Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options

Clin Exp Rheumatol. Mar-Apr 2017;35(2):303-308. Epub 2016 Nov 14.

Abstract

Objectives: We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM).

Methods: We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014.

Results: 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled. Sixteen patients (21.6%) had calcinosis that occurred a mean of 43.7 months after diagnosis of PDM. At multivariate analysis, patients with calcinosis experienced longer follow-up duration (p=0.006), anti-PM/Scl (p=0.033) and anti-NXP2 (p=0.024) positivity compared to patients without calcinosis. Furthermore, anti-NXP-2 positive C+ showed a diffuse form of calcinosis from the beginning and lower frequency of respiratory tract involvement. No single drug or associations of drugs was found effective in the treatment of calcinosis.

Conclusions: A longer follow-up period of time, DM diagnosis and positivity for PM/Scl and NXP-2 could all be considered risk factors which foresee the development of calcinosis. Moreover, the positivity for antibodies to NXP-2 depicts a distinct phenotype of calcinosis with an early onset and quick widespread dissemination.

MeSH terms

  • Adenosine Triphosphatases / immunology
  • Adult
  • Autoantibodies / blood
  • Biomarkers / blood
  • Calcinosis / blood
  • Calcinosis / drug therapy
  • Calcinosis / etiology*
  • Calcinosis / immunology
  • Chi-Square Distribution
  • DNA-Binding Proteins / immunology
  • Dermatomyositis / blood
  • Dermatomyositis / complications*
  • Dermatomyositis / drug therapy
  • Dermatomyositis / immunology
  • Exoribonucleases / immunology
  • Exosome Multienzyme Ribonuclease Complex / immunology
  • Female
  • Humans
  • Japan
  • Logistic Models
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Odds Ratio
  • Polymyositis / blood
  • Polymyositis / complications*
  • Polymyositis / drug therapy
  • Polymyositis / immunology
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Treatment Outcome

Substances

  • Autoantibodies
  • Biomarkers
  • DNA-Binding Proteins
  • Exoribonucleases
  • Exosome Multienzyme Ribonuclease Complex
  • EXOSC10 protein, human
  • Adenosine Triphosphatases
  • MORC3 protein, human