Plasma Exchange for Refractory MDA5 Myositis and ILD
Abstract
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 resp/min), tachycardic (105 bpm), normotensive and afebrile. Laboratory assessment revealed creatine kinase (CK) of 500 fU/L, ESR 89mm/hr, CRP 1.2 mg/di and ferritin 2590 ng/ml. Tacrolimus trough was I 0.5 ng/ml. Blood gas analysis demonstrated pH 7.46, with C02 32.9 mmHg and HC03 22.8 mmol/L.
Document Details
- Document Type
- Technical Report
- Publication Date
- Apr 26, 2017
- Accession Number
- AD1037429
Entities
People
- Anthony Sidari
Organizations
- 59th Medical Wing