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Background The term undifferentiated connective tissue diseases (UCTD) has been widely used to identify conditions not fulfilling classification criteria for a defined connective tissue diseases (CTD). Recently the introduction of new classification criteria for different CTD allowed to take a significant step forward for their early identification and for a more reliable diagnosis of UCTD. Objectives To analyze the clinical and serological characteristics of a large cohort of patients (pts) with UCTD and to evaluate: the incidence of Systemic Lupus Erythematosus (SLE) defined according two different “set” of classification criteria for lupus, ACR 1997 and SLICC 2012, in a time frame of 15 years (1999-2013); to analyse clinical and serological data comparing pts affected by stable UCTD to UCTD evolved to SLE. Methods A cohort of pts referred to our clinic dedicated to UCTD, who met the classification criteria proposed by Mosca et al. [1], were retrospectively analyzed. The pts with a median follow-up less than one year were excluded. Clinical and serological assessment was performed at the time of diagnosis (T0) and at 3 different time points indicated as T1 (time interval between the first and third year), T2 (between the third and fifth year) and T3 (from the fifth to the tenth year). The classification criteria for SLE, ACR 1997 and SLICC 2012, were applied to each patient at time T0, T1, T2 and T3. Results 329 pts were enrolled. An observation period up to T1 was recorded for the entire sample, up to T2 for 273 pts and 206 pts had completed the follow-up to T3. At T0 no pts met SLE/ACR criteria (according to inclusion criteria of study), whilst 44 pts met SLE/SLICC criteria: 41 females (F, 93.2%) and 3 males (M), mean age 40.1±14 years. Overall, at the end of the observation period, the SLE/SLICC criteria were cumulatively observed in 67 pts (20.4%), while 14 pts met the ACR criteria. In 12 pts both sets of criteria were satisfied. At the end of follow-up, 260 pts did not meet any set of classification criteria (UCTD stable, 79% of the whole population, 253 F and 7 M, mean age 53.6±15.5 years). During the follow-up the rate of progression to SLE was stable over the years, with a number of switch to SLE constant even after 5 years of diagnosis of UCTD. A peak rate of new cases was observed at T3 where five new cases of SLE/ACR (2.4%) and 7 cases of SLE/SLICC (3.4%) were recorded. The baseline clinical and serological characteristics of pts with stable UCTD were compared to pts evolved in SLE. Acute or subacute skin rash (p 0.02), serositis (p 0.03) and the presence of antiphospholipid antibodies (p 0.02) were the variables correlated with the progression to SLE/SLICC. Acute or subacute skin rash (p 0.001) and the presence of antiphospholipid antibodies (p 0.03) were the variables correlated with the evolution to SLE/ACR. Conclusions From our study in a cohort of UCTD pts the application of the new set of SLICC classification criteria for SLE, compared with the old ACR criteria, would not seem to have contributed significantly in terms of predictability of the evolution towards lupus, but certainly they expanded the number of pts classified as SLE at onset of the disease allowing a certain diagnosis in a greater number of pts who would otherwise be classified as UCTD. References Mosca M et al. Clin Exp Rheumatol 1999;17:615-620. Disclosure of Interest None declared

Background the overlap syndrome Systemic sclerosis-rheumatoid arthritis (SSc-RA) is characterized by the presence in the same patient of clinical and laboratory features that meet the classification criteria for both diseases. The current literature shows how that lung involvement is more frequent and severe in SSc-RA than in SSc alone [1]. Objectives to describe the frequency of clinically significant interstitial lung disease (ILD), determining the reduction of the forced vital capacity (FVC) or the alveolar-capillary diffusion of carbon monoxide (DLCO) in a group of 25 patients with SSc-RA compared to a control group of 570 patients with SSc alone. Methods data were retrieved from a database and from medical records of patients followed at the outpatient clinic dedicated to SSc of the Rheumatology Unit of S. Anna Hospital, Ferrara. All patients fulfilled the classification criteria for SSc (ACR 1980 and LeRoy 2001) and RA (1987). For both groups (SSc and SSc-RA) the following seroimmunologic data were assessed: anticentromere antibodies (ACA), anti topoisomerase 1 (Scl 70), rheumatoid factor (RF) and anti-citrulline (ACPA). Pulmonary involvement was assessed by high-resolution chest CT scan using the score of Warrick, and spirometry including DLCO. A cut-off of Warrick9s score able to detect patients with ILD has been identified by the application of a regression curve between the total score at HRCT and spirometry data (FVC and/or DLCO 10 able to alter lung function. Results in the SSc control group FR was available in 38% of cases and ACPA only in 8.9% RF and ACPA were available in all 25 SSc-RA patients. Positivity for both FR and ACCP was detected in 23 SSc-RA patients whilst 2 were seronegative. ILD was more frequent in SSc-RA group compared to SSc (76% VS 33%, p Conclusions an increased risk of ILD in SSc-RA compared to SSc was confirmed. In SSc-RA group, ACA seems to lose its role as a “protective” factor against the possible development of pulmonary fibrosis. If this information is confirmed, in SSc-RA patients, despite their ACA positivity, it could justify a more “tight” clinical-instrumental follow-up, to detect lung involvement early and to monitor its evolution. References Systemic sclerosis-rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity. Szucs G, Szekanecz Z, Zilahi E et al. Rheumatology (Oxford). 2007 Jun;46(6):989-93 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5137

Objective To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and functional outcome of interstitial lung disease (ILD). Methods (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared with 7202 anti-PM/Scl−, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case–control study: additional data were collected for 165 anti-PM/Scl+ SSc patients (85 from the EUSTAR registry) and compared with 257 anti-PM/Scl− SSc controls, matched for sex, cutaneous subset, disease duration and age at SSc onset. Results Patients with isolated anti-PM/Scl+, as compared with anti-Pm/Scl−, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls. In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed. Conclusion The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.