Plasma Exchange for Refractory MDA5 Myositis and ILD
59th Medical Wing San Antonio United States
Pagination or Media Count:
Introduction MDA5 antibody associated idiopathic inflammatory myopathy JIM is associated with an increased risk for development of interstitial lung disease ILD, may be more resistant to corticosteroid treatment and is often rapidly progressive. Treatment of HM-associated ILD varies with use of corticosteroids, DMARDs, and biologic treatments. Plasmapheresis may effectively treat systemic disease by removal of autoantibodies, immune complexes and various cytokines. We present a case of refractory myositis with significant improvement after initiation of plasma exchange. Case Description A 55 year old African American male with history of anti-MDA5 dermatomyositis complicated by ILDon tacrolimus 4mg twice daily, prednisone 30mg daily and monthly intravenous immune globulin presented acutely with severe dyspnea, dysphagia, odynophagia and severe weakness while undergoing prednisone taper. He was admitted to the ICU tachypneic 22 respmin, tachycardic 105 bpm, normotensive and afebrile. Laboratory assessment revealed creatine kinase CK of 500 fUL, ESR 89mmhr, CRP 1.2 mgdi and ferritin 2590 ngml. Tacrolimus trough was I 0.5 ngml. Blood gas analysis demonstrated pH 7.46, with C02 32.9 mmHg and HC03 22.8 mmolL.
- Medicine and Medical Research