How is sclerodactyly managed in CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Various treatment regimens including corticosteroids, nonsteroidal anti-inflammatory drugs, D-penicillamine, IFN-gamma, cyclosporine, and cytostatic drugs have been used with limited success in scleroderma.

An open-label study of calcitriol had promising results; however, a recent double-blinded, placebo-controlled trial was too small to draw any conclusions. [115]  After retrospective data showed the benefits of D-penicillamine for scleroderma skin changes, Clements et al [116] performed the first randomized controlled trial of D-penicillamine in scleroderma. This trial compared high-dose D-penicillamine (750-1000 mg/d) to low-dose D-penicillamine (125 mg qod) in patients with early diffuse cutaneous scleroderma. The mean skin thickness score improved over 2 years of treatment in both groups, and no advantage was seen to using the higher dose of D-penicillamine. [116] This study had no placebo group and the authors concluded later that they were not able to tell whether either dose was effective or ineffective. D-penicillamine cannot, therefore, be recommended until placebo-controlled trials are conducted to show effectiveness.

The natural course of diffuse dermal sclerosis involves skin softening after 4-5 years; therefore, placebo-controlled trials are essential for determining an effective therapy. Skin involvement in limited scleroderma typically is not severe; therefore, attempts are not usually made to treat skin involvement.


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