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Previous Volume 360:1989-2003 May 7, 2009 Number 19 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Scleroderma (systemic sclerosis) is a complex disease in which extensive fibrosis, vascular alterations, and autoantibodies against various cellular antigens are among the principal features (Figure 1 and Figure 2).¹ There are two major subgroups in the commonly accepted classification of scleroderma: limited cutaneous scleroderma and diffuse cutaneous scleroderma.² In limited cutaneous scleroderma, fibrosis is mainly restricted to the hands, arms, and face. Raynaud's phenomenon is present for several years before fibrosis appears, pulmonary hypertension is frequent, and anticentromere antibodies occur in 50 to 90% of patients. Diffuse cutaneous scleroderma is a rapidly progressing disorder that affects a large . . . [Full Text of this Article]

Epidemiology and Genetic Susceptibility

Early and Late Lesions

Early Vascular and Inflammatory Alterations

Fibrosis

Cell Types in Lesions

Endothelial Cells

Pericytes and Smooth-Muscle Cells

Fibroblasts

Mononuclear Cells

Soluble Mediators

Cytokines and Growth Factors

            TGF-β

            PDGF

            Other Cytokines and Biologically Active Substances

Extracellular-Matrix Components and Their Receptors

Autoantibodies

Reactive Oxygen Species

The Immune System, Oxidative Stress, and Fibrosis

Conclusions

Source Information

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 a.gabrielli@univpm.it.

Related Letters:

Scleroderma
Demoulin J.-B., Kämpe O., Rorsman F., Kapoor R., Kapoor J. R., Gabrielli A., Avvedimento E. V., Krieg T.
Extract | Full Text | PDF  
N Engl J Med 2009; 361:826-827, Aug 20, 2009. Correspondence

This article has been cited by other articles:

• Demoulin, J.-B., Kampe, O., Rorsman, F., Kapoor, R., Kapoor, J. R., Gabrielli, A., Avvedimento, E. V., Krieg, T. (2009). Scleroderma. NEJM 361: 826-827 [Full Text]