title: Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options.
abstract: OBJECTIVE
  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.
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
authors.forename: Micaela
authors.initials: M
authors.lastname: Fredi
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia; and Università degli Studi di Brescia, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Bartoli
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili Brescia, Italy. ilariacava@virgilio.it.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Cavazzana
authors.affiliation: Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Rozzano (Milan); and BIOMETRA Department, University of Milan, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Ceribelli
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
authors.forename: Nice
authors.initials: N
authors.lastname: Carabellese
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia; and Università degli Studi di Brescia, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Tincani
authors.affiliation: Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Fukuoka, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Satoh
authors.affiliation: Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Franceschini
mesh_terms: D000251:Adenosine Triphosphatases / Q000276:immunology; D000328:Adult; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002114:Calcinosis / Q000097:blood / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D016009:Chi-Square Distribution; D004268:DNA-Binding Proteins / Q000276:immunology; D003882:Dermatomyositis / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D005095:Exoribonucleases / Q000276:immunology; D063326:Exosome Multienzyme Ribonuclease Complex / Q000276:immunology; D005260:Female; D006801:Humans; D007564:Japan; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016017:Odds Ratio; D017285:Polymyositis / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D004268:DNA-Binding Proteins; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human; D000251:Adenosine Triphosphatases; C551732:MORC3 protein, human