To characterize the cutaneous and systemic clinical phenotype of dermatomyositis patients with anti-NXP-2 antibodies.We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. Electronic chart review employing a keyword search strategy was performed to collect clinical and laboratory data. Anti-NXP-2 antibodies were assayed by immunoprecipitation using NXP-2 produced by in vitro transcription/translation.Antibodies to NXP-2 were detected in 20 (11%) of the 178 patients. Anti-NXP-2 antibodies were associated with male gender (50% vs. 25%, p=0.02), dysphagia (74% vs. 39%, p=0.006), myalgia (89% vs. 52%, p=0.002), peripheral edema (35% vs. 11%, p=0.016), and calcinosis (37% vs. 11%, p=0.007). These patients were less likely to be clinically amyopathic (5% vs 23%, p=0.08). Five of the 20 patients with NXP-2 antibodies (25%) had an associated internal malignancy. No other cutaneous characteristics were associated with anti-NXP-2 antibodies except a decreased frequency of Gottron's sign (44% vs. 75%, p=0.012) and the fact that these patients were more likely to have mild skin disease.Dermatomyositis patients with anti-NXP2 antibodies have a distinct and often severe systemic phenotype that includes myalgia, peripheral edema and significant dysphagia despite having milder inflammatory skin disease. This article is protected by copyright. All rights reserved.
View details for DOI 10.1002/acr.23210
View details for PubMedID 28129490