THE TREATMENT of patients with diffuse cutaneous systemic sclerosis (SSc) remains unsatisfactory. Although the pathogenesis of this highly complex disease involves vascular injury and cellular and humoral autoimmunity, it is the progressive fibrosis affecting the skin and multiple organs that ultimately accounts for the most striking clinical features of the disease and is responsible for much of the associated morbidity and mortality. Attempts to treat fibrosis with empirically based therapies, such as penicillamine, colchicine, and immunomodulatory interventions (chlorambucil, methotrexate, fluorouracil, lymphoid irradiation, T-cell depletion, or photochemotherapy), have failed to consistently induce improvement in skin induration, the most readily measured clinical response.
Rational approaches to therapy are based on our emerging understanding of the pathogenesis of fibrosis. Potential targets for antifibrotic intervention include the activation and chemotaxis of immunocompetent cells, or their synthesis and secretion of potentially profibrotic mediators, such as transforming growth factor β; the extra-cellular activation or fibroblast receptor
Varga J. Recombinant Cytokine Treatment for Scleroderma: Can the Antifibrotic Potential of Interferon-? Be Realized Clinically? Arch Dermatol. 1997;133(5):637–642. doi:10.1001/archderm.1997.03890410093013
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