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espanolObjetivo Traduccion, transculturizacion y validez del cuestionario autoadministrado para funcionalidad (Systemic Sclerosis Questionnaire [SySQ]) en esclerosis sistemica al idioma espanol y su relacion con la enfermedad y la calidad de vida. Pacientes y metodos Estudio observacional analitico. Validacion realizada por panel de expertos, en apariencia y contenido. La metodologia incluyo: a) adaptacion al espanol del constructo por traduccion, retrotraduccion, y transculturizacion; b) consistencia interna para todas y cada una de las categorias del SySQ (alfa de Cronbach), y c) la reproducibilidad se evaluo tomando en cuenta todas las ocasiones en que se realizo la prueba con kappa de Cohen. Adicionalmente calculamos el coeficiente de correlacion de Spearman con: 1) escala de severidad de Medsger; 2) Health Assessment Questionnaire, y 3) prueba SF-36. Resultados Se incluyeron 70 pacientes con esclerosis sistemica, edad 17-78 (51 ± 12) anos, 65 (93%) mujeres, subtipo difuso/limitado 64/36%, evolucion de la enfermedad 0,5-40 anos. Observamos consistencia interna optima de la version final del SySQ (alfa de Cronbach 0,961) y buena reproducibilidad intraobservador entre pruebas con intervalo de 2 semanas (kappa de Cohen 0,618) y optima interobservador el mismo dia (kappa de Cohen 0,911). Moderada correlacion entre los cuestionarios de funcionalidad SySQ y de discapacidad de Health Assessment Questionnaire (r = 0,573; p Conclusiones Esta version en espanol del cuestionario autoadministrado SySQ es un instrumento valido para evaluar el estado funcional de pacientes con esclerosis sistemica. La menor funcionalidad esta relacionada con una mayor afeccion a tendon y vascular periferico y con una menor calidad de vida. EnglishObjective Translation, transculturation and validity of the self-administered questionnaire for functionality (Systemic Sclerosis Questionnaires [SySQ]) for use in Spanish patients with systemic sclerosis and its relationship to the severity of the disease and to quality of life. Patients and methods We conducted an observational analytical study to perform a cross-cultural validation of the self-administered questionnaire on functionality in scleroderma. The validity of the form and content was evaluated by an expert panel. The method included: a) adaptation into Spanish of the construct for translation and back translation, and transculturation; b) internal consistency with the SySQ (Cronbach's alpha), and c) reproducibility was assessed taking into account all occasions in which the test was performed with Cohen's kappa. Additionally, we calculated the Spearman correlation coefficient with the Medsger severity scale, Health Assessment Questionnaire score and SF-36 score. Results We included 70 patients with systemic sclerosis: age 17-78 (51 ± 12) years, 65 (93%) were women, diffuse/limited subtype 64/36%, disease duration of 0.5-40 years. Optimal internal consistency for all categories of the final version of SySQ (Cronbach's α of 0.961) and intraobserver reliability in 2 tests over a 2-week interval (Cohen's kappa coefficient 0.618) and optimal interobserver reliability in 2 tests on the same day (Cohen's kappa coefficient 0.911). Moderate correlation between functionality by SySQ and by Health Assessment Questionnaire (r = 0.573, P Conclusions SySQ in this validated Spanish version is a suitable instrument to measure functional status in patients with systemic sclerosis. Reduced functionality is related to greater tendon and peripheral vascular involvement and to a poorer quality of life.

In 2011, Shoenfeld Y and Agmon-Levin described five medical conditions with similar complex of symptoms and signs and a common pathogenesis, namely siliconosis, the Gulf War syndrome, the macrophagic myofasciitis syndrome, postvaccination phenomena, and the sick building syndrome. The authors proposed to gather these five entities under a common syndrome denominated autoimmune/inflammatory syndrome induced by adjuvants (ASIA). Genetically predisposed individuals, the exposure to environmental factors such as silicone, aluminum salts, adjuvant contained in vaccines, mineral oils, collagen, and hyaluronic acid and metal implants are a prerequisite to the appearance of ASIA. The epidemiology of ASIA is unknown; therefore, it is necessary to increase the international database. The immunogenetic, epigenetic, inflammatory mechanisms of each adjuvant and molecular mimicry should continue to be studied. The treatment of ASIA is in agreement with the clinical manifestations and in the identification of risk factors.

Calcinosis is a frequent complication of systemic sclerosis (SSc) that is usually located in extremities but may occur across the board. The aim of our study was to identify and quantify the distribution of calcinosis in a cohort of Mexican patients with SSc and its association with clinical features and autoantibodies. A cohort of patients with SSc (2013 ACR/EULAR criteria), classified in diffuse cutaneous (dcSSc) and limited cutaneous (lcSSc) (Le Roy criteria), was studied. For their analysis, patients were allocated into those with and without calcinosis (clinical and/or radiological). The evaluation included the modified Rodnan scale for skin and Medsger disease severity scale (DSS). Calcium, phosphorus, vitamin D, and parathyroid hormone (PTH) and antinuclear antibodies and extractable nuclear antigens were determined in serum. A total of 109 patients were included, 41 (37 %) with and 68 (63 %) without calcinosis. Calcinosis was more frequent in patients with dcSSc (55 vs 27 %). In total, we identified 354 sites with calcinosis and mean per patient of 12.0 ± 9.1; the most common sites affected were the hands (83 %), proximal upper extremity (27 %), and proximal lower extremity (22 %). Patients with calcinosis had a higher score of Rodnan scale, Mesdger DSS, and frequency of anti-nucleolar and anti-Scl-70 antibodies compared to those without calcinosis. Abnormal PTH elevation was found in 35 % of patients with calcinosis and 23 % without it. The prevalence of calcinosis is high in Mexican patients with SSc, especially in diffuse variety, and is associated with increased severity of disease.

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) encompassing conditions linked to previous exposure to an adjuvant substance. The clinical picture is very heterogeneous, from mild to severe manifestations, including death. However, the systematic analysis of severe ASIA cases has not been performed. The aim of this study was to systematically review the literature on severe ASIA cases. A systematic review of the literature was performed investigating severe ASIA cases. All publications were identified through PubMed, EMBASE, MEDLINE and Cochrane. Articles published from 2011 to 2016 were included. Severe ASIA was arbitrarily defined as follows: major organ involvement, life-threatening conditions, intensive treatment, disability, hospitalization and outcome (survival and death). Cases described before 2011 were excluded. From 2011 to 2016, we identified 4479 ASIA cases, of them 305 fulfilled arbitrary criteria of severe ASIA including our case presentation and 11 deaths. The majority of severe ASIA cases were related to HPV vaccine, silicone, influenza vaccine and mineral oil injections. The interval from exposition to severe manifestation was from 2 days to 23 years. (1) This is the first study that analyzes all cases published on ASIA with severe manifestations. (2) The current HPV vaccine is both effective and generally safe. However, it should be noted that severe autoimmune side effects have been reported in several studies. Severe ASIA may be observed after influenza vaccines, and other vaccines. (3) Efforts should be made to discover the connection between adjuvants, autoimmunity and autoimmune diseases, because there is an increase in cases severe and life-threatening of ASIA.

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 Adequate prenatal counseling in women with autoimmune rheumatic diseases (ARDs) may help minimize maternal-fetal complications. However, the available information about the frequency and quality of prenatal counseling given to these patients is limited. Objectives To estimate the prevalence of preconceptional counseling and associated factors in women with ARDs. Methods A survey was conducted on socio-demographic data, gynecological-obstetric history, and domains related to preconception counseling in women with ARDs at the reproductive stage. It was defined as adequate preconception counseling if the patient knew that the pregnancy should be medically planned, that the complications are associated with severity/activity of the disease, that they should use an effective contraceptive method and that their medications may or may not be used during pregnancy. Descriptive statistics, Student9s T test for quantitative variables with normal distribution, Mann-Whitney U test for non-normal and chi-square distribution were used for categorical or ordinal variables. Results Of a total of 146 surveys, 131 were analyzed. Only 49 (37.4%) patients received adequate preconception counseling. Two thirds of the patients had systemic lupus erythematosus (Table). The time of evolution of the disease was a factor associated with receiving adequate counseling (5.6 vs 3.6 years, p=0.023). Illness, marital status, and level of education were not factors associated with adequate counseling. The adoption of a contraceptive method and the number of pregnancies were not different between the two groups. The history of use of embryotoxic/teratogenic drugs was associated with a higher frequency of adequate counseling (79.6% vs 41.5%, p=0.001). According to the patients, adequate counseling was given by the rheumatologist in 75.5% of the cases (p=0.001). Conclusions Adequate preconceptional counseling in our patients with ARDs at the reproductive stage is deficient. A multidisciplinary strategy is required to improve the frequency and quality of preconception counseling in patients with ARDs. 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

Translation, transculturation and validity of the self-administered questionnaire for functionality (Systemic Sclerosis Questionnaires [SySQ]) for use in Spanish patients with systemic sclerosis and its relationship to the severity of the disease and to quality of life.We conducted an observational analytical study to perform a cross-cultural validation of the self-administered questionnaire on functionality in scleroderma. The validity of the form and content was evaluated by an expert panel. The method included: a) adaptation into Spanish of the construct for translation and back translation, and transculturation; b) internal consistency with the SySQ (Cronbach's alpha), and c) reproducibility was assessed taking into account all occasions in which the test was performed with Cohen's kappa. Additionally, we calculated the Spearman correlation coefficient with the Medsger severity scale, Health Assessment Questionnaire score and SF-36 score.We included 70 patients with systemic sclerosis: age 17-78 (51±12) years, 65 (93%) were women, diffuse/limited subtype 64/36%, disease duration of 0.5-40 years. Optimal internal consistency for all categories of the final version of SySQ (Cronbach's α of 0.961) and intraobserver reliability in 2 tests over a 2-week interval (Cohen's kappa coefficient 0.618) and optimal interobserver reliability in 2 tests on the same day (Cohen's kappa coefficient 0.911). Moderate correlation between functionality by SySQ and by Health Assessment Questionnaire (r=0.573, P.0001). Inverse correlation between SySQ and quality of life mental health domain SF-36 (r=-0.435, P.001) and physical domain SF-36 (r=-0.638, P.001). Medsger severity scale (tendon, heart, lung, vascular) also showed significant correlation with SySQ.SySQ in this validated Spanish version is a suitable instrument to measure functional status in patients with systemic sclerosis. Reduced functionality is related to greater tendon and peripheral vascular involvement and to a poorer quality of life.