hDepartment of Internal Medicine, Hospital Infanta Sofía, Madrid, SpainiDepartment of Internal Medicine, Hospital Gregorio Marañón,Madrid, Spain

Objective: To analyse the epidemiological, clinical and analyticalfeatures at diagnosis and the usefulness of the new 2010 Europeanactivity index (ESSDAI) to predict the development of lympho-proliferative disease in a large cohort of Spanish patients withprimary Sjogren syndrome (SS). Patients: The GEAS-SS multicenterregistry was formed in 2005 with the aim of collecting a large seriesof Spanish patients with primary SS, and included thirteen Spanishreference centers with substantial experience in the management ofSS patients. By January 2013, the database included 921 consecutivepatients fulfilling the 2002 classification criteria for primary SS. Thecumulated ESSDAI index (2010 EULAR-SS disease activity index) wasretrospectively calculated at diagnosis. Statistical values wereexpressed as the hazard ratio (HR) and 95% confidence interval(CI) for death. Results: After excluding 17 patients who werediagnosed with lymphoma before SS diagnosis, we evaluated a totalof 904 patients who were followed a mean of 79 months: 25 (3%)patients developed lymphoproliferative disease after being diag-nosed with primary SS. The following baseline features at diagnosiswere associated with the development of lymphoma: male gender(HR 5.78, CI 2.14–15.63), age (HR 1.04, CI 1–1.07), C3 values b0.82 g/L(HR 3.75, CI 1.38–10.19), C4 values b0.07 g/L (HR 3.22, CI 1.08–9.61),monoclonal serum band (HR 4.23, CI 1.38–13.02) and cryoglobulins(HR 4.44, CI 1.86–10.58). Multivariate Cox proportional-hazardsregression analysis identified gender, low C3, monoclonal band andcryoglobulins as significant independent variables related to lympho-ma. With respect to the ESSDAI domains, activity (score N1) in theconstitutional (HR 4.06, CI 1.54–10.70) and hematological (HR 2.59, CI1.16–5.78) domains was associated with the development of lympho-ma, and the hematological activity was independently associated withlymphoma development. In the constitutional domain, patients withthe highest activity degree (presence of fever N38.5 °C/night sweatsand/or involuntary weight loss of N10% of body weight) showed thehigh risk of development of lymphoma (HR 9.11, CI 2.51–33.12). Nostatistical associations were found between the remaining domains orthe total ESSDAI score and the risk of lymphoma. Conclusion: FeverN38.5 °C, night sweats and/or significantweight losswere the key clinicalfeatures prospectively associated with the development of lympho-proliferative disease in patientswith primary SS. In addition,we identifieda specific hematological and immunological profile (cytopenias, hyp-ocomplementemia, monoclonal band and cryoglobulinemia) as labora-tory predictor of hematological neoplasia in SS. Patients presenting withthis clinical and laboratory profile at diagnosis of primary SS need a closerfollow-up.

doi:10.1016/j.ejim.2013.08.288

ID: 755Mortality prognostic factors of patients with systemicautoimmune diseases admitted to an intensive care unitP.I. Dotia, S. Fernandezb, E. Colomaa, O. Escodaa, I. Rodriguez-Pintóa,P. Castrob, G. Espinosaa, J.M. Nicolásb

aDepartment of Autoimmune Diseases, Hospital Clínic, Barcelona, SpainbMedical Intensive Care Unit, Hospital Clínic, Barcelona, Spain

Objective: To identify mortality prognostic factors of patients withsystemic autoimmune diseases (SAD) admitted in a medical intensivecare unit (ICU). Methods: Retrospective observational study including

all patients with SAD admitted to a medical ICU of a tertiary referralcentre between January 1999 and December 2012. Only patients withthe diagnosis of SAD according to accepted criteria made prior to ICUadmission or during hospitalization were selected. Patients with shortterm irreversible disease and thosewith an ICU stay less than 48 hwereexcluded. The reason for ICU admission, clinical follow-up, immuno-suppressive treatment received before ICU admission, and outcomewere collected. Mortality prognostic factors were identified throughlogistic regression analysis. Results: Seventy patients accounting for 75ICU admissions (48 [68.6%] women) with mean (SD) age of 54 (19.3)years were included. Five patients were admitted twice. Twenty-three(30.7%) patients had systemic lupus erythematosus (SLE) (mean SLEDisease Activity Index [SLEDAI] at ICU admission 8.2 (5.6) [range 0–20]); 23 (30.7%) had systemic vasculitis (mean Birmingham VasculitisActivity Score [BVAS] 14.5 (9.3) [range 0–33]); 7 (9.3%) systemicsclerosis; 7 (9.3%) dermatomyositis, and 5 (6.7%) had Sjögren'ssyndrome. The reasons for ICU admission were infection in 26(34.7%), followed by autoimmune disease flare-up in 17 (22.7%). Othercomplications related or not with the SAD were present in 26 (34.7%)patients. The mean Acute Physiology and Chronic Health Evaluation(APACHE II) at admission was 16.5 (6.5) (range 5–31). At the end offollow-up, 29 (41.4%) patients had died, 10 (14.2%) during the stay atICU, 7 (10%) during hospitalization, and 12 (17.1%) after hospitaldischarge. A logistic regression model showed that multiorgan failure(respiratory failure [p = 0.032], renal failure [p = 0.017]), and the needof renal replacement therapy [p= 0.007] were risk factors associatedwith increased mortality. In addition, corticosteroid therapy [p b 0.005]and the need of intravenous immunoglobulin treatment [p b 0.005]during the stay at ICU, and the use of cyclophosphamide in the previoussix months [p = 0.016] of ICU admission, were also risk factorsassociated with increased mortality. Conclusions: The most prevalentSAD admitted to a medical ICU were SLE and systemic vasculitis beinginfections the main reason for admission. The presence of respiratoryand renal failure, the need of renal replacement therapy, the need ofcorticosteroid or intravenous immunoglobulin during the stay in ICUand the use of cyclophosphamide before the admission were factorsassociated with increased risk of mortality.

doi:10.1016/j.ejim.2013.08.289

ID: 26Vitamin D status in a population of scleroderma patientsL. Groseanua, I. Saulescua, L. Banicab, A. Balanescua, D. Predeteanua,V. Bojincaa, C. Constantinescua, F. Bergheaa, D. Oprisa, R. Ionescua

aRheumatology, Sf Maria Hospital, Bucharest, RomaniabNuclear Receptors, Institutul National de Cercetare_Dezvolatere pentruMicrobiologie-Imunologie Cantacuzino, Bucharest, Romania

Background: Epidemiological evidence indicates a significant asso-ciation between vitamin D deficiency and an increased incidence ofseveral autoimmune diseases. Low vitamin D levels have also beenreported in patients with systemic sclerosis (SSc), but the number ofstudies is limited with conflicting data.Objective: To investigate vitaminD status in a group of systemic sclerosis (SSc) patients and establishconnections with markers of SSc disease activity and severity score,clinical and imunologic features.Method: 50 scleroderma patients wereevaluated during June 2010–June 2012 in Internal Medicine andRheumatology Department of Sf. Maria Hospital, Bucharest, Romania.All patients gave their informed consent for all procedures, which werecarried out with the local ethics committee's approval. We performed acomplete evaluation of all patients following: MEDS evaluation sheets(cutaneous, musculoarticular, gastrointestinal, cardiac, pulmonary or

Abstractse114