The Utility of Intravenous Immunoglobulin (IVIG) in the Treatment of Anti-Melanoma Differentiation Associated Protein 5 (anti-MDA5) Dermatomyositis (DM)

Publication/Presentation Date

8-5-2020

Abstract

Anti-MDA5 positive amyopathic dermatomyositis is a rare variant known to be associated with rapidly progressive interstitial lung disease without well-defined optimal therapy and variable prognosis. Our case explores the use of intravenous immunoglobin (IVIG) as a maintenance therapy for this rare entity.A 71-year-old male presented with nonspecific symptoms of fatigue, weight loss, and progressive dyspnea. Initial evaluation suggested ILD of unclear etiology on CT imaging and confirmatory biopsy. Approximately one year later, the patient presented with symmetric hand swelling with polyarthralgia and fissuring of the palmar surfaces. He was also noted to have violaceous and erythematous redness over his face, chest, and neck. This prompted the workup for amyopathic DM, more specifically anti-synthetase syndrome. He had negative serologies for Aldolase, ANA, Anti-Jo-1, RF, and anti-CCP. His CK was elevated and Anti-Mi-2 was not obtained. Further evaluation at confirmed the diagnosis of Anti-MDA5 DM. The patient was stared on pulse steroids, and a brief course of tacrolimus followed by IVIG. He had imaging and functional improvement. He is currently under consideration for adding rituximab onto his clinical regimen.Anti-MDA5 dermatomyositis can be a diagnostic challenge for most physicians and requires expert consultation for initiating treatment. The current treatment approach varies based on severity of presentation and patient tolerance of the available agents. One Japanese trial demonstrated that the overall 6-month survival rate for anti-MDA5 with ILD is 50%-60%. Although smaller trials have occurred, this case adds to the literature on the role of IVIG in Anti-MDA5 dermatomyositis associated ILD. treated with IVIG when compared to other immunosuppressive therapies such as intravenous cyclophosphamide. Agents such as tacrolimus and rituximab have also demonstrated good results with resolution of ILD. This case report highlights the importance of considering IVIG in patients with anti-MDA5 dermatomyositis. This is a rare subtype of dermatomyositis, with a severe ILD, and highlights the need for additional investigation and comparison of the available treatment strategies.

Disciplines

Medicine and Health Sciences

Department(s)

Department of Medicine, Department of Medicine Faculty

Document Type

Presentation

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