Treatment options in systemic sclerosis

Introduction: The treatment of systemic sclerosis (SSc) still represents a challenge for all physicians because of the complexity of this disease. However, new insights into pathophysiology are driving new approaches to its treatment by acting on key targets of pathogenesis. Areas covered: The management of SSc consists of a symptomatic therapy and a disease modifying therapy. The aim of symptomatic treatment is to control different symptoms, clinical manifestations and organ-based complications while disease modifying therapy interferes with the main pathogenetic mechanisms in order to obtain remission, to stop disease progression and if possible to reverse disease-related skin and internal organ changes. This article will provide an overview of the different treatments for the most common features of SSc. A Medline search was focused on most relevant treatment literature contribution published in the past decade. Expert opinion: As for symptomatic treatment, calcium channel blockers (CCBs) should be considered as the first-line therapy for Raynaud's phenomenon (RP). In case of intolerance or ineffectiveness, a phosphodiesterase type 5 (PDE5) inhibitor alone or in association with CCB can be started. As for pulmonary arterial hypertension (PAH), both bosentan, ambrisentan and sildenafil, can be used as first-line treatments. In patients with organ involvement at risk for progression, cyclophosphamide (CYC) should be considered the disease modifying agent of choice. In case of contraindications to CYC, rituximab (RTX) and mycophenolate mofetil (MMF) have to be taken into account. After induction treatment, maintenance with MMF or azathioprine (AZA) has to be started.