Matches in SemOpenAlex for { <https://semopenalex.org/work/W2943217368> ?p ?o ?g. }
- W2943217368 abstract "Localized scleroderma (LS) is a complex disease characterized by a mixture of inflammation and fibrosis of the skin that, especially in the pediatric population, also affects extracutaneous tissues ranging from muscle to the central nervous system. Although developmental origins have been hypothesized, evidence points to LS as a systemic autoimmune disorder, as there is a strong correlation to family history of autoimmune disease, the presence of shared HLA types with rheumatoid arthritis, high frequency of auto-antibodies, and elevated circulating chemokines and cytokines associated with T-helper cell, IFNγ and other inflammatory pathways. This inflammatory phenotype of the peripheral blood is reflected in the skin via microarray, RNA Sequencing and tissue staining. Research is underway to identify the key players in the pathogenesis of LS, but close approximation of inflammatory lymphocytic and macrophage infiltrate with collagen and fibroblasts deposition supports the notion that LS is a disease of inflammatory driven fibrosis. The immune system is dynamic and undergoes changes during childhood, and we speculate on how the unique features of the immune system in childhood could potentially contribute to some of the differences in LS between children and adults. Interestingly, the immune phenotype in pediatric LS resembles to some extent the healthy adult cellular phenotype, possibly supporting accelerated maturation of the immune system in LS. We discuss future directions in better understanding the pathophysiology of and how to better treat pediatric LS." @default.
- W2943217368 created "2019-05-09" @default.
- W2943217368 creator A5020176584 @default.
- W2943217368 creator A5044888053 @default.
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- W2943217368 creator A5070009221 @default.
- W2943217368 creator A5074699308 @default.
- W2943217368 creator A5088356196 @default.
- W2943217368 date "2019-04-30" @default.
- W2943217368 modified "2023-10-14" @default.
- W2943217368 title "Immunopathogenesis of Pediatric Localized Scleroderma" @default.
- W2943217368 cites W1463896243 @default.
- W2943217368 cites W1485310678 @default.
- W2943217368 cites W1572398678 @default.
- W2943217368 cites W1594245074 @default.
- W2943217368 cites W1712083755 @default.
- W2943217368 cites W1782217370 @default.
- W2943217368 cites W178847858 @default.
- W2943217368 cites W1808798629 @default.
- W2943217368 cites W1891754357 @default.
- W2943217368 cites W1936720470 @default.
- W2943217368 cites W1954735303 @default.
- W2943217368 cites W1964851502 @default.
- W2943217368 cites W1967790513 @default.
- W2943217368 cites W1970601524 @default.
- W2943217368 cites W1971294942 @default.
- W2943217368 cites W1972086046 @default.
- W2943217368 cites W1982919261 @default.
- W2943217368 cites W1988434078 @default.
- W2943217368 cites W1989922845 @default.
- W2943217368 cites W1990977789 @default.
- W2943217368 cites W2000218174 @default.
- W2943217368 cites W2001279904 @default.
- W2943217368 cites W2003949694 @default.
- W2943217368 cites W2004194867 @default.
- W2943217368 cites W2009773004 @default.
- W2943217368 cites W2014749052 @default.
- W2943217368 cites W2016759483 @default.
- W2943217368 cites W2019790147 @default.
- W2943217368 cites W2024476792 @default.
- W2943217368 cites W2024866056 @default.
- W2943217368 cites W2028058670 @default.
- W2943217368 cites W2030286356 @default.
- W2943217368 cites W2031526372 @default.
- W2943217368 cites W2036860785 @default.
- W2943217368 cites W2040477022 @default.
- W2943217368 cites W2043065615 @default.
- W2943217368 cites W2049244261 @default.
- W2943217368 cites W2053637080 @default.
- W2943217368 cites W2069535666 @default.
- W2943217368 cites W2075639487 @default.
- W2943217368 cites W2081163651 @default.
- W2943217368 cites W2085963615 @default.
- W2943217368 cites W2088275441 @default.
- W2943217368 cites W2093696723 @default.
- W2943217368 cites W2093935010 @default.
- W2943217368 cites W2099860193 @default.
- W2943217368 cites W2102919506 @default.
- W2943217368 cites W2108931770 @default.
- W2943217368 cites W2111891637 @default.
- W2943217368 cites W2112763983 @default.
- W2943217368 cites W2117407737 @default.
- W2943217368 cites W2129989036 @default.
- W2943217368 cites W2133179840 @default.
- W2943217368 cites W2133262418 @default.
- W2943217368 cites W2135698289 @default.
- W2943217368 cites W2140684657 @default.
- W2943217368 cites W2145939653 @default.
- W2943217368 cites W2147925625 @default.
- W2943217368 cites W2149389714 @default.
- W2943217368 cites W2155428092 @default.
- W2943217368 cites W2155955569 @default.
- W2943217368 cites W2159665984 @default.
- W2943217368 cites W2165516192 @default.
- W2943217368 cites W2168198817 @default.
- W2943217368 cites W2170165882 @default.
- W2943217368 cites W2233660390 @default.
- W2943217368 cites W2270053514 @default.
- W2943217368 cites W2298683710 @default.
- W2943217368 cites W2315987995 @default.
- W2943217368 cites W2337055300 @default.
- W2943217368 cites W2409194562 @default.
- W2943217368 cites W2528008487 @default.
- W2943217368 cites W2561965154 @default.
- W2943217368 cites W2564327421 @default.
- W2943217368 cites W2593064880 @default.
- W2943217368 cites W2599501186 @default.
- W2943217368 cites W2742350697 @default.
- W2943217368 cites W2767892835 @default.
- W2943217368 cites W2775521179 @default.
- W2943217368 cites W2801441612 @default.
- W2943217368 cites W2884416234 @default.
- W2943217368 cites W2888423166 @default.
- W2943217368 cites W2891564975 @default.
- W2943217368 cites W2896609824 @default.
- W2943217368 cites W2898095106 @default.
- W2943217368 cites W2936727536 @default.
- W2943217368 cites W2979171901 @default.
- W2943217368 cites W3150502808 @default.