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Background: Systemic sclerosis (SS) is an autoimmune disease of unknown etiology, characterized by the presence of fibrosis and vasculopathy in skin and multiple internal organs such as the lungs, the kidneys and the digestive tract. The course of the disease is unpredictable and could remain relatively stable or have a rapid evolution. Multiple studies have been carried out to determine the clinical characteristics and survival prognosis on SS patients. Objectives: To analyze the demographic characteristics, clinical features, treatment and prognosis in a SS disease cohort. Methods: We performed an observational and retrospective study of patients whit SS. The patients had been attended by the Rheumatology department of a tertiary care hospital. We collected demographic, clinical and analytical variables, as well as treatment and prognosis. We classified the disease employing the LeRoy and Medsger, VEDOSS criteria and 2013 aCR/EULAR criteria. Results: Of our 43 patients, 36 (83.7%) were female and only 7 (16.3%) were male. The average age was 60.4 years (SD 15.6), the average age at diagnosis was 53.3 years (SD 17.6) and the mean time of evolution of the disease was 7.9 years (SD 6.3). Of all the patients, 3 patients (6.9%) died, the mean age at death being 53.6 years (SD 23.7) and the mean time from diagnosis to death of 19 years (SD 10.1). The most frequently ocurring presentation was limited SS (with 18 patients (41.9%), followed by preescleroderma with 14 patients (32.6%), diffuse SS with 6 patients (14%) and escleroderma sine escleroderma with 2 patients (4.7%). Three patients (7%) were labeled with MCTD. SS was associated with other autoimmune diseases in 20% of patients. Five (11.6%) patients developed neoplasms throughout the course of the study. The rest of the clinical characteristics are listed in Table 1 and 2, as well as the strength of association of these with the type of SS, calculated using chi square and Fischer test. Conclusion: Worthy of noting in our cohort is the absence of scleroderma in more than 40% of our patients, probably because the new criteria have allowed us to diagnose the disease at an earlier stage and also due to the scarce frequency of puffy fingers with respect to other larger series. Digestive involvement was the most frequent visceral manifestation, followed by pulmonary manifestations, specifically interstitial lung disease (ILD). Despite the small sample size, lung disease was significantly associated with the two forms of systemic sclerosis, but not with the antibody pattern. Both the ILD and the pulmonary arterial hypertension (PAH) were more frequent in patients with SSd. Mortality in all cases was due to interstitial lung involvement. As is frequently described, SS is associated with other systemic autoimmune diseases, constituting an overlap syndrome. References [1] Canete Crespillo, J. (2008). Manual SER de las enfermedades reumaticas. Madrid: Editorial Medica Panamericana [2] Dougherty, D., Kwakkenbos, L., Carrier, M., Salazar, G., assassi, S., Baron, M., Bartlett, S., Furst, D., Gottesman, K., van den Hoogen, F., Malcarne, V., Mouthon, L., Nielson, W., Poiraudeau, S., Sauve, M., Boire, G., Bruns, a., Chung, L., Denton, C., Dunne, J., fortin, P., Frech, T., Gill, a., Gordon, J., Herrick, a., Hinchcliff, M., Hudson, M., Johnson, S., Jones, N., Kafaja, S., Larche, M., Manning, J., Pope, J., Spiera, R., Steen, V., Sutton, E., Thorne, C., Wilcox, P., Thombs, B. And Mayes, M. (2018). The Scleroderma Patient-Centered intervention Network Cohort: baseline clinical features and comparison with other large scleroderma cohorts. Rheumatology, 57(9), pp.1623-1631. [3] CLINICAL FEATURES aND PROGNOSTIC FACTORS for SYSTEMIC SCLEROSIS RELATED inTERSTITIAL LUNG DISEASE. (2018). Respirology, 23, pp.155-155. Disclosure of interests: None declared

Background: Behcet’s disease (BD) is a variable vessel vasculitis and typically presents with mucocutaneous involvement. However, any organ can be affected, being the neurological affectation (neurobehcet, NB) one of the most serious manifestations. Objectives: Our aim was to assess the efficacy and safety of biological therapy as treatment of NB. Methods: We set up a multicenter observational study of 29 patients with NB on treatment with biological therapy (BT). NB diagnosis was made by neuroimaging, CSF analysis and/or suggestive clinical signs of central and/or peripheral nervous system involvement, excluding infectious causes or more prevalent pathology. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate. Results: 29 patients (15♂/14♀) with an average age of 39.6 ± 10.5 years. HLA-B51 was positive in 48.3% of the patients. Table shows the non-neurological manifestations. Regarding the neurological manifestations, 23 patients (79.3%) had parenchymal involvement (hemiparesis (n=6), brainstem involvement (n=1), encephalopathy (n = 4), optic neuropathy (n=3), dysphasia (n=1), polyneuropathy (n=6), cognitive impairment (n=4), and non-steroidal psychosis (n=1), while the remaining 6 patients (20.7%) presented aseptic meningitis as a non-parenchymal affectation (Table). Prior to BT, patients had received the following treatment: oral prednisone (n=27), methylprednisolone bolus (n=9), CsA (n=8), AZA (n=16), MTX (n=14) and mycophenolate (n=2). After a median of 31 [10-60] months since the beginning of the neurological symptoms, the following BT was initiated: infliximab (IFX)(n=17), adalimumab (ADA)(n=7), tocilizumab (TCZ) (n=2), golimumab (GOL) (n=2) and Etanercept (ETN) (n=1). A first switch to ADA was necessary in 8 patients with IFX due to primary failure. In addition, 2 of them needed a second switch to TCZ, getting a partial response. The BT was discontinued in 5 patients, 2 of them for obtaining clinical remission and the remaining 3 for inefficacy. After a median follow-up of 5.4±4.6 years, complete response was obtained in 15 patients, partial response in 11 and no response in the remaining 3. We observed an anaphylactic reaction and psoriasis induced by IFX, without other serious adverse events (Table). Conclusion: BT, especially anti-TNF, seems effective and safe for treatment in NB. Disclosure of Interests: Jose Luis Martin-Varillas: None declared, Inigo Gonzalez-Mazon: None declared, Belen Atienza-Mateo: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Lara Sanchez Bilbao: None declared, Vanesa Calvo-Rio: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Esther Vicente: None declared, Olga Maiz: None declared, Clara Moriano: None declared, Elvira Diez Alvarez: None declared, Jose Luis Andreu Sanchez: None declared, Concepcion Delgado Beltran: None declared, Marta Loredo Martinez: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Angel Ramos Calvo: None declared, Francisca Sivera: None declared, Enrique Raya: None declared, Norberto Ortego: None declared, Jose Luis Callejas-Rubio: None declared, Anahy Brandy-Garcia: None declared, Alejandro Olive: None declared, Sabela Fernandez: None declared, Ricardo Gomez de la Torre: None declared, Ignacio Torre-Salaberri: None declared, Julio Sanchez: None declared, ANA URRUTICOECHEA-ARANA: None declared, Eva Salgado-Perez: None declared, Rafael Melero: None declared, Olga Martinez Gonzalez: None declared, Susana Romero-Yuste: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen