Abstract
Background
Scleromyxedema is a primary fibro-mucinosis whose therapy is still challenging.
Objective
To evaluate the safety and efficacy of high-dose intravenous immunoglobulin (IVIg) for the management of scleromyxedema prospectically using an objective score.
Methods
In a prospective open-label study, IVIg was administered to 8 patients with scleromyxedema in a dose of 2 gr/Kg per month. The patients were followed up to a minimum of 6 months and their disease activity and response to treatment were assessed using the Physician's Global Assessment of disease severity (PGA) and a modified objective skin scoring system for patients with scleroderma (modified Rodnan score system for scleromyxedema or mRSSS). We used a stringent statistical nonparametric test, the Mann-Whitney test, to assess the changes in the mRSSS following therapy with IVIg.
Results
Eight patients were included (5 males) with a mean age of 59 years. Mean duration of scleromyxedema was 19 months (6-37 months). The mean duration of treatment was 36.5 months (range 7-74 months).The patients were followed-up for a minimum of 15 months to a maximum of 87 months (mean 44 months). The mean baseline mRSSS of our cohort was 82.38 (37-145, standard deviation 40.763) at the start of treatment, and this significantly decreased to 14.88 (0-37, standard deviation 12.988) (p=0.012) at the last clinical evaluation with a decrease of mRSSS of 81.6%. No considerable side effects were noted. Paraproteinemia remained substantially unchanged. In 6 cases, maintenance infusions were required to preserve disease control while in two patients therapy was stopped after 7 and 11 months. Relapses, however, occurred respectively after 6 and 25 months.
Conclusions
Our study is the first to demonstrate a statistically clinical objective improvement of clinical symptoms of scleromyxedema with IVIg.
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