High‐dose intravenous immunoglobulin therapy for scleromyxoedema: a prospective open‐label clinical trial using an objective score of clinical evaluation system. (12th April 2017)

• Record Type: Journal Article
• Authors: Guarneri, A.; Cioni, M.; Rongioletti, F.
• Is Part Of: Journal of the European Academy of Dermatology and Venereology. Volume 31:Number 7(2017)
• Page Start: 1157
• Page End: 1160
• Publication Date: 2017-04-12
• Subjects: Dermatology -- Periodicals; Sexually transmitted diseases -- Periodicals; 616.5
• DOI: 10.1111/jdv.14244
• Languages: English
• ISSNs: 0926-9959

Abstract

Abstract: Background: Scleromyxoedema 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 scleromyxoedema prospectively using an objective score. Methods: In a prospective open‐label study, IVIg was administered to eight patients with scleromyxoedema in a dose of 2 g/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 scleromyxoedema or mRSSS). We used a stringent statistical nonparametric test, the Mann–Whitney U ‐test, to assess the changes in the mRSSS following therapy with IVIg. Results: Eight patients were included (five males) with a mean age of 59 years. Mean duration of scleromyxoedema 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, SD 40.763) at the start of treatment, and this significantly decreased to 14.88 (0–37, SD 12.988) ( P = 0.012) at the last clinical evaluation with a decrease in mRSSS of 81.6%. No considerable side effects were noted. Paraproteinemia remained substantially unchanged. In six 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 scleromyxoedema with IVIg.