Abstract
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Systemic sclerosis (SSc; scleroderma) targets several aspects of disease pathophysiology: vascular features that are currently highly treatable; inflammatory features that are currently partly amenable to therapy; fibrotic features for which therapies of modest efficacy (at best) exist; and atrophic, end organ damage for which only supportive therapy is available.
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The extent of skin involvement is neither a robust primary outcome measure for clinical trials nor a reliable guide to the therapy of individual patients.
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Regular pulmonary function testing is a cornerstone of assessment.
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Continuous intravenous epoprostenol, subcutaneous or intravenous treprostinil, and bosentan all have important roles in selected patients with pulmonary arterial hypertension.
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Early recognition of scleroderma renal crisis (SRC) and prompt treatment with angiotensin-converting enzyme (ACE) inhibitors has improved outcomes in SRC dramatically.
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Cyclophosphamide is a cornerstone of interstitial lung disease treatment in SSc, but the therapeutic gains from this agent are relatively small.
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â– Long-term proton-pump inhibition is highly effective in treating the gastroesophageal reflux. High doses, sometimes two to three times the normal therapeutic dose, are required to alleviate symptoms.
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Buch, M.H., Seibold, J.R. (2008). Systemic Sclerosis. In: Klippel, J.H., Stone, J.H., Crofford, L.J., White, P.H. (eds) Primer on the Rheumatic Diseases. Springer, New York, NY. https://doi.org/10.1007/978-0-387-68566-3_45
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DOI: https://doi.org/10.1007/978-0-387-68566-3_45
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