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In these patients, immunosuppressive agents (methotrexate, azathioprine, mycophenolate mofetil, intravenous immunoglobulin, cyclophosphamide, chlorambucil, or cyclosporine) may be an effective means of inducing or maintaining remis­ sion. In addition, most patients are treated with an im­ munosuppressive regimen at disease onset in order to help with corticosteroid-sparing. Roughly half to threequarters of patients treated with an immunosuppressive agent respond, as evidenced by an increase in strength, a reduction in enzyme levels, or a reduction in corticoste­ roid dosage.

Antitopoisomerase antibodies, occurring in ∼20% of patients, are associated with dSSc and increased risks of ILD and mortality. RNA polymerase III antibodies, identifiable in ∼10% of patients, are associated with dSSc, scleroderma renal crisis, and underlying cancer. Importantly, negative SSc-specific autoantibody studies should not dissuade the clinician from making a diagnosis of SSc in the presence of a supportive clinical examination. Skin biopsy can be useful in atypical presentations to help differentiate SSc from sclerodermoid mimics.

The telangiectases of SSc, frequently referred to as “mat” telangiectases, have a characteristic flat, square, or matlike appearance and frequently involve the oral mucosa, helping to distinguish them from other etiologies. While hereditary hemorrhagic telangiectasia can present with prominent mucosal telangiectases, they tend to be more rounded and raised. Management As there is no cure for SSc, management is directed toward limiting the impact of individual disease manifestations, often with the help of several specialists and manifestation-specific medications.