Fibrosis in Systemic Sclerosis

Overview

Journal Rheum Dis Clin North Am

Publisher Elsevier

Specialty Rheumatology

Date 2008 Mar 11

PMID 18329536

Citations 34

Authors

John A Varga

Maria Trojanowska

Affiliations

Soon will be listed here.

Abstract

This article reviews current understanding of the pathophysiology of fibrosis in systemic sclerosis. It highlights recent discoveries, insights, and emerging research, and potential opportunities for the development of targeted antifibrotic therapies.

Citing Articles

Development of immunocompetent full thickness skin tissue constructs to model skin fibrosis for high-throughput drug screening.

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An overview of the role of chemokine CX3CL1 (Fractalkine) and CX3C chemokine receptor 1 in systemic sclerosis.

Pezeshkian F, Shahriarirad R, Mahram H Immun Inflamm Dis. 2024; 12(10):e70034.

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Overlap of Scleroderma and Class V Lupus Nephritis: A Rare Autoimmune Confluence.

Reddy H, Kashiv P, Kumar S, Sejpal K, Acharya S Cureus. 2024; 16(3):e56990.

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The Role of Autophagy and Apoptosis in Affected Skin and Lungs in Patients with Systemic Sclerosis.

Spasovski V, Andjelkovic M, Parezanovic M, Komazec J, Ugrin M, Klaassen K Int J Mol Sci. 2023; 24(13).

PMID: 37446389 PMC: 10342863. DOI: 10.3390/ijms241311212.

Modulation by 17,20S(OH)pD of Fibrosis-Related Mediators in Dermal Fibroblast Lines from Healthy Donors and from Patients with Systemic Sclerosis.

Brown Lobbins M, Slominski A, Hasty K, Zhang S, Miller D, Li W Int J Mol Sci. 2022; 23(1).

PMID: 35008794 PMC: 8745512. DOI: 10.3390/ijms23010367.

References

Klareskog L, Gustafsson R, Scheynius A, Hallgren R . Increased expression of platelet-derived growth factor type B receptors in the skin of patients with systemic sclerosis. Arthritis Rheum. 1990; 33(10):1534-41. DOI: 10.1002/art.1780331011. View

Jun J, Kuechle M, Min J, Shim S, Kim G, Montenegro V . Scleroderma fibroblasts demonstrate enhanced activation of Akt (protein kinase B) in situ. J Invest Dermatol. 2005; 124(2):298-303. DOI: 10.1111/j.0022-202X.2004.23559.x. View

Green M, Sweet H, Bunker L . Tight-skin, a new mutation of the mouse causing excessive growth of connective tissue and skeleton. Am J Pathol. 1976; 82(3):493-512. PMC: 2032428. View

Baxter R, Crowell T, McCrann M, Frew E, Gardner H . Analysis of the tight skin (Tsk1/+) mouse as a model for testing antifibrotic agents. Lab Invest. 2005; 85(10):1199-209. DOI: 10.1038/labinvest.3700331. View

Ferreira A, Takagawa S, Fresco R, Zhu X, Varga J, DiPietro L . Diminished induction of skin fibrosis in mice with MCP-1 deficiency. J Invest Dermatol. 2006; 126(8):1900-8. DOI: 10.1038/sj.jid.5700302. View

Hayashida T, Wu M, Pierce A, Poncelet A, Varga J, Schnaper H . MAP-kinase activity necessary for TGFbeta1-stimulated mesangial cell type I collagen expression requires adhesion-dependent phosphorylation of FAK tyrosine 397. J Cell Sci. 2007; 120(Pt 23):4230-40. DOI: 10.1242/jcs.03492. View

Lakos G, Melichian D, Wu M, Varga J . Increased bleomycin-induced skin fibrosis in mice lacking the Th1-specific transcription factor T-bet. Pathobiology. 2007; 73(5):224-37. DOI: 10.1159/000098208. View

Eckes B, Zigrino P, Kessler D, Holtkotter O, Shephard P, Mauch C . Fibroblast-matrix interactions in wound healing and fibrosis. Matrix Biol. 2000; 19(4):325-32. DOI: 10.1016/s0945-053x(00)00077-9. View

Cheng S, Afif H, Martel-Pelletier J, Pelletier J, Li X, Farrajota K . Activation of peroxisome proliferator-activated receptor gamma inhibits interleukin-1beta-induced membrane-associated prostaglandin E2 synthase-1 expression in human synovial fibroblasts by interfering with Egr-1. J Biol Chem. 2004; 279(21):22057-65. DOI: 10.1074/jbc.M402828200. View

10.

Lakos G, Takagawa S, Chen S, Ferreira A, Han G, Masuda K . Targeted disruption of TGF-beta/Smad3 signaling modulates skin fibrosis in a mouse model of scleroderma. Am J Pathol. 2004; 165(1):203-17. PMC: 1618525. DOI: 10.1016/s0002-9440(10)63289-0. View

11.

Pannu J, Nakerakanti S, Smith E, Ten Dijke P, Trojanowska M . Transforming growth factor-beta receptor type I-dependent fibrogenic gene program is mediated via activation of Smad1 and ERK1/2 pathways. J Biol Chem. 2007; 282(14):10405-13. DOI: 10.1074/jbc.M611742200. View

12.

Chujo S, Shirasaki F, Kawara S, Inagaki Y, Kinbara T, Inaoki M . Connective tissue growth factor causes persistent proalpha2(I) collagen gene expression induced by transforming growth factor-beta in a mouse fibrosis model. J Cell Physiol. 2004; 203(2):447-56. DOI: 10.1002/jcp.20251. View

13.

Zhang Y, McCormick L, Desai S, Wu C, Gilliam A . Murine sclerodermatous graft-versus-host disease, a model for human scleroderma: cutaneous cytokines, chemokines, and immune cell activation. J Immunol. 2002; 168(6):3088-98. DOI: 10.4049/jimmunol.168.6.3088. View

14.

Carulli M, Ong V, Ponticos M, Shiwen X, Abraham D, Black C . Chemokine receptor CCR2 expression by systemic sclerosis fibroblasts: evidence for autocrine regulation of myofibroblast differentiation. Arthritis Rheum. 2005; 52(12):3772-82. DOI: 10.1002/art.21396. View

15.

Helmbold P, Fiedler E, Fischer M, Marsch W . Hyperplasia of dermal microvascular pericytes in scleroderma. J Cutan Pathol. 2004; 31(6):431-40. DOI: 10.1111/j.0303-6987.2004.00203.x. View

16.

Pilewski J, Liu L, Henry A, Knauer A, Feghali-Bostwick C . Insulin-like growth factor binding proteins 3 and 5 are overexpressed in idiopathic pulmonary fibrosis and contribute to extracellular matrix deposition. Am J Pathol. 2005; 166(2):399-407. PMC: 1602317. DOI: 10.1016/S0002-9440(10)62263-8. View

17.

Varga J . Scleroderma and Smads: dysfunctional Smad family dynamics culminating in fibrosis. Arthritis Rheum. 2002; 46(7):1703-13. DOI: 10.1002/art.10413. View

18.

Massague J, Seoane J, Wotton D . Smad transcription factors. Genes Dev. 2005; 19(23):2783-810. DOI: 10.1101/gad.1350705. View

19.

Mauviel A . Transforming growth factor-beta: a key mediator of fibrosis. Methods Mol Med. 2005; 117:69-80. DOI: 10.1385/1-59259-940-0:069. View

20.

Kubo M, Ihn H, Yamane K, Tamaki K . Upregulated expression of transforming growth factor-beta receptors in dermal fibroblasts of skin sections from patients with systemic sclerosis. J Rheumatol. 2002; 29(12):2558-64. View