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. 2021 Mar;8(3):571-578.
doi: 10.1002/acn3.51294. Epub 2021 Feb 8.

Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies

Chiseko Ikenaga  1 , Andrew R Findlay  1 , Namita A Goyal  2 , Sarah Robinson  1 , Jonathan Cauchi  2 , Yessar Hussain  3 , Leo H Wang  4 , Joshua C Kershen  5 , Brent A Beson  5 , Michael Wallendorf  6 , Robert C Bucelli  1 , Tahseen Mozaffar  2 , Alan Pestronk  1 , Conrad C Weihl  1
Affiliations

Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies

Chiseko Ikenaga et al. Ann Clin Transl Neurol. 2021 Mar.

Abstract

Objective: To define the clinicopathologic features and diagnostic utility associated with anti-cytosolic 5'-nucleotidase 1A (NT5C1A) antibody seropositivity in idiopathic inflammatory myopathies (IIMs).

Methods: Anti-NT5C1A antibody status was clinically tested between 2014 and 2019 in the Washington University neuromuscular clinical laboratory. Using clinicopathologic information available for 593 patients, we classified them as inclusion body myositis (IBM), dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy (IMNM), nonspecific myositis, or noninflammatory muscle diseases.

Results: Of 593 patients, anti-NT5C1A antibody was found in 159/249 (64%) IBM, 11/53 (21%) dermatomyositis, 7/27 (26%) antisynthetase syndrome, 9/76 (12%) IMNM, 20/84 (24%) nonspecific myositis, and 6/104 (6%) noninflammatory muscle diseases patients. Among patients with IBM, anti-NT5C1A antibody seropositive patients had more cytochrome oxidase-negative fibers compared with anti-NT5C1A antibody seronegative patients. Among 14 IBM patients initially negative for anti-NT5C1A antibody, three patients (21%) converted to positive. Anti-NT5C1A antibody seropositivity did not correlate with malignancy, interstitial lung disease, response to treatments in dermatomyositis, antisynthetase syndrome, and IMNM, or survival in IIMs.

Interpretation: Anti-NT5C1A antibody is associated with IBM. However, the seropositivity can also be seen in non-IBM IIMs and it does not correlate with any prognostic factors or survival.

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Conflict of interest statement

CI, ARF, SR, JC, YH, JCK, BAB, MW, and RCB report nothing to declare. NAG has received research support from Brainstorm Cell Therapeutics, Cytokinetics, Fulcrum, Kezar, Novartis, Octapharma, Orion, and Orphazyme. She has served on Advisory Boards for Acceleron, Alexion, Argenx, Biogen, CSL Behring, Cytokinetics, MT Pharma, Novartis, Sanofi Genzyme, Sarepta. In relation to these activities, she has received travel reimbursement and honoraria. She has also served on the speaker’s bureau for CSL. LHW reports grants from Muscular Dystrophy Association, grants from Friends of FSH Research.TM has served on advisory boards for Abbvie, Alexion, Amicus, Argenx, Audentes, Sanofi‐Genzyme, Sarepta, and Spark Therapeutics. In relation to these activities, he has received travel reimbursement and honoraria. He has also served on the speaker’s bureau for Alexion, CSL, Grifols, and Sanofi‐Genzyme. He serves on the medical advisory board for the Myositis Association, Neuromuscular Disease Foundation, Myasthenia Gravis Foundation of California, and Myasthenia Gravis Foundation of America. He has received travel funding from the Myositis Association and the Neuromuscular Disease Foundation. He has received research funding from the Myositis Association, the Muscular Dystrophy Association, and from the following sponsors: Alexion, Amicus, Argenx, Audentes, Bristol‐Myers‐Squib, Cartesian Therapeutics, Grifols, Momenta, Ra Pharmaceuticals, Sanofi‐Genzyme, Spark Therapeutics, UCB, and Valerion. He serves on the data safety monitoring board for Acceleron. AP has a patent TS‐HDS and other antibody testing methods with royalties paid. He received research support from Acceleron, Idera, Knopp, Cytokinetics, Biogen Idec, Fulcrum, Genzyme, Ionis, Sanofi, Ultragenyx, and personal fees from Athena. He holds stock in Johnson & Johnson. CCW reports grants and personal fees from Sarepta, personal fees from Acceleron, Casma therapeutics, and ML Bio.

Figures

FIGURE 1
FIGURE 1
Kaplan–Meier survival curves depending on the anti‐NT5C1A antibody status. (A) Kaplan–Meier survival curves of 212 patients with inclusion body myositis. (B) Kaplan–Meier survival curves of 438 patients with idiopathic inflammatory myopathies.
See this image and copyright information in PMC

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