Intravenous immunoglobulin (IVIG) is associated with improvements in patients with refractory cutaneous dermatomyositis (DM) regardless of DM subtype or clinical presentation, according to results from a retrospective cohort study published in Clinical and Experimental Dermatology.

Patients with refractory cutaneous DM were defined as those still having active cutaneous DM after being treated with ≥2 systemic medications. A total of 42 patients with refractory cutaneous DM were treated with IVIG: 15 with refractory skin disease alone; 25 with both refractory skin and muscle disease; 1 with refractory skin and interstitial lung disease; and 1 with refractory skin disease, myositis, and interstitial lung disease.

The mean time from diagnosis of DM to IVIG initiation was 671.6±1207.9 days; mean age at IVIG initiation was 49.9±20.42 years. IVIG was started for refractory cutaneous disease after a mean of 2.7±1.2 systemic medications had been used.

Overall, 83% of patients experienced cutaneous DM improvement, which included 87% who received treatment for refractory skin disease alone and 81% who were treated for refractory skin/muscle/lung disease. Improvements were noted after a mean of 1.82±1.38 IVIG cycles, regardless of DM subtype. The investigators noted that there were no significant predictors of response or nonresponse to IVIG treatment.

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Treatment with IVIG was also associated with decreased use of systemic glucocorticosteroids with or without a decrease in steroid-sparing immunosuppressive medications in 80% of patients.

The investigators reported several limitations of the study, including its retrospective design and its lack of an objective evaluation of cutaneous DM activity.

“These findings suggest that IVIG can be a clinically efficacious and cost-effective treatment for refractory cutaneous DM and warrants prospective study,” the investigators concluded. 

Reference

Galimberti F, Kooistra L, Li Y, Chatterjee S, Fernandez AP. Intravenous immunoglobulin is an effective treatment for refractory cutaneous dermatomyositis [published online June 1, 2018]. Clin Exp Dermatol. doi: 10.1111/ced.13607