How would you approach management of a patient with rapidly progressive systemic sclerosis with worsening skin disease, myositis, arthritis, dysphagia and failure to thrive developing within 6 months?

This is a unique subset of patients with very aggressive disease and high risk for poor outcomes with myopathy, poor GI dysmotility, at risk for early PH. We tend to treat them aggressively. I would consider rapid escalation of immunosuppression such as MMF and consider IVIG up front as well, especi...