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Patients with dermatomyositis, anti-NXP-2 antibodies have severe phenotype
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Patients with dermatomyositis and anti-NXP-2 antibodies had a severe systemic phenotype, which included myalgia, peripheral edema and significant dysphagia, according to recently published data.
“Like most rheumatic diseases, dermatomyositis (DM) can have a wide range of target organ manifestations,” David Fiorentino, MD, PhD, in the Department of Dermatology at the Stanford University School of Medicine, and colleagues wrote. “This heterogeneity of manifestations presents both challenge and opportunity — there is challenge in rendering the correct diagnosis but opportunity to assess if clinical nuance may have pathologic or prognostic significance.”
Fiorentino and colleagues conducted a retrospective cohort analysis of 178 patients with DM at the Stanford University Clinic. To collect lab and clinical data, researchers performed an electronic chart review with a keyword search strategy. In addition, they assayed anti-NXP-2 antibodies with immunoprecipitation.
Researchers detected anti-NXP-2 antibodies in 11% of patients. The antibodies were associated with male gender (50% vs. 25%), dysphagia (74% vs. 39%), myalgia (89% vs. 52%), peripheral edema (35% vs. 11%) and calcinosis (37% vs. 11%). In addition, the antibodies were associated with a reduced risk for being clinically amyopathic (5% vs. 23%) and having a decreased frequency of Gottron’s sign (44% vs. 75%). In addition, five of the 20 patients with anti-NXP-2 antibodies had an associated internal malignancy.
“[We] feel these data provide further evidence that clinical features, both common and rare, can be associated with antigen-specific immune responses and, when viewed as a collection, can help define an anti-NXP-2 ‘core phenotype’ which is variably expressed but often still recognizable in many patients,” the researchers wrote. – by Will Offit
Disclosure: The researchers report no relevant financial disclosures.
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