Topics:
Rheumatology • Systemic sclerosis • Scleroderma • Dermatology • Systemic autoimmune diseases
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?
The patient is a young African American female of reproductive age with positive Fibrillarin antibody and nucleolar antibody. No ILD.
Answer from: at Academic Institution
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...
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