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Pulmonary involvement in juvenile systemic sclerosis (JSSc) is rare in children and contributes to morbimortality. This study aimed to describe the pulmonary function and clinical, radiologic, and tomographic findings in JSSc.Patients with JSSc between 5-14 years of age were included. Clinical, functional, and imaging characteristics were assessed. Patients were excluded if they showed lung disease not associated with JSSc: mixed connective tissue disease, overlap syndrome, or acute cardiopulmonary failure at the time of the study. All patients underwent physical examination, electrocardiogram, spirometry, chest X-ray, high-resolution computed tomography (HRCT) of the chest, echocardiography, lung function tests, and the 6-minute walk test (6-MWT). Descriptive statistics were employed for data analysis.We studied 15 patients with the following characteristics: median age, 11 years; median since symptoms onset, 6 years; median since JSSc diagnosis and the finding of pulmonary involvement, 2 years. Lung disease was detected in 73%, interstitial lung disease (ILD) the most common affection (67%); pulmonary hypertension was found in 6.6%. 6-MWT was positive in 26.6%, forced vital capacity (FVC) was abnormal in 26.6%. No pulmonary involvement was found in four patients.The most frequent pulmonary affection in JSSc was ILD. Thus, early JSSc detection and periodic lung monitoring are mandatory to avoid further complications once JSSc is diagnosed.La afección pulmonar en la esclerosis sistémica juvenil (ESJ) es rara en niños y contribuye a la morbimortalidad. El objetivo de este estudio fue describir los hallazgos de función pulmonar, clínicos, radiológicos y tomográficos en la ESJ.Se incluyeron pacientes con ESJ de 5-14 años de edad. Se evaluaron las características clínicas, funcionales y de imagen. No se incluyeron pacientes con enfermedades pulmonares no asociadas con ESJ en el momento del estudio: enfermedad mixta del tejido conectivo, síndrome de superposición o insuficiencia cardiopulmonar aguda. Se realizaron exploración física, electrocardiograma, espirometría, radiografía de tórax, tomografía computarizada de alta resolución de tórax, ecocardiografía, pruebas de función pulmonar y prueba de caminata de 6 minutos (PC6M). Se utilizó estadística descriptiva para el análisis de los datos.Se estudiaron 15 pacientes con las siguientes características: mediana de edad, 11 años; mediana desde el inicio de los síntomas, 6 años; y mediana desde el diagnóstico de ESJ y hallazgo de afección pulmonar, 2 años. Se detectó enfermedad pulmonar en el 73%. La enfermedad pulmonar intersticial (EPI) fue la afección más común (67%) y se encontró hipertensión pulmonar en el 6.6%. La PC6M fue positiva en el 26.6%, y la capacidad vital forzada resultó anormal en el 26.6%. Cuatro pacientes no presentaron afección pulmonar.La afección pulmonar más frecuente en la ESJ fue la EPI. La detección temprana de ESJ y la monitorización pulmonar periódica son obligatorias para evitar más complicaciones una vez diagnosticada la ESJ.

Mexico is a country with a high frequency of type 2 diabetes mellitus (DM2). DM-related complications can increase the number of medical appointments and hospitalizations, impact patient quality of life, and exacerbate hospital care costs. The goals of this study were to analyze epidemiological data of DM2 over the last 10 years and to compare its comorbidities and mortality in individuals listed at the Instituto Mexicano del Seguro Social (IMSS) in 2013 and 2015. The database was compiled for doctors, validated by engineers in computer systems, and analyzed by statisticians. E-10-E-14 registered patients with DM2 (based on International Classification of Diseases (ICD-10)) from 2005, 2013, and 2015 were analyzed through the Family Medicine Units and Non-Communicable Diseases Analysis System (SANENT)® databases. Incidence, comorbidity, and mortality were described. We included 29,525,905 individuals, including 3,395,389 DM2 patients, in 2013, and 31,389,711 individuals, including 3,547,006 DM2 patients, in 2015, in the IMSS. The incidences of DM2 in 2005 and 2015 were 124 and 120 per 100,000 IMSS members, respectively, without significant differences. The diabetes complications caused partial disability in 253 and 341 per 100,000 individuals in 2013 and 2015, respectively. Overall mortality decreased from 2005 to 2015 (rates of 594 vs. 350 per 100,000 persons; p

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.

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.