D. Furst and P. Clements, Hypothesis for the pathogenesis of SSc, J Rheumatol, vol.24, pp.53-60, 1997.

S. Jimenez, E. Hitraya, and J. Varga, PATHOGENESIS OF SCLERODERMA, Rheumatic Disease Clinics of North America, vol.22, issue.4, pp.647-74, 1996.
DOI : 10.1016/S0889-857X(05)70294-5

P. Clements, P. Lachenbruch, and J. Seibold, Inter-and intraobserver variability of total skin thickness score (modified Rodan TSS) in systemic sclerosis, J Rheumatol, vol.22, pp.1281-1286, 1995.

L. Scussel-lonzetti, F. Joyal, and J. Raynauld, Predicting Mortality in Systemic Sclerosis, Medicine, vol.81, issue.2, pp.154-67, 2002.
DOI : 10.1097/00005792-200203000-00005

V. Steen, T. Medsger, and J. , Severe organ involvement in systemic sclerosis with diffuse scleroderma, Arthritis & Rheumatism, vol.39, issue.11, pp.2437-2481, 2000.
DOI : 10.1002/1529-0131(200011)43:11<2437::AID-ANR10>3.0.CO;2-U

A. Reuter, M. Gross, and W. , Intravenous pulse cyclophosphamide in the treatment of interstitial lung disease due to collagen vascular diseases, Arthritis Rheum, vol.41, pp.1215-1235, 1998.

B. White, W. Moore, and F. Wigley, Cyclophosphamide Is Associated with Pulmonary Function and Survival Benefit in Patients with Scleroderma and Alveolitis, Annals of Internal Medicine, vol.132, issue.12, pp.947-54, 2000.
DOI : 10.7326/0003-4819-132-12-200006200-00004

B. Griffiths, S. Miles, and H. Moss, Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function, J Rheumatol, vol.29, pp.2371-2379, 2002.

D. Tashkin, R. Elashoff, and P. Clements, Cyclophosphamide versus Placebo in Scleroderma Lung Disease, New England Journal of Medicine, vol.354, issue.25, pp.2655-66, 2006.
DOI : 10.1056/NEJMoa055120

D. Farge, J. Passweg, and J. Van-laar, Autologous stem cell transplantation in the treatment of systemic sclerosis

P. Mcsweeney, R. Nash, and K. Sullivan, High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes, Blood, vol.100, pp.1602-1612, 2002.

G. Rodnan, E. Lipinski, and J. Luksick, Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma, Arthritis & Rheumatism, vol.21, issue.2, pp.130-170, 1979.
DOI : 10.1002/art.1780220205

M. Trojanowska, What did we learn by studying scleroderma fibroblasts?, Clin Exp Rheumatol, vol.22, pp.59-63, 2004.

M. Dziadzio, R. Smith, and D. Abraham, Serological assessment of type I collagen burden in scleroderma spectrum disorders: a systematic review, Clin Exp Rheumatol, vol.22, pp.356-67, 2004.

F. Verrecchia and A. Mauviel, Control of connective tissue gene expression by TGF??: Role of smad proteins in fibrosis, Current Rheumatology Reports, vol.116, issue.2, pp.143-152, 2002.
DOI : 10.1007/s11926-002-0010-4
URL : https://hal.archives-ouvertes.fr/inserm-00147404

F. Verrecchia and A. Mauviel, Transforming Growth Factor-?? Signaling Through the Smad Pathway: Role in Extracellular Matrix Gene Expression and Regulation, Journal of Investigative Dermatology, vol.118, issue.2, pp.211-216, 2002.
DOI : 10.1046/j.1523-1747.2002.01641.x

P. Dijke and C. Hill, New insights into TGF-?????Smad signalling, Trends in Biochemical Sciences, vol.29, issue.5, pp.265-73, 2004.
DOI : 10.1016/j.tibs.2004.03.008

Y. Shi and J. Massague, Mechanisms of TGF-?? Signaling from Cell Membrane to the Nucleus, Cell, vol.113, issue.6, pp.685-700, 2003.
DOI : 10.1016/S0092-8674(03)00432-X

R. Derynck and Y. Zhang, Smad-dependent and Smad-independent pathways in TGF-?? family signalling, Nature, vol.425, issue.6958, pp.577-84, 2003.
DOI : 10.1038/nature02006

D. Farge, J. Marolleau, and S. Zohar, Autologous bone marrow transplantation in the treatment of refractory systemic sclerosis: early results from a French multicentre phase I-II study, British Journal of Haematology, vol.93, issue.3, pp.726-765, 2002.
DOI : 10.1002/sim.920

S. Dennler, S. Itoh, and D. Vivien, Direct binding of Smad3 and Smad4 to critical TGFbeta -inducible elements in the promoter of human plasminogen activator inhibitor-type 1gene, The EMBO Journal, vol.17, issue.11, pp.3091-100, 1998.
DOI : 10.1093/emboj/17.11.3091

F. Verrecchia, M. Chu, and A. Mauviel, Identification of Novel TGF-??/Smad Gene Targets in Dermal Fibroblasts using a Combined cDNA Microarray/Promoter Transactivation Approach, Journal of Biological Chemistry, vol.276, issue.20, pp.17058-62, 2001.
DOI : 10.1074/jbc.M100754200

L. Sakkas and C. Platsoucas, Is systemic sclerosis an antigen-driven T cell disease?, Arthritis & Rheumatism, vol.341, issue.6, pp.1721-1754, 2004.
DOI : 10.1002/art.20315

D. Farge, C. Henegar, and M. Carmagnat, Analysis of immune reconstitution after autologous bone marrow transplantation in systemic sclerosis, Arthritis & Rheumatism, vol.50, issue.5, pp.1555-63, 2005.
DOI : 10.1002/art.21036

M. Whitfield, D. Finlay, and J. Murray, Systemic and cell type-specific gene expression patterns in scleroderma skin, Proceedings of the National Academy of Sciences, vol.29, issue.1, pp.12319-12343, 2003.
DOI : 10.1093/nar/29.1.152

D. Furst, P. Clements, and V. Steen, The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis, J Rheumatol, vol.25, pp.84-92, 1998.

J. Torres and J. Sanchez, Histopathologic Differentiation Between Localized and Systemic Scleroderma, The American Journal of Dermatopathology, vol.20, issue.3, pp.242-247, 1998.
DOI : 10.1097/00000372-199806000-00003

P. Clements, P. Lachenbruch, and S. Ng, Skin score: A semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis, Arthritis & Rheumatism, vol.18, issue.8, pp.1256-63, 1990.
DOI : 10.1002/art.1780330828

P. Clements, P. Lachenbruch, and J. Seibold, Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies, J Rheumatol, vol.20, pp.1892-1898, 1993.

S. Chen, W. Yuan, and S. Lo, Interaction of Smad3 with a proximal smad-binding element of the human ?2(I) procollagen gene promoter required for transcriptional activation by TGF-?, Journal of Cellular Physiology, vol.2, issue.3, pp.381-92, 2000.
DOI : 10.1002/(SICI)1097-4652(200006)183:3<381::AID-JCP11>3.0.CO;2-O

W. Yuan and J. Varga, Transforming Growth Factor-?? Repression of Matrix Metalloproteinase-1 in Dermal Fibroblasts Involves Smad3, Journal of Biological Chemistry, vol.276, issue.42, pp.38502-38512, 2001.
DOI : 10.1074/jbc.M107081200

Y. Mori, S. Chen, and J. Varga, Expression and regulation of intracellular SMAD signaling in scleroderma skin fibroblasts, Arthritis & Rheumatism, vol.42, issue.7, pp.1964-78, 2003.
DOI : 10.1002/art.11157

C. Dong, S. Zhu, and T. Wang, Deficient Smad7 expression: A putative molecular defect in scleroderma, Proceedings of the National Academy of Sciences, vol.14, issue.6, pp.3908-3921, 2002.
DOI : 10.1172/JCI6094

C. Denton and D. Abraham, Transgenic analysis of scleroderma: understanding key pathogenic events in vivo, Autoimmunity Reviews, vol.3, issue.4, pp.285-93, 2004.
DOI : 10.1016/j.autrev.2003.10.003

J. Landis and G. Koch, The Measurement of Observer Agreement for Categorical Data, Biometrics, vol.33, issue.1, pp.159-74, 1977.
DOI : 10.2307/2529310