(1) Scleroderma and secondary Raynaud's phenomenon are frequently associated with increased morbidity for which no specific standardised treatment guidelines exist.
(2) Current therapies for scleroderma target the immune system, with the goal of reducing inflammation and secondary tissue injury and fibrosis. Therapy targeting underlying vascular disease is designed to improve the symptoms of Raynaud's phenomenon and to reduce ischemic injury to involved organs.
(3) Few controlled trials of therapy used for scleroderma are completed, and current treatments are largely based on organ-specific therapy and uncontrolled case series suggesting disease modification.
(4) Recent randomised, controlled trials in scleroderma demonstrate promising results in the treatment of interstitial lung disease with cyclophosphamide, and vascular disease of the lungs and digits with endothelin receptor antagonists, the phosphodiesterase inhibitor sildenafil and prostacyclins, while trials with methotrexate show only modest benefit in controlling scleroderma-associated skin disease.
(5) Prostacyclins are a therapeutic option in patients with secondary Raynaud's phenomenon. Modest benefits have also been shown with α1-antagonists and calcium channel blockers, while the effect of ACE inhibitors has been variable. Some data suggest some benefits to the use of the phosphodiesterase inhibitor sildenafil, the serotonin uptake inhibitor fluoxetine and the angiotensin receptor inhibitor losartan.