Use of intravenous immunoglobulin in treatment for refractory dermatomyositis: a case report

INTRODUCTION: Dermatomyositis is a rare autoimmune disease. Its development is often associated with malignant solid tumors. Due to the polymorphism of clinical manifestations and low awareness of the primary care physicians of this disease, dermatomyositis remains undiagnosed for a long time, which leads to a late start of treatment. Untimely start of treatment is associated with a poor prognosis for this group of patients. Factors of poor prognosis include the development and progression of dysphagia and also refractoriness to the standard therapy with systemic glucocorticoids (sGC) and immunosuppressants, when it becomes necessary to use alternative methods of treatment, including intravenous immunoglobulin (IVIG).

AIM: Demonstration of a clinical case of dermatomyositis resistant to high-dose sGC and methotrexate therapy, that required high-dose IVIG therapy.

A clinical case of dermatomyositis in a female patient S., 56 years old, with cutaneous syndrome and muscle weakness, dysphagia and dysphonia is presented. On treatment with prednisolone 1 mg/kg a day orally, methotrexate 15 mg a week subcutaneously, clinical and laboratory dynamics was insignificant, poor tolerance to methotrexate did not permit to increase the dose. Taking into account progressing dysphagia, the medical commission took a decision about high-dose IVIG treatment at a dose 1g/kg once. After four weeks of therapy, clear positive clinical and laboratory dynamics was noted.

CONCLUSION: The described clinical case demonstrated the effectiveness and safety of IVIG for treatment of refractory dermatomyositis with insufficient effectiveness of the previous standard therapy with sGC and immune depressants.