دورية أكاديمية

Immunopathogenesis of Pediatric Localized Scleroderma.

التفاصيل البيبلوغرافية
العنوان: Immunopathogenesis of Pediatric Localized Scleroderma.
المؤلفون: Torok KS; Division of Pediatric Rheumatology, Department of Pediatrics, Childrens's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, United States., Li SC; Division of Pediatric Rheumatology, Department of Pediatrics, Hackensack University Medical Center, Hackensack, NJ, United States.; Hackensack Meridian School of Medicine at Seton Hall University, Clifton, NJ, United States., Jacobe HM; Department of Dermatology, UT Southwestern Medical Center, Dallas, TX, United States., Taber SF; Division of Pediatric Rheumatology, Department of Rheumatology, Hospital for Special Surgery, New York, NY, United States.; Department of Pediatrics, Weill Cornell Medicine, New York, NY, United States., Stevens AM; Division of Pediatric Rheumatology, Department of Pediatrics, University of Washington, Seattle, WA, United States.; Seattle Children's Research Institute, University of Washington, Seattle, WA, United States., Zulian F; Pediatric Rheumatology Unit, Department of Woman's and Child's Health, University of Padua, Padua, Italy., Lu TT; Division of Pediatric Rheumatology, Department of Rheumatology, Hospital for Special Surgery, New York, NY, United States.; HSS Research Institute, Hospital for Special Surgery, New York, NY, United States.; Department of Microbiology and Immunology, Weill Cornell Medicine, New York, NY, United States.
المصدر: Frontiers in immunology [Front Immunol] 2019 Apr 30; Vol. 10, pp. 908. Date of Electronic Publication: 2019 Apr 30 (Print Publication: 2019).
نوع المنشور: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
اللغة: English
بيانات الدورية: Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101560960 Publication Model: eCollection Cited Medium: Internet ISSN: 1664-3224 (Electronic) Linking ISSN: 16643224 NLM ISO Abbreviation: Front Immunol Subsets: MEDLINE
أسماء مطبوعة: Original Publication: [Lausanne : Frontiers Research Foundation]
مواضيع طبية MeSH: Autoantibodies*/genetics , Autoantibodies*/immunology , HLA Antigens*/genetics , HLA Antigens*/immunology , Macrophages*/immunology , Macrophages*/pathology , Scleroderma, Localized*/genetics , Scleroderma, Localized*/immunology , Scleroderma, Localized*/pathology , Skin*/immunology , Skin*/pathology , T-Lymphocytes, Helper-Inducer*/immunology , T-Lymphocytes, Helper-Inducer*/pathology, Child ; Child, Preschool ; Female ; Humans ; Male ; RNA-Seq
مستخلص: 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.
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معلومات مُعتمدة: R01 AI079178 United States AI NIAID NIH HHS
فهرسة مساهمة: Keywords: autoimmune disease; disease etiology; fibrosis; immunophenotype; localized scleroderma; morphea; pediatric rheumatology; skin
المشرفين على المادة: 0 (Autoantibodies)
0 (HLA Antigens)
تواريخ الأحداث: Date Created: 20190523 Date Completed: 20200929 Latest Revision: 20231011
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC6503092
DOI: 10.3389/fimmu.2019.00908
PMID: 31114575
قاعدة البيانات: MEDLINE
الوصف
تدمد:1664-3224
DOI:10.3389/fimmu.2019.00908