The role of therapeutic plasma exchange in clinically amyopathic dermatomyositis with MDA‐5 antibody: A case report and review of the literature. Issue 5 (18th July 2020)

• Record Type: Journal Article
• Authors: Fine, Alexander; Karp, Julie K.; Peedin, Alexis R.
• Is Part Of: Journal of clinical apheresis. Volume 35:Issue 5(2020)
• Page Start: 483
• Page End: 487
• Publication Date: 2020-07-18
• Subjects: clinically amyopathic dermatomyositis -- dermatomyositis -- interstitial lung disease -- MDA‐5 -- therapeutic plasma exchange; Hemapheresis -- Periodicals; Blood -- Transfusion -- Periodicals; Blood -- Transfusion, Autologous -- Periodicals; Cell separation -- Periodicals; Leukapheresis -- Periodicals; Plasmapheresis -- Periodicals; 615.39
• DOI: 10.1002/jca.21815
• Languages: English
• ISSNs: 0733-2459

Abstract

Abstract: Clinically amyopathic dermatomyositis (CADM) is a rare, aggressive variant of dermatomyositis associated with interstitial lung disease (ILD) and refractoriness to immunosuppressants. Antibodies against melanoma differentiation‐associated gene 5 (MDA‐5) are often found in patients with CADM. We report a patient with advanced CADM with ILD and MDA‐5 antibodies who failed to improve with immunosuppressants. We performed 2 TPE over 3 days, using 5% albumin as replacement fluid. Although five total TPE were planned, he was transferred for lung transplant evaluation after the second TPE; he died 16 days after transfer without receiving a transplant. A literature review identified four patients with CADM and MDA‐5 antibodies treated with TPE; all experienced symptomatic improvement of their ILD. We attribute our patient's outcome to the advanced nature of his disease rather than a failure of TPE. Additional research may indicate a possible reclassification of CADM with MDA‐5 antibodies in future ASFA guidelines.