Background:The Hemophagocytic Syndrome (HPS) had a mortality rate between 20% and 90%. The mortality of HPS secondary to autoimmune diseases (AID) is lower than hemato-oncological diseases (HOD). In general, the HOD, thrombocytopenia, age, and a prolongation of prothrombin are considered to be an adverse prognostic factor.(1)Objectives:To describe and identify differences between patients who survived and did not survive to HPS during hospital admission to a tertiary hospital between 2005 and 2019.Methods:This is a retrospective observational study. All patients who met the diagnostic criteria for LHH were included, or who presented haemophagocytic cells in the bone marrow biopsy, or who had diagnosis of HPS in the hospital discharge report.(2) Demographic, clinical, analytical, etiological, underlying disorder and prognosis variables were collected. Continuous variables are described with the mean or median according to the degree of normality. Kruskal Wallis, Fisher test and Mann-Whitney U test were used for the bivariate analysis, and also a multivariate logistic regression analysis was performed.Results:Thirty patients with HPS were included. They were distributed in 5 subgroups (Table 1). Overall mortality was 43.3%, statistically significant higher in the HOD [8 patients (66.7%); p 0.029]. Also, they were divided into 2 groups (survivor vs. non-survivor; Table 2). In the multivariate model the age and INR prolongation were confirmed to be independently associated with the outcome of mortality.Table 1.Etiology of HPSEtiologyn = 30MortalityAID10n = 1 Systemic lupus erythematosus51 Adult Still’s Disease3No Rheumatoid arthritis1No Sclerosing Disease IgG41NoHOD12n = 8* Non-Hodgkin’s lymphoma31 Myelodysplastic syndrome32 Acute leukemia33 Extranodal NK cell lymphoma11 Multiple Myeloma1No Probable lymphoproliferative process11Infectious diseases2n = 1 Pneumocystis carinii in patient with H.I.V.11 Campylobacter yeyuni1NoGlyoblastoma multiforme with temozolomida1n = 0HPS without defined aetiology53HIV: Human Immunodeficiency Virus, NK: Natural Killer. *p = 0,029Table 2.Characteristics and differences between survivor and non-survivor groupsTotalNon-survivorsurvivorn301317p<0,05Age55,5±18,36858,2-74,54034-570,043Women1653,3%761,5%947,1%1,00Comorbidities (≥ 2)516,7%215,4%317,6%1,000Hospital stay35,520-60,82915,5-39138-170,563Splenomegaly1653,3%753,8%952,9%1,000Hepatomegaly1033,3%538,5%529,4%0,705Hb (g/dL)7,16,4-7,9710%6,2-7,87,16,6-7,80,094Pt (x109/L)13 5005 000-52 50016 00011 000-44 00012 0005 000-99 0000,281Pt≤ 100 0002583,3%13100%1270,6%0,052Leu (x109/L)1 250238-3 1531 300150-3 9401 400200-3 3400,457Neu (x109/L)6150-1 5501 29020-3 3006500-1 4000,805Fb (mg/dL) (n=24)171111-358167,00106-253169,00103-4510,796Fer (ng/mL) (n=28)15 3305 434-38 28429 0635 728-74 60413 2258 287-28 7290,108Tg (mmol/L)341226-438254,00184-382471,00341-6040,053GOT (U/L)13977,5-406,3133,00101-513179,00101-512,50,483GPT (U/L)16246-389109,0041-333199,0099-2980,198INR (n=29)1,51,1-1,92,11,2-3,71,51,1-1,60,028Hb: Hemoglobin, Pt: platelets, Leu: leukocytes, Neu: neutrophils, Fb: fibrinogen, Fer: ferritin, Tg: triglycerides, GOT: aspartate aminotransferase, GPT: alanine aminotransferaseConclusion:The HOD presented higher mortality. The non-survivor group presented a longer INR prolongation and a higher age at the time of diagnosis.References:[1]Parikh SA. Prog. factors and outcomes of adults with HLH. Mayo Clin Proc. 2014;89:484–492.[2]Henter JI. HLH-2004: Diag. and therapeutic guidelines for HLH.Pediatr Blood Cancer. 2007;48:124.Disclosure of Interests:César Antonio Egües Dubuc: None declared, Andrea De Diego: None declared, Patricia Cabrera Miranda: None declared, Nerea Alcorta Lorenzo: None declared, Jesús Alejandro Valero Jaimes: None declared, Jose Ramon Furundarena Salsamendi: None declared, Olga Maiz-Alonso: None declared, Luis Maria Lopez Dominguez: None declared, Esther Uriarte Isacelaya: None declared, Jorge Jesús Cancio Fanlo: None declared, Jaime Calvo Grant/research support from: Lilly, UCB, Consultant of: Abbvie, Jansen, Celgene, Joaquin Maria Belzunegui Otano: None declared

A941for the management of rheumatoid arthritis (RA). Several studies have demonstrated efficacy and tolerability of conventional DMARDs, resulting in control of disease activity and improvement of functional disability. The aim of this study was to describe the effectiveness of leflunomide and methotrexate in patients with RA in a real-life cohort in Colombia. Methods: A descriptive cross-sectional study was performed. We included patients receiving leflunomide or methotrexate as monotherapy or in combination. Clinical follow-up was designed by the authors according to DAS28; therapy had to be adjusted with DAS28 > 3.2 unless patient´s conditions don't permit it. We divided patients in two groups: remission-low disease activity (Rem/LDA) patients and moderate-severe disease activity (MDA/SDA). Descriptive epidemiology was done, percentages and averages were calculated; the mean of each variable was analyzed using t-Student and the level of disease activity was analyzed using Pearson´s statistics. Results: We included 3949 patients were 56% were receiving methotrexate in monotherapy, 21% received leflunomide in monotherapy and 23% received combined therapy. Mean age was 59 years ± 12 years old, 83% were female and 17% male. In the group of patients receiving only methotrexate 55% achieved remission and 18% LDA. In the group receiving only leflunomide 45% achieved remission and 20% LDA. Finally in the group receiving combined therapy 44% achieved remission and 19% LDA. There was statistical significance between disease activity and therapy. ConClusions: Leflunomide and/or methotrexate are effective for treatment of RA. In settings and countries like Colombia were there are limited resources the accurate use of this medications are a cost-effective option for disease management.

Background:Biological therapies have revolutionized the management of rheumatic diseases. This study analyzes the paradoxical autoimmune inflammatory manifestations that these therapies can induce. They are described as paradoxical because these are diseases in which the same biological drugs have proven effective.Objectives:The aim of this study is to carry out a descriptive analysis of paradoxical biologics-induced autoimmune manifestations.Methods:A retrospective research was carried out during January 2017 and March 2018 at Hospital Universitario Donostia.Computerized medical records were reviewed. The following variables were recorded: underlying disease; type of developed manifestation; the triggering biologic drug and its duration; concomitant treatment with disease-modifying antirheumatic drugs (DMARDs), previous biological treatments; the adopted measure and the resolution of the complication or not.Qualitative variables are recorded in absolute value and in percentage. The quantitative variables are recorded with the mean and standard deviation. The data has been analyzed with the SPSS Statistics 20 program.Results:Twenty-six cases were analyzed. The most common triggering drugs were infliximab (30.8%), adalimumab (30.8%) and etanercept (15.4%). The most common underlying diseases were spondyloarthritis (42.3%), rheumathoid arthritis (34.6%) and Crohn’s disease (11.5%). The most developed complications were cutaneous affectations (76.9%), psoriasis specifically.Statistical significative difference regarding the molecules causing the complication, has only been found in the previous use of biologic drugs (p 0.003), number of treatment dropouts (p 0.048) and number of resolved complications (regardless of the adopted measure) (p 0.041).Conclusion:Infliximab and adalimumab are the drugs that presented the most paradoxical manifestations, probably because they are the oldest and most used ones. The difference found in previously used biologics, could be due to the fact that new drugs are less frequently presented as the first option. Psoriasis is the most frequently developed complication. The difference found in the number of abandonments of the biologic treatment and the solution of the complication might be more associated with the complication itself, rather than with the responsible molecule. There is no consensus on the measures to be taken when facing the complication. The severity of the manifestation should be assessed, as well as the control of the underlying disease. Most complications resolve overtime.Table 1Infliximabn 8 (31.8%)Adalimumabn 8 (30.8%)Etanerceptn 4 (15.4%)Certolizumabn 2 (7.7%)Golimumabn 2 (7.7%)Abataceptn 1 (3.8%)Tocilizumabn 1 (3.8%)Totaln 26 (100.0%)p valueUnderlying disease0.789Rheumatic (%)6 (75.0)5 (62.5)4 (100.0)2 (100.0)2 (100.0)1 (100.0)1 (100.0)21 (80.0)Digestive (%)2 (25.0)3 (37.5)000005 (19.2)Type of developed manifestation0.235Hematologic (%)01 (12.5)000001 (3.8)Neurologic (%)2 (25.0)0000002 (7.7)Digestive (%)00001 (50.0)001 (3.8)Cutaneous (%)6 (75.0)6 (75.0)4 (100.0)2 (100.0)1 (50.0)01 (100.0)20 (76.9)Other (%)01 (12.5)0001 (100.0)02 (7.7)Table 2Infliximabn 8Adalimumabn 8Etanerceptn 4Certolizumabn 2Golimumabn 2Abataceptn 1Tocilizumabn 1Totaln 2p valueThe triggering biologic drug and its duration (months)43.25(SD 44.81)10.9375.50(SD 66.08)4.5011.006.005.0030.660.635Concomitant treatment with DMARDs (%)2 (25.0)2 (25.0)1 (25.0)1 (50.0)001 (100.0)7 (26.9)0.811Previous biological treatment (%)02 (25.0)1 (25.0)2 (100.0)2 (100.0)1 (100.0)1 (100.0)9 (34.6)0.003Suspension as the adopted measure (%)5 (62.5)7 (87.5)02 (100.0)1 (50.0)1 (100.0)1 (100.0)17 (65.4)0.048Resolution of the complication (%)3 (37.5)8 (100.0)1 (25.0)1 (50.0)1 (50.0)1 (100.0)1 (100.0)16 (64.0)0.041Disclosure of Interests:None declared

Background:Giant cell arteritis (GCA) presents with visual symptoms in approximately 37%1of patients at diagnosis. Patients with visual symptoms present, according to some series, lower levels of C-reactive protein (CRP) at diagnosis and associate less headache, fever, cranial nodules or polymyalgia (PM).Relapses despite glucocorticoid treatment (GC) are frequent (40-60%). Visual symptoms are very rare in relapses (0% in several series). Since most series focus on northern European populations, it is interesting to study the characteristics in our population.Objectives:To describe visual manifestations in both diagnosis and recurrence of stroke, only in patients diagnosed with temporal artery biopsy. To analyze demographic, clinical and analytical features in patients with and without visual symptoms.Methods:We retrospectively reviewed the medical records of patients with positive biopsy for GCA at our center from January 2000 through December 2018. Relapse was defined as the appearance of clinical symptoms in a previously asymptomatic patient requiring a dose increase or restart of GCS. Patients with no response to GCS were not included. Qualitative variables are shown with absolute value and percentage and quantitative variables with mean and standard deviation (SD). Kruskal Wallis, Fisher test and Mann-Whitney U test were used for bivariate analysis.Results:52 patients were found: 39 women (73.6%), with an average age at diagnosis of 77.6 years (SD 6.3). At diagnosis, 28 presented visual symptoms (53.84%): oculomotor paralysis 5 (9.61%), amaurosis fugax 8 (15.38%), blindness 6 (11.54%), decreased visual acuity 8 (28.57%) and other visual symptoms 1 (1.92%). Eleven had monocular symptoms (38%) and 9 binocular (32%), in 10 patients this data was not collected. The symptoms were permanent in 11 (39.2%) despite GC. Type of visual impairment was: Anterior Ischemic Optic Neuropathy (AION) (12, 42.86%), impairment of cranial pairs (5, 17.86%), central retinal artery occlusion (1, 3.57%), cilioretinal artery occlusion (1, 3.57%) and Posterior Ischemic Optic Neuropathy (1, 3.57%).Of the 52 patients, 17 (32.69%) presented a minimum of one recurrence and 3 (5.77%) presented 2 or more. No patient relapsed with visual clinic.There were no differences among patients with and without visual symptoms in the variables studied (table 1). However, patients with visual symptoms at diagnosis had numerically less PM and cranial nodules.Table 1.Demographic, clinical and analytical differences between patients with/without visual symptomsVariablesWith visual symptoms (28)Without visual symptoms (24)ESR >5017 (60.7%)15 (62.5%)ESR <504 (14.3%)2 (8.33%)CRP>1017 (60.7%)13 (54.16%)CRP <103 (10.7%)2 (8.33%)Cephalea24 (85,7%)23 (95,8%)Polymyalgia9 (32,1%)12 (50%)Constitutional syndrome9 (32,1%)7 (29,1%)Jaw claudication17 (60.7%)12 (50%)Cranial nodules4 (14,3%)8 (33,3%)Sex: Man/Woman9/195/19Previous PM diagnosis4 (14,3%)3 (12,5%)Conclusion:In our series, 53% of the patients presented visual symptoms at diagnosis, a number higher than that described in the literature. It is important to remember that only patients with biopsy-confirmed ACG were included. The most frequent manifestations were AION followed by oculomotor paralysis. A numerically lower percentage of PM and cranial nodules was observed at diagnosis in patients with visual symptoms compared to patients without them, as seen in some series. The absence of visual clinic in recurrences coincides with that reported in the available literature.References:[1]Alba M et al. Relapses in Patients With Giant Cell Arteritis Prevalence, Characteristics, and Associated Clinical Findings in a Longitudinally Followed Cohort of 106 Patients. Medicine. 2014;93: 194–201.Disclosure of Interests:None declared

Background:Despite therapeutic advances in recent years, methotrexate (MTX) remains the gold standard for the treatment of rheumatoid arthritis (RA). Among the side effects that have been blamed on it are liver fibrosis (LF) and cirrhosis, although late studies have failed to show such a relation1,2. The only validated test in the diagnosis of LF is biopsy. Given the relevance of MTX in the treatment of RA, it is important to evaluate non-invasive diagnostic options for LF such as transitional elastography (FibroScan, FS).Objectives:To evaluate the percentage of LF in RA patients treated with MTX. Secondly, to assess the correlation between altered liver function, RA activity, and LF. To determine whether dose and/or duration of treatment with MTX may affect the development of LF in such patients.Methods:We did a prospective study between February 2019 and January 2020. Patients affected of RA treated with MTX were included. Patients with basal liver disease (hepatitis B, hepatitis C and steatohepatitis), alcohol consumption, type I diabetes mellitus, chronic renal failure, heart failure, obesity and concomitant treatment with leflunomide or antiretrovirals were excluded. Demographic, clinical, analytical and therapeutic variables were collected. Liver fibrosis was assessed by FS in kilopascals (kpa) and using the APRI score. RA activity was assessed by DAS28 score. Continuous variables are described with mean and standard deviation (SD), and qualitative variables are shown with absolute value and percentage. Spearman’s and Mann-Whitney’s U tests were used for the bivariate analysis.Results:Fifty patients were included (Table 1 and 2). Of these, 38 were women (76%) with mean age of 61.8 years (SD 11.7) and mean RA evolution time of 13.7 years (SD 8.2). The mean DAS28 at the visit was 2.39 (SD 1.1). The FS showed an average of 4.8 kpa (SD 2). The mean duration of treatment with MTX was 85.8 months (SD 93.3) and that of AD-MTX was 5414.6mg (SD 5011). Patients were divided into those with DA-MTX greater than 4000mg (21, 42%) and less than 4000mg (29, 58%) and no significant differences were found in terms of LF in FS (p 0.637) or APRI scale (p 0.806). No significant differences were found in terms of treatment duration either. Six patients (12%) had elevated aspartate aminotransferase (AST) and 9 (18%) had elevated alanine aminotransferase (ALT). No significant difference was found in FS values in relation to ALT, but it was with elevated AST (p 0.021). Similarly, differences were found in APRI based on AST (p 0.045). Metabolic syndrome was collected in 4 patients (8%) without significant differences with FS or APRI values. There were no significant differences in LF depending on gamma-glutamyl transpeptidase (GGT) values.Conclusion:FS and APRI score are useful for the determination of LF in RA patients treated with MTX. There is no evidence of a relationship between AD-MTX and LF by FS or APRI. AST values may be related to the presence of fibrosis as determined by FS or APRI. and the presence of the metabolic syndrome are not.References:[1]G.L. Erre, et al. Methotrexate therapy is not associated with increased liver stiffness and significant liver fibrosis in rheumatoid arthritis patients: A cross-sectional controlled study with real-time two-dimensional shear wave elastography. European Journal of Internal Medicine 69 (2019) 57–63. Internet.[2]R. Conway et al. Risk of liver injury among methotrexate users: a meta-analysis of randomised controlled trials. Semin Arthritis Rheum 2015 Oct;45(2):156–62. Internet.Disclosure of Interests:None declared