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BackgroundSystemic Lupus Erythematosus (SLE) is an autoimmune systemic rheumatic disease that, in our area, presents hematologic manifestations in approximately 70% of cases1. Some of them are very rare so there are no large series whose analysis could provide relevant information.ObjectivesTo study the characteristics of patients with Hemophagocytic Syndrome (HS) in a large sample of SLE patients.MethodsSLE patients from RELESSER database were studied. We analysed SLE manifestations present at 12 different domains (mucocutaneous, renal, musculoskeletal, constitutional, hematologic, vascular, cardiac, respiratory, neuropsychiatric, gastrointestinal, ophthalmic and serological) before, during and after HS diagnosis and until the last available assessment. We also studied activity (SELENA-SLEDAI) and damage (SLICC/ACR DI) indices at those moments.We evaluated the treatment received, HS recurrences and the number of deaths by this entity.Results3,656 SLE patients (≥4 ACR criteria) from 45 Rheumatology Units across Spain were studied. Seven patients (<0.5%) with HS were identified. 71.4% were women, with a mean age (± S.D.) at SH diagnosis of 35.1 (± 17.1) years. In 5 of the 7 cases the HS occurred 115.5 (± 162.9) months after SLE diagnosis. In the other 2 cases the diagnosis of both entities was simultaneous. The main triggers of HS were infections, followed by SLE activity flares. At the time of HS diagnosis, they had high SLE activity with a mean SLEDAI score of 13.1 (± 11.3) and 1.4 (± 2.3) SDI score.Clinically, 100% of the patients presented fever and alterations of the liver profile, 85.8% cytopenias and 71.5% dermatological manifestations. Respiratory manifestations and hemolytic anemia were present in 57.2% of the cases, both; lymph nodes and coagulopathy in 42.9%. Hepatomegaly was detected in 28.6%, as well as neuropsychiatric, digestive and renal manifestations. Splenomegaly was detected in 14.3%. The mean hemoglobin level was 8.6 (± 1.1) g/dl, platelets 85,585 (± 83,390)/mm3, ferritin 7,410 (± 6,470) ng/ml and triglycerides 404.7 (± 235.6) mg/dl. All patients were admitted and undergonebone marrow study, requiring a mean of 2.2 (± 1.5) treatment lines, using 2.8 (± 1.7) drugs. One patient died during the HS episode by the HS itself and another 2 patients had 2 and 3 recurrences, respectively.The following table shows the characteristics of each patient.ConclusionsHS is a rare life-threatening SLE manifestation. It must be suspected in SLE patients with persistent fever who do not respond to antibiotics, cytopenias and evidence of multiorgan involvement. Relapses and death are common in HS associated to SLE.References Pego-Reigosa JM, Rua-Figueroa I, Lopez-Longo FJ, Galindo-Izquierdo M, Calvo-Alen J, Olive-Marques A, et al. Analysis of disease activity and response to treatment in a large Spanish cohort of s with systemic lupus erythematosus. Lupus 2015;24:720–9. AcknowledgementsWork supported by Spanish Society of Rheumatology, FIS/ISCIII (PI11/02857), BIOCAPS from the European Union 7th Framework Programme...

Epidemiological characteristics Sex (female), n (%) 473 (94.4) Ethnicity, caucasian (%) 456 (93.6) Age at disease diagnosis, mean (SD) Age>50 years, n (%) 134 (15.4) Smoke Non-smoker, n (%) 274 (59.4) Ex-smoker, n (%) 120 (26.0) Smoker, n (%) 67 (14.5) Oral contraceptive Non-oral contraceptive, n (%) 258 (69.0) Exoral contraceptive, n (%) 107 (28.6) Oral contraceptive, n (%) 9 (2.4) Family history , n (%) 60 (15.7) Clinical characteristic Disease duration (months), median(p75-p25) 212.0 (120.8-289.0) Malar rash, n (%) 253 (50.8) Discoid rash, n (%) 94 (19.1) Photosensitivity, n (%) 293 (59.7) Oral ulcers, n (%) 218 (44.4) Arthritis, n (%) 393 (79.4) Pleuritis, n (%) 105 (21.3) Pericarditis, n (%) 86 (17.3) Renal disorder, n (%) 132 (26.6) Neurological disorder, n (%) 45 (9.1) hemolytic anemia, n (%) 49 (9.9) leukopenia, n (%) 293 (59.3) thrombocytopenia, n (%) 106 (21.8) Sjögren's syndrome, n (%) 127 (25.7) Antiphospholipid syndrome, n (%) 74 (14.9) mixed connective tissue disease, n (%) 14 (2.7) Raynaud's phenomenon, n(%) 226 (45.8) Lupus Nephritis, n (%) 131 (26.5) Pulmonary fibrosis, n (%) 25 (5.0) Pulmonary hypertension, n (%) 17 (3.4) Laboratory characteristic ANA+, n (%) 497 (99.0) Anti-DNA+, n (%) 350 (71.0) Anti-sm +, n (%) 110 (22.8) Anti-RNP, n (%) 164 (34.1) Anti-ro, n (%) 193 (39.9) Anti-la, n (%) 104 (21.4) AL+, n (%) 70 (20.3) Therapeutic regimen Glucocorticoids, n (%) 439 (91.1) Methotrexate, n (%) 120 (24.7) Hydroxychloroquine, n (%) 369 (76.7) Azathioprine, n (%) 173 (36.0) Cyclophosphamide, n (%) 95 (19.7) Mycophenolate mofetilo, n (%) 60 (12.4) Rituximab, n (%) 44 (9.1) Inmunoglobulin, n (%) 23 (4.8) Abstracts

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Background Autoimmune rheumatic diseases are associated with increased cardiovascular mortality compared to the general population Objectives To evaluate the impact of an intervention carried out by the rheumatologist and nurse in smoker patients. Methods We selected consecutive active smokers attended at the rheumatology clinics with rheumatoid arthritis, spondyloarthritis or connective diseases. The intervention consisted of two phases: 1) A baseline visit which included the rheumatologist advice on the benefits of quitting smoking, oral and written in a specific information form for rheumatic patients. Patients completed a questionnaire that included smoking-dependence tests and previous quit attempts. 2) A telephone follow-up visit at the 3rd month done by the rheumatology nurse, which included oral reinforcement, the offer of an additional visit and the possibility of pharmacological treatment to help quitting. The primary outcome variable was the percentage of smokers with total abstinence in the last 7-days of a phone interview at 3, 6 and 12 months. The secondary endpoint was a reduction in cigarette consumption by at least 50%. Results 945 patients participated in the study, 233 (24.7%) were former smokers and 185 (19.5%) were current smokers. In the last five years, 55 patients quit smoking, sothe annual withdrawal rate was 4.6%. The smoking cessation rate in 152 participants was 11.8% at 3 months, 14.4% at 6 months and 15.7% at 12 months (Table). The OR at 12 months compared with the previous smoking cessation rate was 3.8 (CI 95%: 1.8-8.1). 29 patients (19%) reduced ≥ 50% of the cigarette consumption at 12 months. In a linear regression analysis in which the dependent variable was smoking cessation at 12 months, a score of less dependence (p=0.03) and previous quit attempts (p=0.04) were significantly associated with definitive smoking cessation. Conclusions An educational program for smoker patients, which includes oral and written advice by the rheumatologist and the nurse, makes that one out of six patients cease to smoke at 12 months. As far as we know, this is the first interventional study in smokers with arthritis. It has the advantage of a simple design that can be easily integrated into routine daily practice. Disclosure of Interest None Declared