Anti-cortactin Autoantibodies Are Associated with Key Clinical Features in Adult Myositis but Are Rarely Present in Juvenile Myositis

Iago Pinal-Fernandez¹, Katherine Pak², Albert Gil-Vila³, Andres Baucells⁴, Benjamin Plotz⁵, Maria Casal-Dominguez⁶, Assia Derfoul⁷, Maria Angeles Martinez⁴, Albert Selva-O'Callaghan³, Sara Sabbagh⁸, Frederick W. Miller¹, Lisa G. Rider⁹, Lisa Christopher-Stine¹⁰ and Andrew Mammen⁶, ¹National Institutes of Health, Bethesda, MD, ²NIAMS, NIH, Bethesda, MD, ³Vall d’Hebron Hospital, Barcelona, Spain, ⁴Sant Pau Hospital, Barcelona, Spain, ⁵Division of Rheumatology, New York University Langone Health, New York, NY, ⁶NIH, Bethesda, MD, ⁷NIAMS, NIH, Bethesda, ⁸Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Washington, DC, ⁹Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Garrett Park, MD, ¹⁰Johns Hopkins University, Baltimore, MD

Meeting: ACR Convergence 2020

Keywords: Autoantibody(ies), autoimmune diseases, dermatomyositis, Myopathies, Myositis

Session Information

Date: Sunday, November 8, 2020

Title: Muscle Biology, Myositis & Myopathies Poster

Session Type: Poster Session C

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

Background/Purpose: To define the prevalence and clinical phenotype of anti-cortactin autoantibodies in adult and juvenile myositis.

Methods: In this longitudinal cohort study, anti-cortactin autoantibody tiers were assessed by ELISA in 670 adults and 343 juvenile myositis patients as well as 202 adult and 90 juvenile healthy controls. The prevalence of anti-cortactin autoantibodies was compared among groups. The clinical features of patients with and without anti-cortactin autoantibodies were also compared.

Results: The prevalence of anti-cortactin autoantibodies was not different between all adult myositis patients compared to adult healthy controls (11% vs. 8%; p=0.2). However, compared to adult healthy controls, anti-cortactin autoantibodies were more common in adult dermatomyositis (DM) patients (15%, p=0.005), particularly those with co-existing anti-Mi2 (24%, p=0.03) or anti-NXP2 (23%, p=0.04) autoantibodies. In adult myositis, anti-cortactin was associated with DM-skin involvement (62% vs. 38%, p=0.03), dysphagia (36% vs. 17%, p=0.02) and co-existing anti-Ro52 (47% vs. 26%, p=0.001) or anti-NT5C1a autoantibodies (59% vs. 33%, p=0.001). Moreover, the titers of anti-cortactin antibodies were higher in patients with interstitial lung disease (0.15 vs. 0.12 arbitrary units, p=0.03). The prevalence of anti-cortactin autoantibodies was no different in juvenile myositis (2%) or any juvenile myositis subgroup compared to juvenile healthy controls (4%). Nonetheless, juvenile myositis patients with these autoantibodies had a higher prevalence of mechanic’s hands (25% vs. 7%; p=0.03), a higher number of hospitalizations (2.9 vs. 1.3, p=0.04) and lower peak CK values (368 vs. 818 IU/L, p=0.02).

Conclusion: The prevalence of anti-cortactin autoantibodies is only increased in adult DM patients with co-existing anti-Mi2 or anti-NXP2 autoantibodies. In adults, anti-cortactin autoantibodies are associated with dysphagia and interstitial lung disease.

Disclosure: I. Pinal-Fernandez, None; K. Pak, None; A. Gil-Vila, None; A. Baucells, None; B. Plotz, None; M. Casal-Dominguez, None; A. Derfoul, None; M. Martinez, None; A. Selva-O'Callaghan, None; S. Sabbagh, None; F. Miller, None; L. Rider, NIEHS, NIH, 2, Cure JM Foundation, 2, Bristol Myers Squibb, 2, Hope Pharmaceuticals, 2, Eli Lilly and Company, 9, MedImmune/AstraZeneca, 9; L. Christopher-Stine, None; A. Mammen, None.

To cite this abstract in AMA style:

Pinal-Fernandez I, Pak K, Gil-Vila A, Baucells A, Plotz B, Casal-Dominguez M, Derfoul A, Martinez M, Selva-O'Callaghan A, Sabbagh S, Miller F, Rider L, Christopher-Stine L, Mammen A. Anti-cortactin Autoantibodies Are Associated with Key Clinical Features in Adult Myositis but Are Rarely Present in Juvenile Myositis [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/anti-cortactin-autoantibodies-are-associated-with-key-clinical-features-in-adult-myositis-but-are-rarely-present-in-juvenile-myositis/. Accessed .

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