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

1, 2, 3, 4, 5, 6, 7, 8

 

  1. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
  2. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia; and Università degli Studi di Brescia, Italy.
  3. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili Brescia, Italy. ilariacava@virgilio.it
  4. Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Rozzano (Milan); and BIOMETRA Department, University of Milan, Italy.
  5. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
  6. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia; and Università degli Studi di Brescia, Italy.
  7. Department of Clinical Nursing, School of Health Sciences, University of Occupational and Environmental Health, Fukuoka, Japan.
  8. Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.

CER9657
2017 Vol.35, N°2
PI 0303, PF 0308
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PMID: 27908312 [PubMed]

Received: 13/06/2016
Accepted : 07/09/2016
In Press: 14/11/2016
Published: 15/03/2017

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.

Rheumatology Article