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Rheumatology Advance Access originally published online on January 25, 2007
Rheumatology 2007 46(5):833-841; doi:10.1093/rheumatology/kel451
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
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Skin involvement in scleroderma—where histological and clinical scores meet

F. Verrecchia1, J. Laboureau1, O. Verola2, N. Roos1, R. Porcher3, P. Bruneval4, M. Ertault5, K. Tiev6, L. Michel1, A. Mauviel1 and D. Farge1,7

1INSERM U697 Paris, France, 2Service d’anatomopathologie, 3Département de Biostatistique, Hôpital Saint-Louis, 4Service d’anatomopathologie, Hôpital Européen Georges Pompidou, 5Service d’hématologie et de greffe de moelle, Hôpital Saint-Louis, Paris, France, 6Service de Médecine Interne, Hôpital Saint-Antoine, Paris and 7Service de Médecine Interne, Hôpital Saint-Louis, Paris, France.

Correspondence to: F. Verrecchia or D. Farge, INSERM U697 and Service de Médecine Interne, Hôpital Saint-Louis, 1 avenue Claude Vellefaux 75010 Paris, France. E-mail: franck.verrecchia{at}stlouis.inserm.fr or dominique.farge-bancel{at}sls.ap-hop-paris.fr


  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-ß 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)-ß/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-ß signalling activation in SSc skin fibroblasts appears to parallel the severity of disease.

KEY WORDS: Systemic sclerosis, Haematopoietic stem cell transplantation, Fibrosis, TGF-ß, Smad signalling

Submitted 10 November 2006; revised version accepted 15 December 2006.
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