Monitoring change in volume of calcifications in juvenile idiopathic inflammatory myopathy: A pilot study using low dose computed tomography

Maria Ibarra, Cynthia Rigsby, Gabrielle A. Morgan, Christina L. Sammet, Chiang Ching Huang, Dong Xu, Ira N. Targoff, Lauren M. Pachman*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Background: Dystrophic calcifications may occur in patients with J uvenile Idiopathic Inflammatory Myopathy (JIIM) as well as other connective tissue and metabolic diseases, but a reliable method of measuring the volume of these calcifications has not been established. The purpose of this study is to determine the feasibility of low dose, limited slice, Computed Tomography (CT) to measure objectively in-situ calcification volumes in patients with JIIM over time. Methods: Ten JIIM patients (eight JDM, two Overlap) with calcifications were prospectively recruited over a 2-year period to undergo two limited, low dose, four-slice CT scans. Calculation of the volume of calcifications used a CT post processing workstation. Additional patient data included: Disease Activity Scores (DAS), Childhood Myositis Assessment Scale (CMAS), myositis specific antibodies (MSA), and the TNFα-308 promoter region A/G polymorphism. Statistical analysis utilized the Pearson correlation coefficient, the paired t-test and descriptive statistics. Results: Ten JIIM, mean age 14.54±4.54years, had a duration of untreated disease of 8.68±5.65 months MSA status: U1RNP (1), PM-Scl (1), Ro (1, 4 indeterminate), p155/140 (2), MJ (3), Mi-2 indeterminate (1), negative (3). 4/8 JDM (50%) were TNF-α-308 A+. Overall, the calcification volumes tended to decrease from the first to the second CT study by 0.5cm3 (from 2.79±1.98cm3 to 2.29±2.25cm3). The average effective radiation dose was 0.007±0.002, 0.010±0.005, and 0.245mSv for the upper extremity, lower extremity and chest, respectively (compared to a standard chest x-ray-- 0.02mSV effective dosage). Conclusion: We conclude: 1) the limited low dose CT technique provides objective data about volume of the calcifications in JIIM; 2) measuring the volume of calcifications in an extremity is associated with minimal radiation exposure; 3) This method may be useful to evaluate the efficacy of therapies for JIIM dystrophic calcification.

Original languageEnglish (US)
Article number64
JournalPediatric Rheumatology
Volume14
Issue number1
DOIs
StatePublished - Nov 29 2016

Funding

Support for this study was provided by the Cure JM Foundation.

Keywords

  • Calcification
  • Calcification volume
  • Computed Tomography (CT)
  • Juvenileidiopathic inflammatory myopathy
  • Overlap syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Rheumatology
  • Immunology and Allergy

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