Complete, refractory dysphagia in a dermatomyositis patient with positive anti-NXP-2 antibodies

Abstract

Dermatomyositis (DM) is a rare autoimmune disorder defined by weakness of the striated muscles and a distinctive skin rash. Dysphagia is a serious symptom that can be difficult to manage, severely impacting quality of life and long-term survival. The aim of this report is to highlight a case of an anti-NXP-2 positive DM with severe dysphagia refractory to multiple therapies, including steroids, cyclophosphamide and intravenous immunoglobulins. Anti-NXP-2 autoantibodies indicate a specific disease phenotype adding severe muscle weakness, dysphagia, peripheral edema and underlying malignancy.