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Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy.
- Source :
-
Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2020 Apr 01; Vol. 59 (4), pp. 767-771. - Publication Year :
- 2020
-
Abstract
- Objectives: We examined the effectiveness of plasma exchange (PE) therapy to reduce the mortality of rapidly progressive interstitial lung disease (RP-ILD) in patients positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.<br />Methods: Among 142 patients newly diagnosed with PM/DM or clinically amyopathic DM from 2008 to 2019 at our hospital, 10 were diagnosed with refractory RP-ILD and were positive for anti-MDA5 antibodies. PE was used as an adjunct to standard therapy and consisted of fresh frozen plasma as replacement solution. The primary outcome was non-disease-specific mortality.<br />Results: Anti-MDA5 antibodies were detected in 28 patients, of whom 21 were diagnosed with RP-ILD and 10 were refractory to intensive immunosuppressive therapy. Six patients received PE (PE group) and four did not (non-PE group). The 1-year survival rate of the PE group was higher than that of the non-PE group (100% and 25%, respectively, P = 0.033). Regarding adverse events associated with PE, two patients had anaphylactic shock, one had high fever due to fresh frozen plasma allergy and one had a catheter infection. All adverse events resolved with appropriate treatment.<br />Conclusion: We evaluated the association between 1-year survival rate and PE for refractory RP-ILD in patients positive for anti-MDA5 antibodies. Intensive immunosuppressive therapy improved the survival rate in RP-ILD patients with anti-MDA5 antibodies, but 20-30% of cases were still fatal. PE could be administered to patients with active infectious disease who were immunocompromised by intensive immunosuppressive therapy. PE may be considered in refractory RP-ILD patients positive for anti-MDA5 antibodies.<br /> (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
- Subjects :
- Adult
Aged
Anaphylaxis epidemiology
Anaphylaxis etiology
Dermatomyositis complications
Drug Resistance
Female
Humans
Lung Diseases, Interstitial complications
Lung Diseases, Interstitial immunology
Male
Middle Aged
Plasma
Plasma Exchange adverse effects
Plasmapheresis
Survival Rate
Transfusion Reaction epidemiology
Transfusion Reaction etiology
Treatment Outcome
Autoantibodies immunology
Dermatomyositis immunology
Immunosuppressive Agents therapeutic use
Interferon-Induced Helicase, IFIH1 immunology
Lung Diseases, Interstitial therapy
Plasma Exchange methods
Subjects
Details
- Language :
- English
- ISSN :
- 1462-0332
- Volume :
- 59
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Rheumatology (Oxford, England)
- Publication Type :
- Academic Journal
- Accession number :
- 31504956
- Full Text :
- https://doi.org/10.1093/rheumatology/kez357