Early Initiation of Plasma Exchange Therapy for Anti-MDA5Dermatomyositis with Refractory Rapidly Progressive Interstitial Lung Disease.

Objectives Dermatomyositis (DM) is often associated with fatal anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive rapidly progressive interstitial lung disease (RP-ILD). RP-ILD often fails to respond to intensive treatment and has a poor prognosis. We examined the effectiveness of early plasma exchange therapy plus intensive treatment with high-dose corticosteroids and multiple immunosuppressants.

Early initiation of plasma exchange therapy for a patient with anti-MDA5 autoantibody-positive dermatomyositis developing rapidly progressive interstitial lung disease.

Dermatomyositis (DM) is a categorised as one of idiopathic inflammatory myopathy (IIM) indicated by symmetrical proximal muscle weakness as well as characteristic cutaneous manifestations typical of DM. Clinically amyopathic dermatomyositis (CADM), a subtype of DM, shows only the skin involvement without any clinical signs of myositis. This condition is often associated with fatal anti-MDA5 antibody-positive rapidly progressive interstitial lung disease (RP-ILD), especially in Eastern Asian populations.