Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.
Multi-year sets of ground-based sun-photometer measurements conducted at 12 Arctic sites and 9 Antarctic sites were examined to determine daily mean values of aerosol optical thickness () at visible and near-infrared wavelengths, from which best-fit values of Ångström"s exponent were calculated. Analysing these data, the monthly mean values of (0.50 µm) and and the relative frequency histograms of the daily mean values of both parameters were determined for winterspring and summer-autumn in the Arctic and for austral summer in Antarctica. The Arctic and Antarctic covariance plots of the seasonal median values of versus (0.50 µm) showed: (i) a considerable increase in (0.50 µm) for the Arctic aerosol from summer to winter-spring, without marked changes in ; and (ii) a marked increase in (0.50 µm) passing from the Antarctic Plateau to coastal sites, whereas decreased considerably due to the larger fraction of sea-salt aerosol. Good agreement was found when comparing ground-based sun-photometer measurements of () and at Arctic and Antarctic coastal sites with Microtops measurements conducted during numerous AERONET/MAN cruises from 2006 to 2013 in three Arctic Ocean sectors and in coastal and offshore regions of the Southern Atlantic, Pacific, and Indian Oceans, and the Antarctic Peninsula. Lidar measurements were also examined to characterise vertical profiles of the aerosol backscattering coefficient measured throughout the year at Ny-Ålesund. Satellite-based MODIS, MISR, and AATSR retrievals of () over large parts of the oceanic polar regions during spring and summer were in close agreement with ship-borne and coastal ground-based sun-photometer measurements. An overview of the chemical composition of fine and accumulation/coarse mode particles is also presented, based on in-situ measurements at Arctic and Antarctic sites. Fourteen log-normal aerosol number size-distributions were defined to represent the average features of fine and accumulation/coarse mode particles for Arctic haze, summer background aerosol, Asian dust and boreal forest fire smoke, and for various background austral summer aerosol types at coastal 4 and high-altitude Antarctic sites. The main columnar aerosol optical characteristics were determined for all particle modes, based on in-situ measurements of the scattering and absorption coefficients. Diurnally averaged direct aerosol-induced radiative forcing and efficiency were calculated for a set of multimodal aerosol extinction models, using various Bidirectional Reflectance Distribution Function models over vegetation-covered, oceanic and snow-covered surfaces. These gave a reliable measure of the pronounced effects of aerosols on the radiation balance of the surface-atmosphere system over polar regions.
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