Association of antinuclear antibody status with clinical features and malignancy risk in adult-onset dermatomyositis

J Am Acad Dermatol. 2019 May;80(5):1364-1370. doi: 10.1016/j.jaad.2018.11.023. Epub 2018 Nov 17.


Background: The clinical significance of antinuclear antibody (ANA) status in adults with dermatomyositis (DM) has yet to be fully defined.

Objective: We compared the incidence of amyopathic disease, risk of malignancy, and clinical findings in ANA+ and ANA- patients with adult-onset DM.

Methods: This was a retrospective cohort study of patients with ANA+ or ANA- adult-onset DM determined by enzyme-linked immunosorbent assay.

Results: Of 231 patients, 140 (61%) were ANA+ and 91 (39%) were ANA-. Compared with the ANA- patients, the ANA+ patients had a lower frequency of dysphagia (15% vs 26% [P = .033]) and heliotrope rash (38% vs 53% [P = .026]). In all, 54 patients (23%) developed malignancy within 3 years of diagnosis of their DM; 11% of the ANA+ patients developed malignancy versus 43% of the ANA- patients (P < .001). There was a strong association between ANA positivity and lower likelihood of malignancy in multivariable analysis (odds ratio, 0.16; P < .001). Conversely, ANA positivity was not associated with amyopathic disease (odds ratio, 0.94; P = .87).

Limitations: The retrospective nature of the study was a limitation.

Conclusion: In patients with adult-onset DM, ANA negativity is associated with increased likelihood of development of malignancy within 3 years of diagnosis of their DM. Particularly close follow-up and frequent malignancy screening may be warranted in ANA- individuals with DM.

Keywords: antinuclear antibody; connective tissue disease; dermatomyositis; inflammatory myopathy; malignancy; paraneoplastic.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Antinuclear / blood*
  • Deglutition Disorders / epidemiology
  • Dermatomyositis / blood*
  • Dermatomyositis / epidemiology
  • Exanthema / epidemiology
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Neoplasms / diagnosis
  • Neoplasms / epidemiology*
  • Retrospective Studies
  • Time Factors
  • Young Adult


  • Antibodies, Antinuclear

Supplementary concepts

  • Amyopathic dermatomyositis