Anti-MDA5 Antibody-positive Dermatomyositis with Rapidly Progressive Interstitial Pneumonia Presenting with Nephrotic Syndrome during Treatment with Corticosteroids and Cyclosporine

Abstract

A 50-year-old Japanese woman with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis presenting with rapidly progressive interstitial pneumonia was treated with corticosteroids and cyclosporine. She developed nephrotic syndrome during the treatment regimen with corticosteroids and cyclosporine. A kidney biopsy revealed a thrombotic microangiopathy (TMA) glomerular lesion. Anti-MDA5 antibody-positive dermatomyositis is prone to severe interstitial lung disease (ILD) and is often exacerbated and refractory to treatment. Renal symptoms might be due to TMA of the kidney, and this may be a sign that more intensive treatment is needed. Patients sometimes develop acute kidney injury, which may be due to the TMA.

Vasculopathy in dermatomyositis

“…In addition, CNIs, including tacrolimus and cyclosporine A, may hinder VEGF-mediated angiogenesis. Kawamoto et al [13] reported a case of DM patient who developed secondary thrombotic microangiopathy which may be associated with CNIs, although the association is considered to be low. Janus kinase (JAK) inhibitors are considered a therapeutic option for refractory DM.…”