How is skin thickening treated in Systemic Sclerosis?
Based on limited data, therapeutic options for skin thickening primarily include mycophenolate mofetil (MMF), methotrexate, or cyclophosphamide (CYC).
Small series and anecdotal evidence suggest that there is a possible role for high-dose intravenous immunoglobulin (2 g/kg per month), and studies are underway to assess whether tocilizumab, abatacept, or other novel agents have a role in the treatment of scleroderma. Data from clinical trials of autologous hematopoietic stem cell transplantation (HSCT) demonstrate that this modality can be effective for skin thickening as well.
Given significant treatment-related morbidity and mortality, this treatment should only be considered for the more severe or refractory cases that have failed conventional therapy.
Low-dose prednisone (<15 mg/day) and benzodiazepines can provide relief for the pruritus associated with the acute phase of skin thickening and may supplement conservative management with topical emollients (alcohol free), oatmeal baths, use of lukewarm (rather than hot) showers or baths, and avoidance of scratching (scratch-itch cycle).
Symptoms of pruritus may also be relieved with agents such as gabapentin or pregabalin; in recalcitrant disease, low-dose naltrexone may be considered based on limited reports in the literature.