The role of therapeutic plasma exchange in clinically amyopathic dermatomyositis with MDA-5 antibody: A case report and review of the literature.

Autor: Fine A; Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA., Karp JK; Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA., Peedin AR; Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Jazyk: angličtina
Zdroj: Journal of clinical apheresis [J Clin Apher] 2020 Sep; Vol. 35 (5), pp. 483-487. Date of Electronic Publication: 2020 Jul 18.
DOI: 10.1002/jca.21815
Abstrakt: 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.
(© 2020 Wiley Periodicals LLC.)
Databáze: MEDLINE
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