Which antifibrotic and immunomodulatory agents are used in the treatment of systemic sclerosis (SSc)?

Updated: Mar 17, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Answer

Antifibrotic agents have been investigated, although results have varied and none is clearly shown to be of consistent benefit. These have included D-penicillamine, interferon alfa and interferon gamma, and immunomodulatory agents. Immunomodulatory agents have included the following:

  • Photopheresis [49]

  • Corticosteroids (in inflammatory myositis, pericarditis, refractory arthritis, or alveolitis)

  • Methotrexate (15 mg/wk) [50]

  • Chlorambucil

  • Mycophenolate mofetil may benefit patients with diffuse progressive cutaneous systemic sclerosis of recent onset, a small prospective study suggested [51]

  • Cyclosporine [52]

  • FK506 (tacrolimus)

  • Thalidomide

  • Low-dose intravenous cyclophosphamide [53, 54]

  • Statins [55, 56]

  • Immunosuppressive drugs with autologous peripheral hematopoietic stem cell transplantation [57]

Because of their antifibrotic properties, the tyrosine kinase inhibitors imatinib mesylate and nintedanib may be potential therapies. [54, 55, 58, 59, 60]


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