Following the Molecular Pathways toward an Understanding of the Pathogenesis of Systemic Sclerosis

Reviews6 January 2004Following the Molecular Pathways toward an Understanding of the Pathogenesis of Systemic SclerosisSergio A. Jimenez, MD and Chris T. Derk, MDSergio A. Jimenez, MDFrom Thomas Jefferson University, Philadelphia, Pennsylvania.Search for more papers by this author and Chris T. Derk, MDFrom Thomas Jefferson University, Philadelphia, Pennsylvania.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-140-1-200401060-00010 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Clinical PrinciplesSystemic sclerosis is an autoimmune connective tissue disorder of unknown cause.It is clinically heterogeneous, ranging from limited skin involvement to diffuse skin sclerosis with severe internal organ involvement.The Raynaud phenomenon, swelling of the extremities, and diffuse arthralgias often precede the onset of skin tightness and induration.Visceral involvement with fibrosis, microvascular alterations, and mononuclear cell infiltration of the gastrointestinal tract, lungs, heart, and kidneys is present to a variable extent in most patients.Autoantibodies, some with very high specificity, are present almost universally.Mortality and morbidity are substantial and are directly related to the extent of the fibrotic and microvascular alterations.A better ...References1. 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Endothelins: effect on matrix biosynthesis and proliferation in normal and scleroderma fibroblasts. J Cardiovasc Pharmacol. 1998;31 Suppl 1:S360-3. [PMID: 9595482] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Sergio A. Jimenez, MD; Chris T. Derk, MDAffiliations: From Thomas Jefferson University, Philadelphia, Pennsylvania.For definition of terms used, see Glossary.Acknowledgments: The authors thank Kate Salmon for expert assistance in the preparation of the manuscript and M. Sonsoles Piera-Velazquez, PhD, for assistance in the preparation of some of the illustrations.Grant Support: By National Institutes of Health Grant AR19616. Dr. Derk was supported by National Institutes of Health Training Grant AR07583.Disclosures: None disclosed.Corresponding Author: Sergio A. Jimenez, MD, Division of Rheumatology, Thomas Jefferson University, 233 South 10th Street, Room 509 BLSB, Philadelphia, PA 19107-5541.Current Author Addresses: Dr. Jimenez: Division of Rheumatology, Thomas Jefferson University, 233 South 10th Street, Room 509 BLSB, Philadelphia, PA 19107-5541.Dr. Derk: Division of Rheumatology, Thomas Jefferson University, 1025 Walnut Street, Room 613 Curtis, Philadelphia, PA 19107-5541. 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