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Abstract Number: 1073

Anti Melanoma Differentiation-associated Protein Gene 5 Antibody Titer Monitoring Is a Useful Indicator for Early Detection of Recurrence in Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis

Yuji Hosono1, Azusa Kojima2, Keigo Shimura2, Akira Ishii2, Yuto Izumi2, Kazuki Hirano2, Sho Sasaki2, Takayoshi Kurabayashi2, Noriko Sasaki2, Chiho Yamada2 and Shinji Sato2, 1Tokai University School of Medicine, Kanagawa, Japan, 2Tokai University School of Medicine, Isehara, Kanagawa, Japan

Meeting: ACR Convergence 2020

Keywords: Autoantibody(ies), interstitial lung disease, Myositis

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Session Information

Date: Sunday, November 8, 2020

Session Title: Muscle Biology, Myositis & Myopathies Poster

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Anti melanoma differentiation-associated gene 5 (MDA5) antibody (Ab) positive dermatomyositis (DM) often complicates rapidly progressive interstitial lung disease (RP-ILD), which shows fatal prognosis at early phase from the onset in spite of intensive immunosuppressive therapies. Although relapse of RP-ILD after remission with initial treatment is relatively rare in this condition, its long-term clinical course still remains unclear. Therefore, we investigated clinical characteristics and possible indicators for early detection of recurrence in patients with anti-MDA5 Ab positive DM and RP-ILD.

Methods: Adult Japanese DM patients who were treated at Tokai University Hospital from 2012 to 2019 were screened. Anti-MDA5 Ab was identified by immunoprecipitation with [35S] methionine-labeled HeLa cells, and anti-MDA5 Ab titers were measured using anti-MDA5 enzyme-linked immunoabsorbent assay. Patients who showed the exacerbation of RP-ILD and/or DM symptom after stabilized for more than a year were considered as recurrence. Clinical and immunological features were retrospectively collected. Statistical analyses were performed using the Fisher’s exact test.

Results: Thirty patients were diagnosed as DM with anti-MDA5 Ab. All had ILD and required hospitalization for intensive treatment. Seventeen of 30 (57%) achieved an improvement in respiratory function in response to their initial treatment and were discharged from the hospital. During their disease courses in outpatient care, 3 (18%) of 17 patients experienced recurrence and required re-hospitalization. Among 3 relapsed cases, 2 (67%) showed exacerbation of RP-ILD and remaining 1 (33%) revealed muscle weakness. Although all 3 relapsed patients showed anti-MDA5 Ab titer elevation at the time of relapse, no increase in the titer was seen in 14 non-relapsed patients (100% vs. 0%, P=0.002) Interestingly, marked increase of anti-MDA5 Ab titers preceded appearance of recurrent symptoms in all relapsed cases, whereas ferritin levels were within normal range at the recurrence (38-120 ng/dl) and gradually increased after readmission.

Conclusion: These results suggest that continuous measurement of anti-MDA5 Ab titer as well as careful observation of physical and imaging findings is crucial for monitoring disease activity and early detection of relapse in patients with anti-MDA5 Ab positive DM.


Disclosure: Y. Hosono, None; A. Kojima, None; K. Shimura, None; A. Ishii, None; Y. Izumi, None; K. Hirano, None; S. Sasaki, None; T. Kurabayashi, None; N. Sasaki, None; C. Yamada, None; S. Sato, None.

To cite this abstract in AMA style:

Hosono Y, Kojima A, Shimura K, Ishii A, Izumi Y, Hirano K, Sasaki S, Kurabayashi T, Sasaki N, Yamada C, Sato S. Anti Melanoma Differentiation-associated Protein Gene 5 Antibody Titer Monitoring Is a Useful Indicator for Early Detection of Recurrence in Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/anti-melanoma-differentiation-associated-protein-gene-5-antibody-titer-monitoring-is-a-useful-indicator-for-early-detection-of-recurrence-in-rapidly-progressive-interstitial-lung-disease-associated-wi/. Accessed February 26, 2021.
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