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.
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.
Regular pulmonary function testing is a cornerstone of assessment.
Continuous intravenous epoprostenol, subcutaneous or intravenous treprostinil, and bosentan all have important roles in selected patients with pulmonary arterial hypertension.
Early recognition of scleroderma renal crisis (SRC) and prompt treatment with angiotensin-converting enzyme (ACE) inhibitors has improved outcomes in SRC dramatically.
Cyclophosphamide is a cornerstone of interstitial lung disease treatment in SSc, but the therapeutic gains from this agent are relatively small.
■ 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.
KeywordsPulmonary Arterial Hypertension Interstitial Lung Disease Systemic Sclerosis Mycophenolate Mofetil Scleroderma Renal Crisis
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