title: Cutaneous and Systemic Findings Associated With Nuclear Matrix Protein 2 Antibodies in Adult Dermatomyositis Patients.
abstract: To characterize the cutaneous and systemic clinical phenotype of dermatomyositis patients with antinuclear matrix protein 2 (anti-NXP-2) antibodies.
  
  
  
  We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. An 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 of the 178 patients (11%). Anti-NXP-2 antibodies were associated with male sex (50% versus 25%; P = 0.02), dysphagia (74% versus 39%; P = 0.006), myalgia (89% versus 52%; P = 0.002), peripheral edema (35% versus 11%; P = 0.016), and calcinosis (37% versus 11%; P = 0.007). These patients were less likely to be clinically amyopathic (5% versus 23%; P = 0.08). Five of the 20 patients with anti-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% versus 75%; P = 0.012) and a greater likelihood of having mild skin disease.
  
  
  
  Dermatomyositis patients with anti-NXP-2 antibodies have a distinct and often severe systemic phenotype that includes myalgia, peripheral edema, and significant dysphagia, despite having milder inflammatory skin disease.
authors.affiliation: Stanford University School of Medicine, Stanford, California.
authors.forename: Anna
authors.initials: A
authors.lastname: Rogers
authors.affiliation: Stanford University School of Medicine, Stanford, California, and Department of Veterans Affairs, Palo Alto Health Care System, Palo Alto, California.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.affiliation: Stanford University School of Medicine, Stanford, California.
authors.forename: Shufeng
authors.initials: S
authors.lastname: Li
authors.affiliation: Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Livia
authors.initials: L
authors.lastname: Casciola-Rosen
authors.affiliation: Stanford University School of Medicine, Stanford, California.
authors.forename: David F
authors.initials: DF
authors.lastname: Fiorentino
mesh_terms: D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002140:California; D004268:DNA-Binding Proteins / Q000276:immunology*; D003680:Deglutition Disorders / Q000209:etiology / Q000276:immunology; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D004487:Edema / Q000209:etiology / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D063806:Myalgia / Q000209:etiology / Q000276:immunology; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012867:Skin / Q000276:immunology* / Q000473:pathology; D014157:Transcription Factors / Q000276:immunology*
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; D004268:DNA-Binding Proteins; D014157:Transcription Factors; C087950:nuclear matrix protein 2