The role of therapeutic plasma exchange in clinically amyopathic dermatomyositis with MDA-5 antibody: A case report and review of the literature.
J Clin Apher
; 35(5): 483-487, 2020 Sep.
Article
in English
| MEDLINE | ID: covidwho-801272
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.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Plasma Exchange
/
Autoantibodies
/
Dermatomyositis
/
Interferon-Induced Helicase, IFIH1
Type of study:
Case report
/
Experimental Studies
/
Prognostic study
/
Reviews
Topics:
Variants
Limits:
Humans
/
Male
/
Middle aged
Language:
English
Journal:
J Clin Apher
Year:
2020
Document Type:
Article
Affiliation country:
Jca.21815
Similar
MEDLINE
...
LILACS
LIS