Ibarra Maria, Rigsby Cynthia, Morgan Gabrielle A, Sammet Christina L, Huang Chiang-Ching, Xu Dong, Targoff Ira N, Pachman Lauren M
Division of Pediatric Rheumatology, Children's Mercy Hospital , 2401 Gillham Road, Kansas City, Missouri, 64108-4619, USA.
Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Pediatr Rheumatol Online J. 2016 Nov 29;14(1):64. doi: 10.1186/s12969-016-0123-3.
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.
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.
Ten JIIM, mean age 14.54 ± 4.54 years, 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 0.5 cm (from 2.79 ± 1.98 cm to 2.29 ± 2.25 cm). The average effective radiation dose was 0.007 ± 0.002, 0.010 ± 0.005, and 0.245 mSv for the upper extremity, lower extremity and chest, respectively (compared to a standard chest x-ray-- 0.02mSV effective dosage).
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.
营养不良性钙化可发生于青少年特发性炎性肌病(JIIM)患者以及其他结缔组织和代谢性疾病患者中,但尚未建立一种可靠的测量这些钙化体积的方法。本研究的目的是确定低剂量、有限层厚的计算机断层扫描(CT)在一段时间内客观测量JIIM患者原位钙化体积的可行性。
在2年期间前瞻性招募了10例有钙化的JIIM患者(8例皮肌炎,2例重叠综合征),进行两次有限的、低剂量、4层CT扫描。使用CT后处理工作站计算钙化体积。其他患者数据包括:疾病活动评分(DAS)、儿童肌炎评估量表(CMAS)、肌炎特异性抗体(MSA)以及TNFα - 308启动子区域A/G多态性。统计分析采用Pearson相关系数、配对t检验和描述性统计。
10例JIIM患者,平均年龄14.54±4.54岁,未治疗疾病持续时间为8.68±5.65个月。MSA状态:U1RNP(1例)、PM - Scl(1例)、Ro(1例,4例不确定)、p155/140(2例)、MJ(3例)、Mi - 2不确定(1例)、阴性(3例)。8例皮肌炎患者中有4例(50%)TNF - α - 308 A+。总体而言,从第一次CT检查到第二次CT检查,钙化体积倾向于减少0.5 cm(从2.79±1.98 cm降至2.29±2.25 cm)。上肢、下肢和胸部的平均有效辐射剂量分别为0.007±0.002、0.010±0.005和0.245 mSv(与标准胸部X线相比——有效剂量为0.02 mSv)。
我们得出结论:1)有限低剂量CT技术可提供关于JIIM中钙化体积的客观数据;2)测量肢体中的钙化体积辐射暴露最小;3)该方法可能有助于评估JIIM营养不良性钙化治疗的疗效。