Objective: Dermatomyositis (DM) patients typically present with proximal weakness and autoantibodies that are associated with distinct clinical phenotypes. We observed that DM patients with autoantibodies recognizing the nuclear matrix protein NXP-2 often presented with especially severe weakness. The aim of this study was to characterize the clinical features associated with anti–NXP-2 autoantibodies. Methods: There were 235 DM patients who underwent testing for anti–NXP-2 autoantibodies. Patient characteristics, including muscle strength, were compared between those with and without these autoantibodies. The number of cancer cases observed in anti–NXP-2-positive subjects was compared with the number expected in the general population. Results: Of the DM patients, 56 (23.8%) were anti–NXP-2-positive. There was no significant difference in the prevalence of proximal extremity weakness in patients with and without anti–NXP-2. In contrast, anti–NXP-2-positive patients had more prevalent weakness in the distal arms (35% versus 20%; P = 0.02), distal legs (25% versus 8%; P < 0.001), and neck (48% versus 23%; P < 0.001). Anti–NXP-2-positive subjects were also more likely to have dysphagia (62% versus 35%; P < 0.001), myalgia (46% versus 25%; P = 0.002), calcinosis (30% versus 17%; P = 0.02), and subcutaneous edema (36% versus 19%; P = 0.01) than anti–NXP-2-negative patients. Five anti–NXP-2-positive subjects (9%) had cancer-associated myositis, representing a 3.68-fold increased risk (95% confidence interval 1.2–8.6) compared to the expected prevalence in the general population. Conclusion: In DM, anti–NXP-2 autoantibodies are associated with subcutaneous edema, calcinosis, and a muscle phenotype characterized by myalgia, proximal and distal weakness, and dysphagia. As anti–NXP-2-positive patients have an increased risk of cancer, we suggest that they undergo comprehensive cancer screening.
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