3/119 0.01 HAQ-DI mean (sd) 0.65 (0.7) 0.5 (0.7) 0.037 Range (median) 0-0.275 (0.375) 0-0.25 (0.125) Severity scale Late onset (138) Early onset(122) p 0-1 2-3-4 0-1 2-3-4 Heart 117 21 121 1 <0.0001 Kidney 138 0 117 5 0.02 Conclusion: Our study demonstrate that similarly to systemic lupus erithematosus and rheumatoid arthritis, distinct differences related to age at onset also occur in SSc patients. References: • Calvo-Alen J. et al, Clin Rheumatol 2005 • Lalani S. et al, J Rheumatol 2010 • Ho CT et al, Ann Rheum 1998 Disclosure of Interest: None declared Citation: Ann Rheum Dis 2010;69(Suppl3):247 Session: Scleroderma, Myositis and related syndromes

48. [FRI0313] CYCLOPHOSPHAMIDE PULSE THERAPY IN THE TREATMENT OF SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE G. Abignano, M. Iudici, A. Petrillo, G. Cuomo, G. Valentini. Rheumatology Unit, Second University of Naples, Napoli, Italy Background: Cyclophosphamide (CYC) is currently used in the treatment of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). A recent meta-analysis1 (Nannini C et al), pooling data from studies based on different entry criteria, questions its usefulness. Objectives: To investigate the effectiveness of low dose pulse CYC (500 mg/dose) in the treatment of recently deteriorating SSc-ILD. Methods: 51 patients with SSc-ILD, all of them satisfying ACR criteria for the classification of the disease and presenting with a recent (<6 months) decrease (≥10% of the predictive value) of either Forced Vital Capacity (FVC) or Diffusing Lung Capacity for CO (DLCO), were enrolled in the study. All of them underwent a concurrent prednisone therapy (10 mg/daily). CYC was administered i.v. at a dose of 500 mg weekly. Total CYC dose ranged from 4.5 to 11.5 g (median 7.5). An increase of 10% in either FVC or DLCO was considered indicative of improvement; a change between <10% and >10% of stable disease; a decrease ≥10% of worsening. Results: 22 out of the 51 SSc patients (43.14%) resulted to improve at the end of the CYC course; 17 (33.33%) remained stable; 12 (23.53%) worsened. No patients withdrew CYC treatment because of side effects. Conclusion: Our study suggests that the effectiveness of CYC in SSc-ILD may depend on entry criteria. Actually about 76% of patients who had experienced a recent deterioration of lung function, suggesting active alveolitis, underwent improvement or stabilization of their disease in our study. References:[ol][li]Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008, 10:R124.[/li][/ol]Disclosure of Interest: None declared Ann Rheum Dis 2009;68(Suppl3):460 Session: Scleroderma, myositis and related syndromes

50. [FRI0311] CORRELATIONS BETWEEN CLINICAL FEATURES OF154 PATIENTS WITH SYSTEMIC SCLEROSIS (SSC) AND SHORT FORM36 (SF36) SCORES G. Cuomo, M. Iudici, G. Abignano, A. Petrillo, G. Valentini. Rheumatology Unit, Second University of Naples, Naples, Italy Background: The quality of life is reduced in patients with systemic sclerosis (SSc) as evaluated by both disease specific (HAQ-DI; SHAQ) or generic questionnaires. The correlations between SF-36 scores and some disease features have not yet been explored. Objectives: To evaluate the relationships between health values as evaluated by SF-36 and clinical features in SSc patients. Methods: From 9/01/07 to 8/31/08 154 consecutive SSc patients (144 females; 10 males); aged from 20–82 years (median age 54), with disease duration from 1-47 years (median 10,5 years); attending the outpatient clinic of the Rheumatology Unit of Second University of Naples, were enrolled in the study. The scores of the eight areas of SF-36 General Health: Physical Function (PF), Role Physical (RP), Bodily Pain (BP), General Health (GH), Vitality (VT), Social function (SF), Role Emotional (RE) and Mental Health (MH), were evaluated in the range of 0–100, the higher values reflecting a better Quality of life (Qol). In addition two global series i.e. the Physical Component Summary Score (PCS) and the Mental Component Summary Score (MCS) were calculated. The patients were also investigated for disease subset (limited or diffuse); skin involvement by the modified Rodnan skin score; disability by HAQ-DI; disease activity by European Activity Index, and disease severity by Medsger severity scale. Results: SF36 score did not differ between the 31 patients with diffuse vs the 123 with limited disease All the items of SF36 were significantly correlated to activity Index (Rho from –0.28 to -0.32: p<0.0001) and HAQ-DI (Rho from -0.38 to -0.82: p<0.0001). GH resulted to correlate with mRss (Rho -0.17; p<0.05) The significant correlations between each item of the Medsger's severity scale and each item of SF36 are reported in table. PF RP BP GH VT SF RE MH PCS/MCS Sev_Gen (Rho) – – – – – – – – –/– Sev_Vasc(Rho) – – – – – – – – –/– Sev_Skin (Rho) – – – – – – – – –/– Sev_Joint (Rho) – – – -0.18* – – – – -0.21**/– Sev_muscle (Rho) -0.25** – – -0.16* -0.18* -0.22* – -0.20* -0.20*/– Sev_GI (Rho) -0.20* – – -0.22** – – – – –/– Sev_lung (Rho) -0.28*** – – -0.23** -0.17* -0.23** -0.22** – -0.19*/– Sev_Heart (Rho) – – -0.22* – – – – – -0.21*/– Sev_kidney (Rho) – – – – – – – – – *p<0.05; **p<0.001; ***p<0.0001. Conclusion: The present study, confirms that the Health Value, as evaluated by SF36, is diminished in SSc. Our study point out a previously univestigated correlation between SF-36 scores and disease activity. Disclosure of Interest: NONE declared Ann Rheum Dis 2009;68(Suppl3):459 Session: Scleroderma, myositis and related syndromes

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