دورية أكاديمية
Immunopathogenesis of Juvenile Systemic Sclerosis.
| العنوان: | Immunopathogenesis of Juvenile Systemic Sclerosis. |
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| المؤلفون: | Stevens AM; Division of Rheumatology, Department of Pediatrics, University of Washington, Seattle, WA, United States., Torok KS; Division of Pediatric Rheumatology, Department of Pediatrics, Children'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 Meridian School of Medicine at Seton Hall University, Hackensack, NJ, 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., 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., Zulian F; Rheumatology Unit, Department of Woman's and Child's Health, University of Padua, Padua, Italy. |
| المصدر: | Frontiers in immunology [Front Immunol] 2019 Jun 25; Vol. 10, pp. 1352. Date of Electronic Publication: 2019 Jun 25 (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: | Antibodies, Antinuclear/*immunology , Dendritic Cells/*immunology , Dermis/*immunology , Scleroderma, Systemic/*immunology , Subcutaneous Fat/*immunology, Adult ; Antibodies, Antinuclear/genetics ; Child ; Dendritic Cells/pathology ; Dermis/pathology ; Female ; Humans ; Scleroderma, Systemic/genetics ; Scleroderma, Systemic/pathology ; Subcutaneous Fat/pathology |
| مستخلص: | Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ from women with SSc, resembling instead the genes of adult males with SSc, with additional HLA genes uniquely associated with childhood-onset disease. Current treatments are aimed at inhibiting the inflammatory aspect of disease, but important mechanisms of fibrosis regulated by dermal white adipose tissue dendritic cells may provide an avenue for targeting and potentially reversing the fibrotic stage. |
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| معلومات مُعتمدة: | R01 AI079178 United States AI NIAID NIH HHS |
| فهرسة مساهمة: | Keywords: Pediatric Rheumatology; autoimmune disease; disease etiology; fibrosis; genetics; skin; systemic sclerosis |
| المشرفين على المادة: | 0 (Antibodies, Antinuclear) |
| SCR Disease Name: | Juvenile systemic scleroderma |
| تواريخ الأحداث: | Date Created: 20190712 Date Completed: 20201016 Latest Revision: 20231012 |
| رمز التحديث: | 20240628 |
| مُعرف محوري في PubMed: | PMC6603145 |
| DOI: | 10.3389/fimmu.2019.01352 |
| PMID: | 31293569 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1664-3224 |
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| DOI: | 10.3389/fimmu.2019.01352 |