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Abstract Number: 1344

Anti-Splicing Factor Proline/Glutamine-Richautoantibodies Rarely Co-Exist with Anti-Melanoma Differentiation-Associated Gene 5 Autoantibodies in a Cohort of Dermatomyositis Patients from the United States

Yuji Hosono1, Iago Pinal-Fernandez1, Katherine Pak1, Jemima Albayda2, Eleni Tiniakou3, Julie J. Paik4, Christopher A. Mecoli5, Sonye K. Danoff6, Lisa Christopher-Stine6 and Andrew Mammen1,7, 1National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 2Johns Hopkins University School of Medicine, Baltimore, MD, 3Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 4Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 5Internal Medicine/Rheumatology, Johns Hopkins University, Baltimore, MD, 6Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, 7Neurology, Johns Hopkins University School of Medicine, Baltimore, MD

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Antibodies, dermatomyositis, interstitial lung disease and myositis

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Session Information

Date: Monday, October 22, 2018

Session Title: Muscle Biology, Myositis and Myopathies Poster II: Basic and Translational Science

Session Type: ACR Poster Session B

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

Background/Purpose:

Anti-melanoma differentiation-associated gene 5 (MDA5) autoantibodies are common among Japanese dermatomyositis (DM) and clinically amyopathic DM (CADM) patients who develop rapidly progressive interstitial lung disease (RP-ILD). Recently, autoantibodies recognizing the 100 kDa splicing factor proline/glutamine-rich (SFPQ) were discovered to co-exist in half of Japanese anti-MDA5-positive DM patients. Anti-SFPQ autoantibodies were often absent in initial serum samples but appeared later during the course of disease and in a seasonal pattern. Anti-MDA5-positive patients with anti-SFPQ autoantibodies were older and had a higher prevalence of mechanic’s hands than those without the co-existing autoantibodies. To date, anti-SFPQ autoantibodies have not been described in anti-MDA5-positive patients outside of Japan. Thus, the purpose of this study was to identify the prevalence and clinical significance of anti-SFPQ autoantibodies among anti-MDA5-positive DM patients in a cohort of DM patients from the United States.

Methods:

We included all anti-MDA5-positive DM patients enrolled in a longitudinal cohort study at the Johns Hopkins Myositis Center. Anti-SFPQ autoantibodies were detected in patient serum samples collected at the first and most recent visits by immunoprecipitating radioactively-labeled proteins from [35S]-methionine-labeled HeK cells. The identities of immunoprecipitated proteins were confirmed by peptide mass fingerprinting.

Results:

Fifty-five anti-MDA5-positive DM/CADM patients were included in this study. From among these, autoantibodies recognizing the 100kDa autoantigen were detected in 4 (7.3%) of the DM/CADM patients. The corresponding polypeptide was confirmed to be SPFQ by mass spectrometry. Among the 4 patients with co-existing anti-MDA5 and anti-SFPQ autoantibodies, 1 was also positive for anti-U1-RNP autoantibodies and 1 was anti-Ro52-positive. All anti-SFPQ autoantibodies were detected at the time of first visit to the Johns Hopkins Myositis Center rather than appearing later during the disease course. 75% (3/4) of the anti-SFPQ antibody positive patients at initial symptom onset in March-April, and all had ILD; one of these died from severe ILD. The remaining patient had diagnosis in October and did not have ILD.

Conclusion:

Although more than half of Japanese anti-MDA5-positive DM patients eventually have co-existing anti-SFPQ autoantibodies, only 7.3% of anti-MDA5-positive DM patients in this United States cohort were positive for anti-SFPQ autoantibodies. These findings highlight an important difference between anti-MDA5-positive DM/CADM patients in the United States and Japan.


Disclosure: Y. Hosono, None; I. Pinal-Fernandez, None; K. Pak, None; J. Albayda, None; E. Tiniakou, None; J. J. Paik, None; C. A. Mecoli, None; S. K. Danoff, None; L. Christopher-Stine, None; A. Mammen, None.

To cite this abstract in AMA style:

Hosono Y, Pinal-Fernandez I, Pak K, Albayda J, Tiniakou E, Paik JJ, Mecoli CA, Danoff SK, Christopher-Stine L, Mammen A. Anti-Splicing Factor Proline/Glutamine-Richautoantibodies Rarely Co-Exist with Anti-Melanoma Differentiation-Associated Gene 5 Autoantibodies in a Cohort of Dermatomyositis Patients from the United States [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/anti-splicing-factor-proline-glutamine-richautoantibodies-rarely-co-exist-with-anti-melanoma-differentiation-associated-gene-5-autoantibodies-in-a-cohort-of-dermatomyositis-patients-from-the-united-st/. Accessed May 26, 2022.
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