Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.

BACKGROUND: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. METHODS: We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. RESULTS: One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. CONCLUSIONS: We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication.

Análisis de la relación entre la enfermedad pulmonar obstructiva crónica y los contaminantes atmosféricos atendiendo al origen y trayectoria de las masas de aire en el Norte de España / Relationship Between Chronic Obstructive Pulmonary Disease and Air Pollutants Depending on the Origin and Trajectory of Air Masses in the North of Spain

Objetivos: La enfermedad pulmonar obstructiva crónica es una enfermedad de alta prevalencia y una de las principales causas de muerte a nivel mundial. El presente trabajo analiza la relación de esta enfermedad con las variables meteorológicas y los niveles de contaminantes atmosféricos en Santander, atendiendo al origen y trayectoria de las masas de aire. Métodos: Se recogieron datos diarios de visitas a urgencias en el Hospital Marqués de Valdecilla de un periodo de 8 años, así como la concentración de los principales contaminantes atmosféricos y de las variables meteorológicas. Posteriormente se calcularon las retrotrayectorias con destino en Santander, a una altura de 1.500m sobre el nivel del terreno. Finalmente, se elaboró un modelo de correlación para evaluar el efecto de los contaminantes sobre las urgencias por EPOC. Resultados. Existe una asociación directa entre los niveles de PM10 y las urgencias por EPOC. Por cada 10 μg/m3 de aumento del contaminante, las urgencias incrementan un 3,34% (p = 0,00005), y el efecto se intensifica en las personas mayores de 74 años. Cuando los niveles de PM10 son dependientes de masas de aire procedentes del Sur, así como ante situaciones de recirculación, el efecto es mayor. Con el resto de contaminantes la relación con las urgencias no es estadísticamente significativa. Conclusiones: La exposición a PM10 provoca descompensaciones en los pacientes con EPOC. Atendiendo al patrón de circulación atmosférica se puede estimar si los niveles de PM10 van a ser elevados, y también se obtiene información sobre los componentes de las partículas (AU)

Objectives: Chronic obstructive pulmonary disease (COPD) is a common respiratory condition and one of the leading causes of death. Our aim was to analyze the association between emergency room visits due to this disease and meteorological variables and atmospheric contaminant levels in Santander, depending on the origin and trajectory of air masses. Methods: Data from emergency room visits at Hospital Marqués de Valdecilla were collected on a daily basis during an 8-year period. Data on concentrations of the main atmospheric pollutants and meteorological variables were also recorded. Retro trajectories leading to Santander at a height of1,500 meters above sea level were then calculated. Finally, a correlation model was produced to evaluate the effect of the contaminants on emergency visits due to COPD. Results: There is a direct association between PM 10 levels and the number of visits to the emergency room due to COPD. For every 10μg/m3 increase in pollutant levels, emergency visits increase by 3.34% (p = 0.00005), and this effect is enhanced in individuals over 74 years of age. This effect is heightened when PM10 levels depend on air masses from the South and when air recirculation occurs. There is no association between other pollutants and the number of visits to the emergency room. Conclusions: Exposure to high levels of PM10 causes exacerbations in COPD patients. By studying the atmospheric circulation pattern, we can predict whether PM10 levels will be inappropriately high, and we can also obtain information about the particle components (AU)

Trombocitopenia como factor de riesgo trombótico en pacientes con anticuerpos antifosfolipídicos sin criterios de enfermedad / Thrombocytopenia as a thrombotic risk factor in patients with antiphospholipid antibodies without disease criteria

Introducción: El síndrome antifosfolípido (SAF) es un trastorno inmunitario adquirido, definido por la presencia de trombosis (arterial y/o venosa) y/o morbilidad del embarazo junto con la presencia de anticuerpos antifosfolipídicos (aFL) positivos. Existe una relación clara entre los aFL y algunas manifestaciones no incluidas en los criterios clínicos, entre ellas, las hematológicas. Objetivos: a) estudiar la probabilidad de desarrollar SAF clínico en pacientes con aFL positivos y trombocitopenia; b) identificar posibles factores de riesgo para trombosis, y c) estudiar la asociación entre trombocitopenia y aFL. Métodos: Estudio retrospectivo de 138 pacientes con aFL positivos sin cumplir criterios clínicos de SAF. Se definió trombocitopenia como una cifra de plaquetas≤100.000/μl. Se excluyeron los pacientes con otras causas de trombocitopenia. Resultados: Diecisiete de los 138 (12%) pacientes incluidos en el estudio presentaban trombocitopenia. La cifra media de plaquetas fue de 60.000/μl. El riesgo para desarrollar trombocitopenia fue mayor en los pacientes fumadores (OR 2,8; p=0,044), en aquellos con anticoagulante lúpico (OR 13,5; p<0,001) y en los que tenían una mayor carga de aFL (OR 50,8; p<0,001). Tras un seguimiento medio de 146±60,3 meses, 5 pacientes con trombocitopenia (29,4%) desarrollaron trombosis. Conclusiones: En nuestra serie, la incidencia de trombocitopenia es del 12%. Los pacientes con aFL positivos que desarrollan trombocitopenia tienen un riesgo potencial de desarrollar trombosis. El tabaco podría ser un factor de riesgo para trombocitopenia. La carga de autoanticuerpos es un factor de riesgo para el desarrollo de trombocitopenia (AU)

Introduction: The antiphospholipid syndrome (APS) is an acquired immune disorder defined by the presence of thrombosis (arterial and/or venous) and/or pregnancy morbidity along with the presence of positive antiphospholipid antibodies (aPL). There is a clear relationship between aPL and some events not included in the clinical criteria, including haematologic. Objectives: a) to study the probability of developing clinical APS in patients with positive aPL and thrombopenia; b) to identify potential risk factors for thrombosis, and c) to study the association between thrombocytopenia and aPL. Methods: A retrospective study of 138 patients with positive aPL without fulfilling clinical criteria for APS. Thrombocytopenia was defined as a platelet count≤100,000/μl. Patients with other causes of thrombocytopenia were excluded. Results: Seventeen of the 138 (12%) patients in the study had thrombocytopenia. The mean platelet count was 60,000/μl. The risk of developing thrombocytopenia was higher in smokers (OR 2.8; P=.044), in those with lupus anticoagulant (OR 13.5; P<.001) and those with higher burden of aPL (OR 50.8; P<.001). After a mean follow-up of 146±60.3 months, 5 patients with thrombocytopenia (29.4%) developed thrombosis. Conclusions: In our series, the incidence of thrombocytopenia is 12%. aPL-positive patients who develop thrombocytopenia have a potential risk of developing thrombosis. Tobacco could be a risk factor for thrombocytopenia. Autoantibodies load is a risk factor for the development of thrombocytopenia (AU)