Effect of abatacept added to mycophenolate mofetil for refractory calcinosis in juvenile dermatomyositis.

Abstract

Calcinosis is an intractable condition in juvenile dermatomyositis. The effect of abatacept on calcinosis remains controversial. We describe a case of an 8-year-old boy in whom the addition of abatacept to mycophenolate mofetil was effective against calcinosis in juvenile dermatomyositis. The patient with anti-nuclear matrix protein 2 autoantibody suffered from refractory juvenile dermatomyositis despite long-term treatment with corticosteroids and various immunosuppressive agents. Subcutaneous calcinosis with repeated spontaneous pain at the calcinosis site emerged 2 years after the start of treatment and gradually increased despite clinical improvement in muscle symptoms. The addition of intravenous abatacept to mycophenolate mofetil at the age of six halted the increase in calcinosis. Spontaneous pain in calcinosis disappeared within 2 months. The calcinosis had significantly decreased by 1 year. An important strategy on calcinosis in juvenile dermatomyositis is getting complete control of the inflammation. Our case report suggests that abatacept may contribute to the improvement of calcinosis in juvenile dermatomyositis, at least as part of combination therapy with mycophenolate mofetil.