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Skin involvement in scleroderma--where histological and clinical scores meet.

Abstract : OBJECTIVES: A clinico-pathological study in diffuse systemic sclerosis (SSc) patients was performed to analyse whether the skin histological organization and the pro-fibrotic signals elicited by TGF-beta in fibroblasts vary according to the modified Rodnan skin score (mRSS). METHODS: Twenty-seven SSc patients underwent 45 skin biopsies with simultaneous measure of mRSS before or after treatment by immunosuppressive drugs, with or without autologous peripheral haematopoietic stem cell transplantation (HSCT). RESULTS: Double-blind optic microscopy analysis of the biopsies standard extracellular matrix stains allowed to define three histological subgroups: 6 with grade 1 weak fibrosis, 30 with grade 2 moderate fibrosis and 9 with grade 3 severe fibrosis. A significant (P < 0.0001) was identified between the grades of fibrosis and the mRSS. In skin fibroblast cultures, Smad3 phosphorylation levels, as well as mRNA steady-state levels of two transforming growth factor (TGF)-beta/Smad3 targets, COL1A2 and PAI-1, increased in parallel with the mRSS. When compared with pre-transplant values the degree of fibrosis observed after HSCT in the papillary and in the reticular dermis decreased in parallel with the fall in mRSS (n = 5 consecutive patients with repeated biopsies). CONCLUSIONS: The histological extent of skin fibrosis correlates closely with the mRSS. Both parameters appeared to regress after HSCT. The extent of TGF-beta signalling activation in SSc skin fibroblasts appears to parallel the severity of disease.
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https://www.hal.inserm.fr/inserm-00166113
Contributor : Franck Verrecchia <>
Submitted on : Wednesday, August 1, 2007 - 10:25:05 AM
Last modification on : Wednesday, August 19, 2020 - 11:58:38 AM
Long-term archiving on: : Friday, November 25, 2016 - 6:38:32 PM

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Franck Verrecchia, Julien Laboureau, Olivier Verola, Nina Roos, Raphaël Porcher, et al.. Skin involvement in scleroderma--where histological and clinical scores meet.. Rheumatology, Oxford University Press (OUP), 2007, 46 (5), pp.833-41. ⟨10.1093/rheumatology/kel451⟩. ⟨inserm-00166113⟩

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