Di Giovanni C, Puggina S, Meneghel A, Vittadello F, Martini G, Zulian F
Department of Woman and Child Health, University of Padua, Via Giustiniani 3, 35128, Padova, Italy.
Affidea Group, Unix Radiology Service, Piove di Sacco, Italy.
Pediatr Rheumatol Online J. 2018 Jan 3;16(1):1. doi: 10.1186/s12969-017-0218-5.
To date, standardized methods for assessing the disease progression of linear scleroderma of the face (LSF) are lacking.
We investigated whether Cone Beam Computed Tomography (CBCT) may represent a reliable tool for assessing linear scleroderma of the face (LSF).
Ten patients with LSF and five age-matched controls underwent CBCT assessment. The transverse sections at three anatomic levels of the maxillofacial bones were analyzed. Measurements of soft tissue and total thickness of both affected and unaffected side of the face were made by a standardized methodology. Six raters evaluated CBCTs twice and blindly one from the other. The intra- and inter-rater reliability was assessed by the Intraclass Correlation Coefficient (ICC).
CBCT was fast and well tolerated by the patients. The inter-rater concordance for the total thickness was excellent, mean ICC 0.75 for patients, 0.89 for controls. The mean ICC for soft tissue thickness was 0.49 for patients, 0.66 for controls. 58.3% of the measurements for patients and 91.2% of those for controls showed excellent ICC results (≥ 0.75). The intra-rater concordance resulted optimal (ICC 0.77-0.99).
CBCT is a reliable technique to assess skin and bony changes of LSF.
迄今为止，尚缺乏评估面部线状硬皮病（LSF）疾病进展的标准化方法。
我们研究了锥形束计算机断层扫描（CBCT）是否可作为评估面部线状硬皮病（LSF）的可靠工具。
对10例LSF患者和5例年龄匹配的对照者进行CBCT评估。分析颌面骨三个解剖层面的横断面。采用标准化方法对面部患侧和未患侧的软组织厚度和总厚度进行测量。6名评估者对CBCT进行两次评估，且相互之间不知情。采用组内相关系数（ICC）评估评估者间和评估者内的可靠性。
患者对CBCT耐受性良好且检查速度快。总厚度的评估者间一致性极佳，患者的平均ICC为0.75，对照者为0.89。患者软组织厚度的平均ICC为0.49，对照者为0.66。患者测量值的58.3%和对照者测量值的91.2%显示ICC结果极佳（≥0.75）。评估者内一致性最佳（ICC 0.77 - 0.99）。
CBCT是评估LSF皮肤和骨骼变化的可靠技术。
