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Regulation of Monocyte Adhesion and Type I Interferon Signaling by CD52 in Patients With Systemic Sclerosis. Arthritis Rheumatol. 2021 Sep;73(9):1720-1730.
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Busulfan FDA Label
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Iloprost FDA Label
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ClinicalTrials.gov (NCT04166552) A Phase IIa, Double-Blind, Randomised, Intracohort Placebo-Controlled, Multicentre Study to Evaluate the Safety, Tolerability and Preliminary Efficacy of EHP-101 in Patients With Diffuse Cutaneous Systemic Sclerosis. U.S.National Institutes of Health.
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Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
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ClinicalTrials.gov (NCT04948554) A Phase 1b Randomized, Double-Blind, Placebo-Controlled, Multiple-Ascending Dose Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of ACE-1334 Plus Standard of Care in Participants With Systemic Sclerosis. U.S.National Institutes of Health.
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Deregulated PSGL-1 Expression in B Cells and Dendritic Cells May Be Implicated in Human Systemic Sclerosis Development.J Invest Dermatol. 2018 Oct;138(10):2123-2132. doi: 10.1016/j.jid.2018.04.003. Epub 2018 Apr 22.
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The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis.Clin Appl Thromb Hemost. 2018 Sep;24(6):920-927. doi: 10.1177/1076029617736382. Epub 2017 Nov 12.
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An allograft inflammatory factor 1 (AIF1) single nucleotide polymorphism (SNP) is associated with anticentromere antibody positive systemic sclerosis.Rheumatology (Oxford). 2007 Aug;46(8):1248-51. doi: 10.1093/rheumatology/kem057. Epub 2007 May 23.
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Attenuation of aquaporin-3 and epidermal growth factor receptor expression and activation in systemic sclerosis dermal fibroblasts.J Cell Physiol. 2019 Aug;234(8):12876-12883. doi: 10.1002/jcp.27952. Epub 2018 Dec 7.
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Circulating follicular helper T cells are increased in systemic sclerosis and promote plasmablast differentiation through the IL-21 pathway which can be inhibited by ruxolitinib.Ann Rheum Dis. 2019 Apr;78(4):539-550. doi: 10.1136/annrheumdis-2018-214382. Epub 2019 Feb 13.
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Immune rebound associates with a favorable clinical response to autologous HSCT in systemic sclerosis patients.Blood Adv. 2018 Jan 23;2(2):126-141. doi: 10.1182/bloodadvances.2017011072.
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The Long Non-coding RNA NRIR Drives IFN-Response in Monocytes: Implication for Systemic Sclerosis.Front Immunol. 2019 Jan 31;10:100. doi: 10.3389/fimmu.2019.00100. eCollection 2019.
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Epidermal Growth Factor Like-domain 7 and miR-126 are abnormally expressed in diffuse Systemic Sclerosis fibroblasts.Sci Rep. 2019 Mar 14;9(1):4589. doi: 10.1038/s41598-019-39485-8.
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Fibroblast Growth Factor 23 to Alpha-Klotho Index Correlates with Systemic Sclerosis Activity: A Proposal for Novel Disease Activity Marker.J Clin Med. 2018 Dec 17;7(12):558. doi: 10.3390/jcm7120558.
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Transcription factor Fra-2 and its emerging role in matrix deposition, proliferation and inflammation in chronic lung diseases.Cell Signal. 2019 Dec;64:109408. doi: 10.1016/j.cellsig.2019.109408. Epub 2019 Aug 29.
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Identification of Cysteine-Rich Angiogenic Inducer 61 as a Potential Antifibrotic and Proangiogenic Mediator in Scleroderma.Arthritis Rheumatol. 2019 Aug;71(8):1350-1359. doi: 10.1002/art.40890. Epub 2019 Jun 12.
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Copy Number Variation of HLA-DQA1 and APOBEC3A/3B Contribute to the Susceptibility of Systemic Sclerosis in the Chinese Han Population.J Rheumatol. 2016 May;43(5):880-6. doi: 10.3899/jrheum.150945. Epub 2016 Apr 1.
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Skin Gene Expression Is Prognostic for the Trajectory of Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis.Arthritis Rheumatol. 2018 Jun;70(6):912-919. doi: 10.1002/art.40455.
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IL-16 expression is increased in the skin and sera of patients with systemic sclerosis.Rheumatology (Oxford). 2020 Mar 1;59(3):519-523. doi: 10.1093/rheumatology/kez318.
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Serum concentrations of IL-17A, IL-17B, IL-17E and IL-17F in patients with systemic sclerosis.Arch Med Sci. 2019 May;15(3):706-712. doi: 10.5114/aoms.2019.84738. Epub 2019 Apr 30.
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Cross-Disease Innate Gene Signature: Emerging Diversity and Abundance in RA Comparing to SLE and SSc.J Immunol Res. 2019 Jul 16;2019:3575803. doi: 10.1155/2019/3575803. eCollection 2019.
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Scleroderma Peripheral B Lymphocytes Secrete Interleukin-6 and Transforming Growth Factor and Activate Fibroblasts.Arthritis Rheumatol. 2017 May;69(5):1078-1089. doi: 10.1002/art.40016.
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Identification of NF-B and PLCL2 as new susceptibility genes and highlights on a potential role of IRF8 through interferon signature modulation in systemic sclerosis.Arthritis Res Ther. 2015 Mar 21;17(1):71. doi: 10.1186/s13075-015-0572-y.
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Increased expression of integrin alpha(v)beta3 contributes to the establishment of autocrine TGF-beta signaling in scleroderma fibroblasts.J Immunol. 2005 Dec 1;175(11):7708-18. doi: 10.4049/jimmunol.175.11.7708.
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Killer cell immunoglobulin-like receptor (KIR) genes in systemic sclerosis.Clin Exp Immunol. 2010 Jun;160(3):325-30. doi: 10.1111/j.1365-2249.2010.04095.x. Epub 2010 Jan 15.
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The investigation of killer cell immunoglobulin-like receptor genotyping in patients with systemic lupus erytematosus and systemic sclerosis.Clin Rheumatol. 2016 Apr;35(4):919-25. doi: 10.1007/s10067-016-3222-0. Epub 2016 Mar 9.
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Differential expression of tissue kallikrein in the skin of systemic sclerosis.Histol Histopathol. 2005 Apr;20(2):415-22. doi: 10.14670/HH-20.415.
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TH17 cellsexpressing CD146 are significantly increased in patients with Systemic sclerosis.Sci Rep. 2019 Nov 27;9(1):17721. doi: 10.1038/s41598-019-54132-y.
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The effects of oral treatment for systemic sclerosis related pulmonary arterial hypertension: A systematic review and meta-analysis.Mod Rheumatol. 2021 Jan;31(1):151-161. doi: 10.1080/14397595.2019.1704125. Epub 2020 Jan 4.
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Role of PDGF in fibrotic diseases and systemic sclerosis.Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v2-4. doi: 10.1093/rheumatology/ken265.
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Inactivation of urokinase-type plasminogen activator receptor (uPAR) gene induces dermal and pulmonary fibrosis and peripheral microvasculopathy in mice: a new model of experimental scleroderma?.Ann Rheum Dis. 2014 Sep;73(9):1700-9. doi: 10.1136/annrheumdis-2013-203706. Epub 2013 Jul 12.
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Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo.BMJ Case Rep. 2019 Nov 26;12(11):e232987. doi: 10.1136/bcr-2019-232987.
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Sphingosine-1-Phosphate Receptor 5 Modulates Early-Stage Processes during Fibrogenesis in a Mouse Model of Systemic Sclerosis: A Pilot Study.Front Immunol. 2017 Sep 29;8:1242. doi: 10.3389/fimmu.2017.01242. eCollection 2017.
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Leri's pleonosteosis, a congenital rheumatic disease, results from microduplication at 8q22.1 encompassing GDF6 and SDC2 and provides insight into systemic sclerosis pathogenesis.Ann Rheum Dis. 2015 Jun;74(6):1249-56. doi: 10.1136/annrheumdis-2013-204309. Epub 2014 Jan 17.
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Involvement of collagen-binding heat shock protein 47 in scleroderma-associated fibrosis.Protein Cell. 2015 Aug;6(8):589-598. doi: 10.1007/s13238-015-0171-3. Epub 2015 Jun 20.
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Extracellular SPARC cooperates with TGF- signalling to induce pro-fibrotic activation of systemic sclerosis patient dermal fibroblasts.Rheumatology (Oxford). 2020 Sep 1;59(9):2258-2263. doi: 10.1093/rheumatology/kez583.
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Antibodies against specific extractable nuclear antigens (ENAs) as diagnostic and prognostic tools and inducers of a profibrotic phenotype in cultured human skin fibroblasts: are they functional?.Arthritis Res Ther. 2019 Jun 24;21(1):152. doi: 10.1186/s13075-019-1931-x.
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Association of TAP1 and TAP2 gene polymorphisms with systemic sclerosis in Korean patients.Hum Immunol. 2005 Jul;66(7):810-7. doi: 10.1016/j.humimm.2005.03.006.
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The genetics of scleroderma (systemic sclerosis).Curr Opin Rheumatol. 2010 Mar;22(2):133-8. doi: 10.1097/BOR.0b013e3283367c17.
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TBK1: A key regulator and potential treatment target for interferon positive Sjgren's syndrome, systemic lupus erythematosus and systemic sclerosis.J Autoimmun. 2018 Jul;91:97-102. doi: 10.1016/j.jaut.2018.02.001. Epub 2018 Apr 16.
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Localized expression of tenascin in systemic sclerosis-associated pulmonary fibrosis and its regulation by insulin-like growth factor binding protein 3.Arthritis Rheum. 2012 Jan;64(1):272-80. doi: 10.1002/art.30647.
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Gene-level association analysis of systemic sclerosis: A comparison of African-Americans and White populations.PLoS One. 2018 Jan 2;13(1):e0189498. doi: 10.1371/journal.pone.0189498. eCollection 2018.
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Upregulation and pathogenic roles of CCL18-CCR8 axis in IgG4-related disease.Mod Rheumatol. 2020 Jul;30(4):729-737. doi: 10.1080/14397595.2019.1632061. Epub 2019 Aug 8.
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Depletion of protein kinase Cepsilon in normal and scleroderma lung fibroblasts has opposite effects on tenascin expression.Arthritis Rheum. 2001 Jun;44(6):1370-81. doi: 10.1002/1529-0131(200106)44:6<1370::AID-ART230>3.0.CO;2-2.
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Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis.Arthritis Rheumatol. 2019 Aug;71(8):1339-1349. doi: 10.1002/art.40862. Epub 2019 Jun 18.
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The status of pulmonary fibrosis in systemic sclerosis is associated with IRF5, STAT4, IRAK1, and CTGF polymorphisms.Rheumatol Int. 2017 Aug;37(8):1303-1310. doi: 10.1007/s00296-017-3722-5. Epub 2017 Apr 22.
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Significance of the angiotensin I/angiotensin II/angiotensin-(1-7) axis in the pathogenesis of systemic sclerosis.J Eur Acad Dermatol Venereol. 2020 Mar;34(3):558-564. doi: 10.1111/jdv.16103. Epub 2019 Dec 18.
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The arachidonate 5-lipoxygenase activating protein gene polymorphism is associated with the risk of scleroderma-related interstitial lung disease: a multicentre European Scleroderma Trials and Research group (EUSTAR) study.Rheumatology (Oxford). 2017 May 1;56(5):844-852. doi: 10.1093/rheumatology/kew499.
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Single Cell RNA Sequencing Identifies HSPG2 and APLNR as Markers of Endothelial Cell Injury in Systemic Sclerosis Skin.Front Immunol. 2018 Oct 1;9:2191. doi: 10.3389/fimmu.2018.02191. eCollection 2018.
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Clinical relevance of antiphospholipid antibodies in systemic sclerosis: A systematic review and meta-analysis.Semin Arthritis Rheum. 2017 Apr;46(5):615-624. doi: 10.1016/j.semarthrit.2016.10.004. Epub 2016 Oct 13.
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Bactericidal/permeability-increasing protein and cathepsin G are the major antigenic targets of antineutrophil cytoplasmic autoantibodies in systemic sclerosis.J Rheumatol. 2003 Jun;30(6):1248-52.
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Aberrant expression of the innate restriction factor bone marrow stromal antigen-2 in primary Sjgren's syndrome.J Craniomaxillofac Surg. 2018 Nov;46(11):1899-1904. doi: 10.1016/j.jcms.2018.08.008. Epub 2018 Aug 29.
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Analysis of gene expression patterns in systemic sclerosis fibroblasts using RNA arbitrarily primed-polymerase chain reaction for differential display.J Rheumatol. 2007 Apr;34(4):747-53.
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CD1a+ survivin+ dendritic cell infiltration in dermal lesions of systemic sclerosis.Arthritis Res Ther. 2015 Sep 30;17:275. doi: 10.1186/s13075-015-0785-0.
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Blocking CD248 molecules in perivascular stromal cells of patients with systemic sclerosis strongly inhibits their differentiation toward myofibroblasts and proliferation: a new potential target for antifibrotic therapy.Arthritis Res Ther. 2018 Oct 3;20(1):223. doi: 10.1186/s13075-018-1719-4.
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The role of B7 family costimulatory molecules and indoleamine 2,3-dioxygenase in primary Sjgren's syndrome and systemic sclerosis.Immunol Res. 2017 Jun;65(3):622-629. doi: 10.1007/s12026-016-8880-0.
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Epigenetic mechanisms: An emerging role in pathogenesis and its therapeutic potential in systemic sclerosis.Int J Biochem Cell Biol. 2015 Oct;67:92-100. doi: 10.1016/j.biocel.2015.05.023. Epub 2015 Jun 1.
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Macrophages and cadherins in fibrosis and systemic sclerosis.Curr Opin Rheumatol. 2019 Nov;31(6):582-588. doi: 10.1097/BOR.0000000000000657.
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Microparticles in systemic sclerosis: Potential pro-inflammatory mediators and pulmonary hypertension biomarkers.Respirology. 2019 Jul;24(7):675-683. doi: 10.1111/resp.13500. Epub 2019 Feb 12.
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The centromere kinesin-like protein, CENP-E. An autoantigen in systemic sclerosis.Arthritis Rheum. 1996 Aug;39(8):1355-61. doi: 10.1002/art.1780390813.
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A systemic sclerosis and systemic lupus erythematosus pan-meta-GWAS reveals new shared susceptibility loci.Hum Mol Genet. 2013 Oct 1;22(19):4021-9. doi: 10.1093/hmg/ddt248. Epub 2013 Jun 4.
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A genome-wide association study follow-up suggests a possible role for PPARG in systemic sclerosis susceptibility.Arthritis Res Ther. 2014 Jan 9;16(1):R6. doi: 10.1186/ar4432.
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Targeting the myofibroblast genetic switch: inhibitors of myocardin-related transcription factor/serum response factor-regulated gene transcription prevent fibrosis in a murine model of skin injury. J Pharmacol Exp Ther. 2014 Jun;349(3):480-6. doi: 10.1124/jpet.114.213520. Epub 2014 Apr 4.
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Distinct Properties of Human M-CSF and GM-CSF Monocyte-Derived Macrophages to Simulate Pathological Lung Conditions In Vitro: Application to Systemic and Inflammatory Disorders with Pulmonary Involvement.Int J Mol Sci. 2018 Mar 17;19(3):894. doi: 10.3390/ijms19030894.
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ATX-S10(Na)-PDT shows more potent effect on collagen metabolism of human normal and scleroderma dermal fibroblasts than ALA-PDT.Arch Dermatol Res. 2006 Nov;298(6):257-63. doi: 10.1007/s00403-006-0689-6. Epub 2006 Sep 15.
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Increased expression levels of FcRIIB on nave and double-negative memory B cells in patients with systemic sclerosis.Clin Exp Rheumatol. 2019 Jul-Aug;37 Suppl 119(4):23-31. Epub 2019 Jan 17.
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Differential effects of the immunosuppressant FK-506 on human alpha2(I) collagen gene expression and transforming growth factor beta signaling in normal and scleroderma fibroblasts.Arthritis Rheum. 2005 Apr;52(4):1237-47. doi: 10.1002/art.20934.
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Depletion of activated macrophages with a folate receptor-beta-specific antibody improves symptoms in mouse models of rheumatoid arthritis.Arthritis Res Ther. 2019 Jun 7;21(1):143. doi: 10.1186/s13075-019-1912-0.
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Prediction of organ involvement in systemic sclerosis by serum biomarkers and peripheral endothelial function.Clin Exp Rheumatol. 2018 Jul-Aug;36 Suppl 113(4):102-108. Epub 2018 Apr 12.
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Analysis of killer cell immunoglobulin-like receptors (KIRs) and their HLA ligand genes polymorphisms in Iranian patients with systemic sclerosis.Clin Rheumatol. 2017 Apr;36(4):853-862. doi: 10.1007/s10067-016-3526-0. Epub 2017 Jan 24.
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Acyltransferase skinny hedgehog regulates TGF-dependent fibroblast activation in SSc.Ann Rheum Dis. 2019 Sep;78(9):1269-1273. doi: 10.1136/annrheumdis-2019-215066. Epub 2019 Jun 8.
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Soluble HLA-G Expression Inversely Correlates With Fetal Microchimerism Levels in Peripheral Blood From Women With Scleroderma.Front Immunol. 2018 Aug 14;9:1685. doi: 10.3389/fimmu.2018.01685. eCollection 2018.
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Heat shock protein 90 (Hsp90) inhibition targets canonical TGF- signalling to prevent fibrosis.Ann Rheum Dis. 2014 Jun;73(6):1215-22. doi: 10.1136/annrheumdis-2012-203095. Epub 2013 May 9.
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T follicular helper-like cells contribute to skin fibrosis.Sci Transl Med. 2018 Mar 7;10(431):eaaf5307. doi: 10.1126/scitranslmed.aaf5307.
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FOXP3, ICOS and ICOSL gene polymorphisms in systemic sclerosis: FOXP3 rs2294020 is associated with disease progression in a female Italian population.Immunobiology. 2018 Jan;223(1):112-117. doi: 10.1016/j.imbio.2017.10.004. Epub 2017 Oct 5.
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Angiotensin-converting enzyme insertion/deletion polymorphism and susceptibility to systemic sclerosis: a meta-analysis.Genet Mol Res. 2014 Oct 7;13(4):8174-83. doi: 10.4238/2014.October.7.12.
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Type I IFNs Regulate Inflammation, Vasculopathy, and Fibrosis in Chronic Cutaneous Graft-versus-Host Disease.J Immunol. 2016 Jul 1;197(1):42-50. doi: 10.4049/jimmunol.1502190. Epub 2016 May 25.
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Upregulation of myxovirus-resistance protein A: a possible marker of type I interferon induction in systemic sclerosis.J Rheumatol. 2008 Nov;35(11):2192-200. doi: 10.3899/jrheum.080418. Epub 2008 Oct 1.
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Insights into IL-29: Emerging role in inflammatory autoimmune diseases.J Cell Mol Med. 2019 Dec;23(12):7926-7932. doi: 10.1111/jcmm.14697. Epub 2019 Oct 2.
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IFNL3 genotype is associated with pulmonary fibrosis in patients with systemic sclerosis.Sci Rep. 2019 Oct 16;9(1):14834. doi: 10.1038/s41598-019-50709-9.
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IL13RA2 gene polymorphisms are associated with systemic sclerosis.J Rheumatol. 2006 Oct;33(10):2015-9. Epub 2006 Sep 15.
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Treatment of psoriasis with ustekinumab improved skin tightening in systemic sclerosis.Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):208-210. Epub 2017 Apr 19.
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Desmoglein-2-integrin Beta-8 interaction regulates actin assembly in endothelial cells: deregulation in systemic sclerosis.PLoS One. 2013 Jul 11;8(7):e68117. doi: 10.1371/journal.pone.0068117. Print 2013.
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A possible implication of reduced levels of LIF, LIFR, and gp130 in vasculopathy related to systemic sclerosis.Arch Dermatol Res. 2017 Dec;309(10):833-842. doi: 10.1007/s00403-017-1786-4. Epub 2017 Oct 16.
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Suppressive Regulation by MFG-E8 of Latent Transforming Growth Factor -Induced Fibrosis via Binding to v Integrin: Significance in the Pathogenesis of Fibrosis in Systemic Sclerosis.Arthritis Rheumatol. 2019 Feb;71(2):302-314. doi: 10.1002/art.40701. Epub 2019 Jan 4.
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Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis.Ann Rheum Dis. 2017 May;76(5):924-934. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.
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A novel miRNA-4484 is up-regulated on microarray and associated with increased MMP-21 expression in serum of systemic sclerosis patients.Sci Rep. 2019 Oct 3;9(1):14264. doi: 10.1038/s41598-019-50695-y.
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Serum levels of Krebs von den Lungen-6 as a promising marker for predicting occurrence and deterioration of systemic sclerosis-associated interstitial lung disease from a Chinese cohort.Int J Rheum Dis. 2019 Jan;22(1):108-115. doi: 10.1111/1756-185X.13452. Epub 2018 Dec 28.
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Association of NCF1 polymorphism with systemic lupus erythematosus and systemic sclerosis but not with ANCA-associated vasculitis in a Japanese population.Sci Rep. 2019 Nov 8;9(1):16366. doi: 10.1038/s41598-019-52920-0.
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Analysis of the genetic component of systemic sclerosis in Iranian and Turkish populations through a genome-wide association study.Rheumatology (Oxford). 2019 Feb 1;58(2):289-298. doi: 10.1093/rheumatology/key281.
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Comparison of dextran-based sirolimus-eluting stents and PLA-based sirolimus-eluting stents in vitro and in vivo.J Biomed Mater Res A. 2017 Jan;105(1):301-310. doi: 10.1002/jbm.a.35898. Epub 2016 Oct 31.
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Protein kinases G are essential downstream mediators of the antifibrotic effects of sGC stimulators.Ann Rheum Dis. 2018 Mar;77(3):459. doi: 10.1136/annrheumdis-2017-212489. Epub 2018 Jan 8.
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Efficacy and Safety of Selexipag in Adults With Raynaud's Phenomenon Secondary to Systemic Sclerosis: A Randomized, Placebo-Controlled, Phase II Study.Arthritis Rheumatol. 2017 Dec;69(12):2370-2379. doi: 10.1002/art.40242.
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