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Background Autonomic dysfunction, smooth muscle fibrosis and vascular damage lead to small intestinal bacterial overgrowth (SIBO) in Systemic Sclerosis (SSc). SIBO is characterized by diarrhea, abdominal pain, bloating, malabsorption and malnutrition. Objectives To evaluate by NIH PROMIS® gastrointestinal symptoms scales and SIBO by hydrogen breath test (HBT) in patients with SSc. Methods We include 68 patients with SSc (ACR-EULAR 2015) who signed informed consent. NIH PROMIS®questionarie was applied to evaluated gastrointestinal symptoms and classified in not symptomatic, least, mildy, moderately and most symptomatic. Glucose HBT was applied after 14 hours fast, oral hygiene and 30 days free of antibiotics. Patients who has a negative HBT with symptoms associated to glucose ingestion we repeat test with lactulose. Results We applied questionnaire to 58 SSc patients, age 52 (26–75)years, 65 (96%) female and 3 (4%) males, disease duration 13 (1–40) years, limited SSc 41 (59%) and diffuse 27 (41%), body mass index 24 (12–39). They are using prednisone (28%), micofenolate (14%), methotrexate (19%), azatioprin (5%), amlodipine or nifedipine (33%). Patients had continuous and very high increase of parts per millon (ppm) of exhaled Hydrogen: min0: 13 ppm (5–21), min15:17 ppm (5–43), min30:17 (3–49), min45:18ppm (7–103), min 60:22ppm (8–145), min90:18ppm (2–250), min120:25ppm (3–212), min150:71ppm (3–235). Normal values: Frequency of gastrointestinal symptoms were flatulence (87.5vs81.2%), nausea/vomiting (72.7vs37.6%), constipation (65.6vs40%), diarrhea (45.2vs33.4%), abdominal pain %) and incontinence (39.4vs31.3%) respectively between SCB (+) positive and negative. Hyperproduction of hydrogen in breath had a direct correlation to severity of their symptoms (p≤0.05). The severity of diarrhea was in close relation to the severity of its rectal incontinence (r=0.73,p=0.001), and greater abdominal pain with flatulence (r=72, p=0.001). Conclusions Gastrointestinal symptoms are common in SSc regardless of whether they have SIBO. However, a higher Row Score SGI or moderate severe status (NIH PROMIS) correlates with high H scores from the 30th minute, therefore, the questionnaire is useful within the SSc assessment. Disclosure of Interest None declared

Background Systemic sclerosis (SSc) is characterized by fibrosis, autoimmunity and vasculopathy. ES is classified subtypes: diffuse (dSSc) and limited (ISSc). More than 35% develop calcinosis. Calcium, phosphorus, parathormone, vitamin D, TGF-β, nitric oxide and osteonectin and osteopontin involved in bone mineralization. Objectives The aim was to compare osteonectin (ON), osteopontin (OP), TGF-β, nitric oxide (NO), Paratohormone (PTH), Vitamin D and minerals concentrations in ES with and without calcinosis. Methods Cross-sectional study in ES patients (ACR criteria). We quantified OP, ON, TGF-β, ON, Calcium, Phosphorus, PTH and vitamin D in serum by ELISA. We performed descriptive statistics, Student t, Pearson correlation (significance p Results We included 71 patients, age 52.94 (± 11.47); 28 (40%) with calcinosis (18 dSSc/10 lSSc), and 43 (60%) without calcinosis (13 dSSc/30 lSSc). Biochemical parameters between two groups. In the whole population the higher PCR had moderate positive correlation (r =0.41, p=0.042), and serum calcium level had a moderate negative correlation (r = -0.47, p=0.021); ON increased in direct relation to OP (r =0.3, p=0.014) and the serum levels of VitD had lower indirect relation with the evolution time of SSc (r = -0.28, P=0.025) and the ON increase in direct relation to serum creatinine (r =0.039, p=0.006). Conclusions Patients with a longer time evolution of SSc have less serum levels of VitD and those with higher inflammation (PCR) have a higher TGF-β than a potent inducer of fibrosis. PCR and TGF-B have a moderate direct correlation, PCR and Calcium have moderate indirect correlation, ON and OP have a low direct correlation, VitD and evolution of diseases (years) had a low indirect correlation and NO and creatinine had a very low direct correlation. References Fueki H, Hino R, Yoshioka M, Nakamura M, Tokura Y.Calcinosis cutis associated with primary Sjogren9s syndrome: strong expression of osteonectin and matrix Gla protein.Rheumatology (Oxford) 2011; 50(12):2318–20. Kim SY, Choi HY, Myung KB, Choi YW.The expression of molecular mediators in the idiopathic cutaneous calcification and ossification. J Cutan Pathol 2008; 35(9):826–31. Ahmed S, O9Neill KD, Hood AF, Evan AP, Moe SM. Calciphylaxis is associated with hyperphosphatemia and increased osteopontin expression by vascular smooth muscle cells. Am J Kidney Dis 2001;37(6):1267–76. Kawakami T, Soma Y, Mizoguchi M, Saito R. Immunohistochemical expression of transforming growth factor beta3 in calcinosis in a patient with systemic sclerosis and CREST syndrome. Br J Dermatol. 2000;143(5):1098–100. Disclosure of Interest None declared

Background Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been associated with previous exposure to various agents such as silicone implants, which elicit chronic stimulation of the immune system against the prosthetic material and clinical manifestation of autoimmune disease. This is particularly the case in genetically susceptible hosts. Objectives The aim is to describe de prevalence, family background and main autoimmune rheumatic disease (ARD) associated to silicone breast implant (SBI). Methods We study a cohort of 150 patients with diagnosis of ASIA associated injection of mineral oil and silicone breast implant (SBI) in a tertiary Hospital, from 2011 to 2016. All patients were evaluated for the fulfilment of ASIA criteria. We only included patients with ASIA criteria associated with SBI plus criteria for an autoimmune rheumatic disease according to The American College of Rheumatology or EULAR. We excluded patient with ASIA and without ARD. Results There were 17 women patients with mean age 42.4±15.3 years, mean disease duration of disease 8±3. The clinical manifestation post SBI appeared 8±2 years later. The ARD were systemic sclerosis (SSc) 5, systemic lupus erythematosus (SLE) 3, rheumatoid arthritis (AR) 3, overlap syndrome 2 (SSc plus SS and SLE plus SSc, Sjogren syndrome 1, Takayasu arteritis 1, Still disease 1, antiphospholipid syndrome 1, and 3 patients also had secondary fibromyalgia. Five Patients had more than 2 autoantibodies, 4 patients had relatives with an ARD. All patients are being treated according to the ARD (steroids plus immunosuppressive 8 patients, immunosuppressive 7, and only steroids 2), in 4 patients the prosthesis were withdrawn with improvement of clinical manifestations. Conclusions We found a prevalence of ASIA associated to SBI of 11%. The main ARD were SSc, SLE and RA. In these cases of ASIA associated with SBI some had genetic predisposition to ARD. The use of SBI is not recommended in women who have a family history of ARD. References Colaris MJ et al. Two hundred cases of ASIA syndrome following silicone implants: a comparative study of 30 years and rewiew of current literature. Immunol Res 2016 Jul 13. Jara LJ, et al. Severe manifestation of autoimmune syndrome induced by adjuvants (Shoenfeld9s syndrome) Immunol Res Jul 13. Watad et a. Autoimmune/Inflammatory induced by adjuvants (Shoenfeld9s syndrome) UN update. Lupus 2017 Jan 1. Disclosure of Interest None declared

Background Systemic Lupus Erythematosus (SLE) is an autoimmune disease of unknown etiology affecting different organs and systems, and requiring the use of immunosuppressors (IS), which increases the risk of infections such as tuberculosis (TB) and its deliberate search is mandatory. Patients with SLE are prone to infections as a consequence of either intrinsic immunologic defects or the IS mainly steroids. The prevalence of TB is higher in SLE patients compared with the general population and has been reported in endemic areas up to 5%. Objectives To determine the prevalence of pulmonary TB - extrapulmonary and associated factor in SLE patients. Methods we performed a retrospective study in a cohort of 328 SLE patients between 2010–2015 in tertiary level hospital. We included SLE patients with TB diagnosis which was made by PCR, culture, histopathology (HP), acid fast bacillus (AFB) and therapeutic trial (TT). We investigated associated factors for TB: IS drugs 6 months prior diagnosis of Tb (steroids, cyclophosphamide pulse (CY), azathioprine, mycophenolate mophetil (MM) and rituximab), comorbidities (diabetes mellitus, chronic renal disease, arterial hypertension). We also investigated previous exposure and vaccination for TB, type of TB: pulmonary and extra pulmonary. Time evolution, organ involved and SLEDAI at time of diagnosis of Tb were analyzed. Descriptive statistics were performed descriptive statistical. Results We found 13 SLE patients with TB (mean age 43.5± 9.06 years, mean disease evolution of SLE: 6.6±3.2 years). SLE organ involvement: Renal: 7, hematological: 4 Neuropsychiatric: 2; joint -mucocutaneous 6. Active SLE: 9 (SLEDAI 11.5±4.9), inactive SLE: 4. Use of IS: MM 6, Azathioprine: 7, Cy: 5, steroid: 13 (prednisone: median 2.5, range 2.5 to 50 mg/day and methylprednisolone pulse 3g bimonthly in 2 patients), Rituximab: 3. Prevalence of TB was 13 of 328 (4%) patients, extrapulmonary:10 (77%) renal: 4, meningeal: 3, cerebral tuberculoma: 1 peritoneal: 1 disseminated: 1, and pulmonary TB: 3 (23%). Manifestation associated with TB: Fever 12 (92%) appetite loss: 3 (23%), weight loss 5 (38%), asthenia 8 (61%) headache: 3 (23%), hematuria: 2 (15%). TB diagnosis: AFB: 9 (69%), HP: 1 (7.5%), culture and PCR: 2 (15%), TT: one patients with miliary TB who died. Conclusions The prevalence of TB in SLE patients was about 4% and its main form was extrapulmonary. The presence of tuberculosis in patients LES constitute a chalence and should be identified in promptly in order to initiate an adequate treatment. TB diagnosis in patients with active SLE is difficult because both diseases share clinical and laboratory manifestations. References Lu MC, Lai CL, Tsai CC et al. Increased risk of pulmonary and extra-pulmonary tuberculosis in patients with rheumatic diseases. Int J Tuberc Lung Dis. 2015;19:1500–6. doi: 10.5588/ijtld.15.0087. Disclosure of Interest None declared

Background Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease characterized by clinically heterogeneous manifestations. Infections can be the initial trigger to the development of autoimmunity. Initial presentation of SLE can mimic infections, and in turn infections can mimic disease flares in established SLE. Infections are an important cause of morbidity and mortality in SLE. Bacterial infections are most frequent, followed by viral and fungal infections. The impaired cellular and humoral immune functions seen in patients with SLE are predisposing conditions. Disease activity and high doses of methylprednisolone or cyclophosphamide are well-recognized risk factors for infection. Objectives To determine the prevalence and mortality from infection in SLE patients as well as to identify etiology, associated factors to severity and site of infection. Methods We performed a retrospective study from 2010 to 2014 in a referral hospital. We analyzed risk factors (RF) on admission, involved organ, infectious type and etiology. Statistical analysis: We did descriptive statistical, Chi2, multivariate analysis. Results There were 328 patients, 87% women and 13% men, mean age 32.4±9.9 years. Infections as cause of hospitalizations were 42.2%, prevalence of infections was 49%, and mortality from severe infections was 45%. The RF for severe infections were: renal involvement Odds Ratio (OR) 3.057, P Conclusions Infections are an important cause of morbidity, mortality and hospitalization in SLE patients. The lower respiratory tract infections of bacterial origen were most frequent. The renal and hematological involvement, increased dsAnti-DNA, hypocomplementaemia, and leukolymphopenia were the most important factors for severe infections. Infections in active SLE patients constitute a true challenge for the clinician References Lee YH, Choi SJ, Ji JD, Song GG. Overall and cause-specific mortality in systemic lupus erythematosus: an updated meta-analysis. Lupus. 2016 Jan 24. Tektonidou MG, Wang Z, Dasgupta A, Ward MM. Burden of Serious Infections in Adults With Systemic Lupus Erythematosus: A National Population-Based Study, 1996–2011. Arthritis Care Res (Hoboken). 2015;67:1078–85. Disclosure of Interest None declared

Background Systemic sclerosis (SSc) and Sjogren9s syndrome (SS) are autoimmune diseases, which can coexist with sicca syndrome due to accumulation of collagen or chronic inflammation of the exocrine glands (salivary and endocrine). The prevalence of secondary SS to SSc ranges from 4 to 60%. Objectives To determine the prevalence of SS and sicca syndrome in Mexican patients with SSc. Methods Cross-sectional study. Seventy seven patients with SSc were studied at a tertiary hospital. SS and sicca syndrome were investigated according to the 2002 revised international criteria for Sjogren9s syndrome by the American European Consensus Group (AECG). All patients underwent minor salivary gland biopsy, and the results of fibrosis andinflammation were classified according to Tarpley9s classification with a score ≥1. Anti-Ro/SSA and Anti-La/SSB antibodies were quantified in 34 patients with positive histological findings for SS. All patients with ocular symptoms were also performed Schirmer9s I test to evaluate the ocular manifestations. Results Infiltration characteristic of SSc, and 21 (27%) revealed SS lymphocytic infiltrate. Isolatedxerostomia was found in 17 (22%) patients, and isolated xerophthalmia in 5 (7%) patients.Five (6.5%) patients referred parotid glands swelling. Anti-Ro/SSA and/or anti-La/SSBautoantibodies were found in 28.5% (6/21) of patients with SS. 21 (27%) patients met the2002 revised criteria for classification for SS by the AECG, Schirmer9s I test revealed dryeye severity level 2 in 4 (19%) patients, level 3 in 12 (57%) patients and level 4 in 5 (23.8%) patients. Conclusions The prevalence of sicca syndrome was 71%, 44 per cent related to systemic sclerosis9s collagen infiltration, and 27% due to Sjogren9s syndrome9s lymphocytic infiltrate. It is important to give a treatment to improve symptoms and considering the high frequency of non Hodgkin lymphoma in patients with SSc and SS. References Kobak S, Oksel F, Aksu K, Kabasakal Y. The frequency of sicca symptoms and Sjogren9s syndrome in patients with systemic sclerosis. Int J Rheum Dis. 2013;16:88–92. Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, Luciano N, Talarico R, Tani C, Tavoni AG, Bombardieri S. Overlap of ACA-positive systemic sclerosis and Sjogren9s syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma. Clin Exp Rheumatol. 2013;31:272–80. Avouac J, Sordet C, Depinay C, et al. Systemic Sclerosis - Associated Sjogren9s Syndrome and Relationship to the Limited Cutaneous Subtype. Arthritis Rheum. 2006; 54: 2243–2249. Disclosure of Interest None declared

Background Diastolic dysfunction (DD) is the earliest hallmark of cardiac disease in systemic sclerosis (SSc) and is due to endothelial dysfunction and ischemia-reperfusion damage. NT-proBNP is released by endothelial cells and ischemic myocites enhancing the recruitment of endothelial progenitor cells (EPC) from bone marrow for vasculogenesis and cardiac regeneration. This mechanism has not been explored in SSc. Objectives To investigate the relation among DD and its severity, NT-proBNP levels and EPC recruitment in patients with SSc compared to healthy controls. Methods Cross sectional study was carried out; 100 SSc patients and 56 healthy controls were included. Systolic dysfunction, structural cardiopathy, neoplasms, overlap syndrome, diabetes and hypertension were excluded. DD was stratified in mild, moderate and severe according to 2009 EAE/ASE guidelines. NT-proBNP was measured by electroquimioluminiscence. EPC were characterized by flow cytometry into three subtypes according to the expression of CD309, CD34 and CD133.We performed univariate, multivariate and subanalysis among DD subtypes and organic damage in SSc. P value of 0.05 was considered significant. Results Mean age was 48.28±3.88 for controls and 49.48±9.88 for patients (p=0.19); 53 controls (95%) and 97 patients (97%) were women (p=0.23). Diastolic dysfunction was present in 1 control (1.7%) and 61 SSc patients (61%) (p=0.0003). NT-proBNP levels were 22.24 pg/ml ±8.48 for controls, 50.51 pg/ml (12-253) for patients without DD and 336.15 pg/ml (17-2250) for patients with DD (p=0.0001). CD309/34+ EPC was 0.84 cels/ml (0.1-3.1) for controls, 1.71 cels/ml (0.1-5.5) for patients without DD and 0.86 cels/ml (0.01-5.5) for patients with DD (p=0.0001); same tendency was observed with other two subtypes of EPC. Subanalysis showed that EPC was different (p=0.0001) among mild, moderate and severe DD. Conclusions NTproBNP is related to severity of asymptomatic DD and could be useful as a predictive biomarker of cardiac damage in SSc. We observed that EPC recruitment in the presence of DD is diminished compared to controls, nevertheless in absence of DD, EPC recruitment is higher than controls. This observation suggest that recruitment of EPC is substancial in the cardiac regeneration and the lost of this mechanism of regeneration could explain the progression of cardiac damage in SSc. References Hinchcliff M, Desai CS, Varga J, Shah SJ. Prevalence, prognosis and factors associated with left ventricular diastolic dysfunction in systemic sclerosis. Clin Exp Rheumatol 2012;30(suppl 71):S30-S37. de Lemos JA, Mcguire DK, Drazner MH. B-type natriuretic peptide in cardiovascular disease. Lancet 2003;361:9370. Tschope C, Kasner M, Westermann D, Gaub R, Poller WC, SchultheissHP. The role of NT-proBNP in the diagnosis of isolated diastolic disfunction: correlation with echocardigoraphic and invasive measurements. Eur Heart H 2005;26:2277-2284. Disclosure of Interest None declared

Background Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis, immunological and vascular abnormalities. Cerebral hypoperfusion can be caused by microvascular cerebral ischemia. Cognitive impairment (CI) are a major cause of morbidity in SSc. Objectives To estimate the frequency of CI in SSc patients and alterations in cerebral perfusion (CP). Methods We studied 88 patients with SSc. The Montreal Test (MT) was given to all patients to evaluate CI. To 15 patients with CI and without systemic hypertension, diabetes mellitus, cerebrovascular disease, vasculitis, hypothyroidism, depression, and drugs that interfere with the cognitive assessment, the CP was measured by cerebral gammagram (CG). Results Of the 88 woman patients with SSc, 58 (66%) had CI by MT. The CP was decreased in 12 of 15 (80%) patients. The mean age of patient with CI was 45.9±10.5 vs patients without CI 43.1±14.1 (p Conclusions The CI is common in SSc. A decrease in CP was more frequent in the frontal lobe, predominating in older patients and with longer duration of SSc. It is important to investigate if vasodilators treatment could improve the CP. References Giuliodori G1, Fraticelli P, Bartolini M, Cagnetti C, Baruffaldi R, Rocchi MB, Provinciali L, Gabrielli A, Silvestrini M. Cognitive and cerebral hemodynamic impairment in scleroderma patients. Eur J Neurol. 2009;16(12):1285-90. Cutolo M, Nobili F, Sulli A, Pizzorni C, Briata M, Faelli F, Vitali P, Mariani G, Copello F, Seriolo B, Barone C, Rodriguez G. Evidence of cerebral hypoperfusion in scleroderma patients. Rheumatology (Oxford). 2000; 39(12):1366-73. Disclosure of Interest None declared

Background Patients with systemic lupus erythematosus (SLE) have accelerated atherosclerosis that can be assessed by the carotid intima media thickness (CIMT) measurement. A prompt hypolipidemic treatment should be a part of the integral management Objectives To determine the effect of therapy with pravastatin plus ezetimibe on the CIMT in SLE patients. Patients and Methods Longitudinal, prospective, quasi-experimental trial. Out of 60 SLE patients in whom a carotid ultrasound was performed, we chose 22 with a CIMT >0.7 mm who were administered pravastatin plus ezetimibe during 6 months with determination of CIMT at the end of the study. We performed the following tests: total cholesterol (TC), HDL-cholesterol, LDL-cholesterol, tryglicerides, C-reactive protein (CRP), liver function, muscle enzimes and glucose, basal and at the end of treatment. Statistical analysis: Descriptive statistics and Wilcoxon test were used. Results There were 22 women with an age of 42±6.3 years, average disease evolution 7.5±6.6 years, of whom, 18 concluded the study. Right basal CIMT was 0.829±0.1448 vs. final 0.688±0.1453, p Conclusions Treatment with pravastatin plus ezetimibe decreases the CIMT with improvement in the concentration of total cholesterol, LDL-C and CRP levels with good toleration. References Petri MA, Kiani AN, Post W, Christopher-Stine L, Magder LS. Lupus Atherosclerosis Prevention Study (LAPS). Ann Rheum Dis. 2011;70(5):760-5 Manzi S, Selzer F, Sutton-Tyrrell K, Fitzgerald SG, Rairie JE, Tracy RP, et al. Prevalence and risk factors of carotid plaque in women with systemic lupus erythematosus. Arthritis Rheum. 1999;42:51-60. Riboldi P, Gerosa M, Moroni PL. Statins and autoinmune diseases. Lupus 2005;14:765-68. Disclosure of Interest None declared

Background Pulmonary arterial hypertension (PAH) is a devastating vascular complication of systemic sclerosis (SSc) and is found in 10-15% of cases carrying a severe prognosis. PAH has a dramatic impact on the clinical course and overall survival, being the most common cause of death in patients with this entity. Doppler echocardiography represents the best initial screening tool. Objectives To determine the prevalence of PAH in mexican patients with systemic sclerosis. Methods We studied 136 patients who met the American College of Rheumatology classification criteria for Systemic Sclerosis from January to December 2011 in the Internal Medicine Departments of a tertiary Hospital. This is a cross sectional study, that included patients of ages 18 or older with the diagnosis of SSc in the SSc outpatient clinic. We performed a Doppler Echocardiography and determined PAH. We used a descriptive statistical analysis. Results There were 136 patients and the mean age was 51±13 years: women 125 (91.2%) and men 12 (8.7%). Median disease evolution: 6 years with a range from 1-32 years. The limited cutaneous SSc (lcSSc) was present in 70 patients (50.09%), diffuse cutaneous SSc (dcSSc) in 66 (48.17%) and Scleroderma sine Scleroderma in 1 (0.72%). PAH was found in 59 patients (43.3), of whom 23 (38.9%) had mild form, 27 (45.7%) moderate and 9 (15.2%) had severe PAH. In dsSSC PAH was found in 22 (33.8%) patients and in lcSSc in 36 (51.4%) patients Conclusions The prevalence of PAH in SSc was 43%. Of our patients with PAH: 15% was severe, 27 moderate and 43% mild. Therefore we should make a prompt diagnosis and start an adequate treatment. References Elliot GC, Barst RJ, Seeger W et al. Worlwide physician education and training in pulmonary hypertension: pulmonary vascular disease: the global perspective. Chest 2010: 137:85S-94S Vera Lastra O, Porres. Pulmonary arterial hypertension associated with systemic sclerosis: Current diagnosis approach and therapeutic strategies. World J Rheumatol 2012; 28: 2:12-20 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4858

Background Microvascular oclussion may occur as part of the manifestations of the antiphospholipid syndrome (APS). One of these manifestations is Sensorineural hearing loss (SHL). Objectives To determine the prevalence of inner ear dysfunction in patients with primary APS and its association, if any, with carotid intima-media thickness (IMT). Methods From a cohort of 50 patients with primary APS we investigated the presence of inner ear dysfunction defined as SHL and/or labyrinthine affection based on the following symptoms: unilateral or bilateral sudden or progressive hearing loss, tinnitus and recurrent vertigo. Patients with the above-described symptoms underwent audiometric assessment. Those with vestibular symptoms also had balance function testing. Inner ear dysfunction was defined as hearing loss below normal values (mild hearing loss: audition loss 60 to 90dB) or presence of labyrinthine affection. Cardiovascular risk factors such as dyslipidemia, diabetes mellitus, arterial hypertension and carotid IMT were assessed. For the statistical analysis we employed descriptive statistics and chi square test. Results We detected 15 patients with hearing loss (30%) with a mean age of 51.6 years, mean APS disease evolution 12.7 years, treated with oral anticoagulants (INR between 2-3) and statins, 3 had arterial hypertension, 2, diabetes mellitus, 6 with dyslipidemia, and 8 had history of ischemic stroke. One patient presented sudden SHL that improved with corticosteroids, another one had left vestibular paresis as a stroke sequelae. Twelve patients were diagnosed with mild to moderate bilateral SHL and 3 with unilateral SHL. Only 2 patients had conductive hypoacusia due to otosclerosis, 5 had tinnitus and vertigo and were diagnosed as labyrinthine affection that improved with vestibular rehabilitation therapy. Three patients required hearing aid. Interestingly 12/15 patients (80%) with bilateral hearing loss presented carotid IMT with a mean of 1.2 mm ±0.2 (range from 0.8 to 2.9 mm). In contrast, 14/35 (40%) with carotid IMT did not have hearing loss (p Conclusions Inner ear dysfunction is present in primary APS patients and is associated with carotid IMT. SHL may be an atherosclerosis expression. Other alternative therapies, besides the strict control of risk factors for atherosclerosis are needed. Auditive assessment must be performed at least once a year in order to identify and delay the presence of disability. References Mouadeb DA, et al. Antiphospholipid inner ear syndrome. Laryngoscope 2005;115: 879-83. Yehudai D, Shoenfeld Y, Toubi E. The autoimmune characteristics of progressive or sudden sensorineural hearing loss. Autoimmunity. 2006;39(2):153-8. Disclosure of Interest None Declared

Background Dendritic cells (DCs) are antigen presenting cells with the ability to induce primary immune responses and preserve immunological tolerance during normal pregnancy. Prolactin (PRL) plays an important role as an immunostimulator hormone-cytokine and it is considered to be associated with fetal loss and systemic lupus erythematosus (SLE) activity during pregnancy. However, the interaction between DCs and PRL in the gestational period of SLE patients is unknown. Objectives To determine the PRL and DCs concentrations in pregnant patients with SLE and its relationship with lupus activity and obstetric outcome. Methods Pregnant patients with SLE from the Pregnancy and Autoimmune Rheumatic Diseases Clinic of CMN “La Raza” IMSS Mexico, and healthy pregnant women (controls) were included. All patients were under treatment with low doses of prednisone (< 15 mg/day) and chloroquine (150 mg /day). Peripheral blood was drawn for the PRL analysis by quimioluminiscense (IMMULITE 2000 Prolactin) and DCs: DC1 (monocytoid, immunogenic cells) and DC2 (plamocytoid, tolerogenic cells), by flow cytometer. These determinations were determined each trimester of pregnancy using specific antibodies. The PRL results were expressed in ng/dL and the DCs in percentages. The m-SLAM scale was used to measure the SLE activity each trimester of pregnancy (clinical activity: score ≥ 4). For the statistical analysis we used Student’s T test and Pearson correlation. The significant level was considered as p< 0.05. Results We included 13 pregnant women with SLE and 11 healthy pregnant women. The mean age of SLE patients was 25.67 ± 3.84 and 29.2 ± 5.3 years old in the control group. The m-SLAM was 0.62 (0-3). All SLE patients were in clinical remission. The mean DC1 for the SLE patients was 11.42 ± 9.4 vs. 56.15 ± 22.32 in healthy pregnant women (p

Background Vascular dysfunction usually is observed before clinically detectable fibrosis of systemic sclerosis (SSc). The eNOS catalyses the synthesis of nitric oxide (NO), which maintains basal vascular tone and endothelial function. Abnormal production of e-NOS and/or iNOS impairs NO availability causing vascular disease. Genetic polymorphism may participatein these alterations. Objectives To investigate vascular differences associated to polymorphisms T-786C and G894T of the eNOS gene on differential expression of eNOS/iNOS in the skin and vascular Duplex Sonography parameters of SSc patients. Methods We included 139 consecutive SSc patients. The genotyping of T-786C and G894T polymorphisms of eNOS gene was performed by Polymerase Chain Reaction Real-Time Assay. The control group included 180 age-matched healthy volunteers. For the eNOS/iNOS skin expression we included 31 patients (14 lcSSc and 17 dcSSc). Results In lcSSc: T-786C prevalence was TT65.5%, TC30.2% and CC4.3% (OR 1.8, IC 0.4–7.9 associated to SSc); and the G894T prevalence was GT74.3%, GT20.3% and TT5.4% (OR 1.94, IC 0.54–7.04 associated to SSc). In dcSSc: the T-786C prevalence was TT76.6%, TC20%, and CC3.3%, and G894T polymorphism was GT 82%, GT 18% and TT 0% withoutassociation with SSc. In control group: the prevalence of T-786C polymorphism was TT68.45%, TC29.4%, and CC2.22%; for G894T polymorphismwas GG74.1%, GT23.02%, TT2.87%. The mean relative expression of eNOS was 10.17+/-15.5 and iNOS of 7.6+/-13.05 in lcSSc. For dcSSc the mean relative expression of eNOS was 1.74+/-1.16 and iNOS of 2.1+/-2.5.1.08+/-0.3 and 1.36+/-0.7 in skin of volunteers of control+group. Left brachial intima-media thickness was statistically significantly greater in allelic variants of eNOS in SSc (p=0.025). Conclusions In the skin of both subtypes of systemic sclerosis, the relative expression of eNOS and iNOS is increased. Genetic polymorphisms of eNOS is associated with anormal intima-media thicknes in SSc patients. Disclosure of Interest None Declared

Background Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which vascular inflammatory lesions are limited to the brain and spinal cord. Diagnostic criteria include a newly acquired neurological deficit that is unexplained by other processes and angiographic or central nervous system (CNS) biopsy features of vasculitis. In contrast secondary central nervous system vasculitis (SCNSV) is vascular inflammation in which CNS involvement is due to diverse causes. Objectives To investigate the clinical, laboratory, neuroimaging characteristics and treatment in Primary Central Nervous System Vasculitis (PCNSV) versus Secondary Central Nervous System Vasculitis (SCNSV). Methods We studied twenty female patients with central nervous system vasculitis (CNS) during a period of 3 years in a tertiary level center. The clinical manifestations, laboratory, neuroimaging diagnosis and treatment were analyzed. Patients were divided in PCNSV and SCNSV. Biopsy CNS was performed in 3 patients. Results We studied 10 PCNSV and 10 SCNSV patients: systemic lupus erythematous 5, systemic sclerosis (SSc) 4, Sjogren syndrome (SS) 1. Mean age in the PCNSV was 26±5 years and SCNSV 47±7years. Clinical manifestation in PCNSV versus SCNSV were: headache 90% vs 60% (NS), fatigue 80% versus 40% ( Conclusions PCNSV had lower frequency of motor deficit, as well as lower ANA’s, and hypocomplementemia in comparison with SCNSV; an increase of CSF proteins was observed in all patients. MRI showed more periventricular and subcortical hyperintense lesions in SCNSV than PCNCV. All patients responded successfully to treatment with steroids plus cyclophosphamide. Early recognition and treatment may reduce poor outcomes. References Salvarini C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis: an update. curr Opin Rheumatol 2012;24:46-52 Salvarini C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, Giannini C, Meschia JF, Huston J3rd, Hunder GG Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007;62:442-51 Disclosure of Interest None Declared

Background Primary central nervous system vasculitis (PCNSV) is a challenging clinical problem due to itsnon- specific signs and symptoms, inaccessibility of the central nervous system (CNS) tissue for pathologic examination, lack of efficient non-invasive diagnostic tests and the relative rarity of it presentation. Secondary central nervous system vasculitis (SCNSV) occurs in association with autoimmune rheumatic diseases (ARD), infections, lymphoproliferative diseases, drug abuse, and systemic vasculitis. Objectives To compare the initial clinical, laboratory and imaging features in primary central nervous system vaculitis (PCNSV) versus secondary central nervous system vasculitis (SCNSV) and follow-up after treatment with intravenous cyclophosphamide (IV CYC) plus methylprednisolone (MP). Methods Neurological (focal and non-focal manifestations), laboratory (cerebrospinal fluid and immunological tests) and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Both groups received at onset MP plus IV CYC during 6 months, followed by bimonthly IV CYC plus oral glucocorticosteroids (CGS) for 12 months. All the patients were followed for 36 months. Results Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non- focal manifestations were similar in both groups (p=NS); headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy low leukocytes and platelets, elevatederythrocyte sedimentation rate, positive ANA, anti ds-DNA, ANCA, low complement, and rheumatoid factor were more frequent in SCNSV (p cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in both groups. After treatment, Kaplan-Meier survival curve showed higher relapse free survival in PCNSV (p Conclusions There are significant differences in the clinical manifestations, laboratory, and CSF findings between PCNSV and SCNSV. After treatment with IV CYC and GCS patients with PCNSV had higher relapse-free survival than SCNSV. Disclosure of Interest None Declared

Background Vascular dysfunction usually preceds clinically detectable fibrosis of systemic sclerosis (SSc). Vascular affection occurs more frequently in limited subtype of SSc (lcSSc) than diffuse subtype (dcSSc). The eNOS catalyses the synthesis of nitricoxide (NO), which maintains basal vascular tone andendothelial function. Abnormal production of e-NOS and/or iNOS impairs NO availability causing disease Objectives To investigate the prevalence of polymorphisms T-786C and G894T of the eNOS gene and the differential expression of eNOS/iNOS in the skin of SSc patients. Methods We included 139 consecutive SSc patients. The genotyping of T-786C and G894T polymorphisms of eNOS gene was performed by Polymerase Chain Reaction Real-Time Assay. The control group comprised 180 age-matched healthy volunteers. For the eNOS/iNOS skin expression we included 31 patients (14 lcSSc and 17 dcSSc). Results Revalence of scleroderma was 53.2% lcSSc (74/139) and46.7% (65/139) dsSSc. In control group: the prevalenceof T-786C polymorphismwas TT 68.45%, TC 29.4%, andCC2.22%;for G894T polymorphism was GG 74.1%, GT 23.02%, TT 2.87%.In lcSSc: T-786Cprevalence was TT 65.5%, TC 30.2% and CC 4.3% (OR 1.8, IC 0.4-7.9 associated to SSc); and the G894T prevalencewas GT 74.3%, GT 20.3% and TT 5.4% (OR 1.94, IC 0.54-7.04 associated to SSc).In dcSSc: the T-786Cprevalence was TT 76.6%, TC 20%, and CC 3.3%, and G894Tpolymorphism was GT 82%, GT 18% and TT 0% without association with SSc.The mean relative expression of eNOS was 10.17±15.5 and iNOS of 7.6±13.05 in lcSSc. For dcSSc the mean relative expression of eNOS was 1.74±1.16 and iNOS of 2.1±2.5. 1.08±0.3 y 1.36±0.7 in skin of volunteers of control group. Conclusions Genetic polymorphisms of eNOS were not arisk forpresenting systemic sclerosis in this sample.In the skin of both subtypes of systemic sclerosis, the relative expression of eNOS and iNOS is increased. However this increase was more meaningful in the lcSSc subtype of the disease. References Kahaleh B. vascular disease in scleroderma: mechanisms of vascular injury. Rheum Dis Clin North Am 2008;34:57-71. A. Dooley, S. Y. Low, A. Holmes, A. G. Kidane, D. J. Abraham, C. M. Black, and K. R. Bruckdorfer Nitric oxide synthase expression and activity in the tight-skin mouse model of fibrosis Rheumatology, 2008; 47: 272 - 280. Disclosure of Interest None Declared