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Detection of Anti-MDA5 Autoantibodies Using HeLa Cells and Immunocytochemistry with Light Microscopy
Published on: October 31, 2025
Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy
Yoshiyuki Abe1, Makio Kusaoi1, Kurisu Tada1
1Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.
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.
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.
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.
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.
Insights
Plasma exchange (PE) therapy may improve survival for patients with rapidly progressive interstitial lung disease (RP-ILD) who test positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies. This treatment showed a higher 1-year survival rate in a small patient group.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Rapidly progressive interstitial lung disease (RP-ILD) associated with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies presents a significant mortality risk.
- Standard intensive immunosuppressive therapy has limitations, with a notable percentage of patients remaining refractory and experiencing fatal outcomes.
Purpose of the Study:
- To evaluate the effectiveness of plasma exchange (PE) as an adjunctive therapy for reducing mortality in patients with refractory anti-MDA5 antibody-positive RP-ILD.
- To assess the safety and impact of PE on survival rates in this specific patient cohort.
Main Methods:
- A retrospective analysis was conducted on 142 patients diagnosed with PM/DM or clinically amyopathic DM.
- Ten patients with refractory anti-MDA5 antibody-positive RP-ILD were identified; six received PE alongside standard therapy, while four did not.
- The primary outcome measured was non-disease-specific mortality, with a focus on 1-year survival rates.
Main Results:
- The 1-year survival rate was significantly higher in the PE group (100%) compared to the non-PE group (25%) (P = 0.033).
- Adverse events associated with PE included anaphylactic shock, fever due to plasma allergy, and catheter infection, all of which resolved with treatment.
- Despite intensive immunosuppressive therapy, a 20-30% fatality rate persists in anti-MDA5 positive RP-ILD cases.
Conclusions:
- Plasma exchange may be a viable therapeutic option for refractory anti-MDA5 antibody-positive RP-ILD, particularly in immunocompromised patients with active infections.
- PE can be considered as an adjunct to standard therapy to improve survival outcomes in this high-risk patient population.
- Further research is warranted to confirm the efficacy and safety profile of PE in managing anti-MDA5 antibody-associated RP-ILD.
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