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Review ArticleMechanisms of DiseaseFree Preview

Scleroderma

List of authors.
  • Armando Gabrielli, M.D.,
  • Enrico V. Avvedimento, M.D.,
  • and Thomas Krieg, M.D.

This account of the mechanisms of scleroderma highlights the genetic and molecular changes that cause the vascular changes and fibrosis in the disease. The role of autoantibodies, of which there are several in scleroderma, is unclear. The authors outline a stepwise process that begins with inflammation, reaches its zenith in fibrosis, and ends in atrophy.

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Funding and Disclosures

Supported in part by grants from Associazione Italiana per la Ricerca sul Cancro, Associazione Italiana per la Lotta alla Sclerodermia, Ministero Italiano per l'Università e la Ricerca Scientifica, Fondazione Cariverona, the German Federal Ministry of Education and Research, and Deutsche Forschungsgemeinschaft (SFB 829, to Dr. Krieg).

Dr. Gabrielli reports receiving lecture fees from Actelion; and Dr. Krieg, lecture fees and grant support from Actelion and DIGNA. No other potential conflict of interest relevant to this article was reported.

We thank Dr. Beate Eckes, Department of Dermatology, University of Cologne, Germany, Dr. Monique Aumailley, Institut für Biochemie, University of Cologne, Germany, and Dr. Oliver Distler, Department of Rheumatology, University Hospital, Zurich, Switzerland, for reviewing an earlier version of the manuscript and for helpful suggestions.

Author Affiliations

From the Department of Medical Science and Surgery, Section of Clinical Medicine, Università Politecnica delle Marche, and Ospedali Riuniti — both in Ancona (A.G.); and the Department of Molecular and Cellular Biology and Pathology, Institute of Endocrinology and Experimental Oncology, Consiglio Nazionale delle Ricerche, University of Naples Federico II, Naples (E.V.A.) — all in Italy; and the Department of Dermatology, University of Cologne, Cologne, Germany (T.K.).

Address reprint requests to Dr. Gabrielli at the Department of Medical Science and Surgery, Section of Clinical Medicine, Polo Didattico, Via Tronto, 10, Ancona, Italy, or at .

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