Division of Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA, USA.
Department of Pediatrics, Los Angeles General Medical Center, Los Angeles, CA, USA.
Rheumatol Int. 2024 Nov;44(11):2577-2582. doi: 10.1007/s00296-023-05385-4. Epub 2023 Jul 11.
Calcinosis is a sequela of Juvenile Dermatomyositis (JDM) with significant morbidity. A retrospective study observing risk factors for JDM calcinosis, including a possible association between higher intensity of subcutaneous and myofascial edema in initial magnetic resonance imaging (MRI) and development of calcinosis was performed at a tertiary pediatric medical center. Data from the past 20 years on JDM patients with MRIs at the time of JDM diagnosis were obtained. MRIs were individually evaluated by two pediatric musculoskeletal radiologists who blindly graded the intensity of edema on a 0-4 Likert scale. Clinical data and edema scores were compared between patients who developed calcinosis and who did not. Forty-three patients (14 with calcinosis and 29 without calcinosis) were identified. The calcinosis group contained more racial and ethnic minorities, younger ages of JDM onset and longer time to reach JDM diagnosis. Muscle enzyme levels at JDM diagnosis were lower in the calcinosis group, especially Creatinine Kinase (CK) (p = 0.047) and Alanine Aminotransferase (ALT) (p = 0.015). The median score for edema in both groups was 3 (p = 0.39) with an inter-rater reliability of 95%. There was no association between increased subcutaneous and myofascial edema in MRIs at the time of JDM diagnosis and development of calcinosis. Earlier age of JDM onset, racial and ethnic minority, and delay in JDM diagnosis could be risks for developing calcinosis. The calcinosis group presented with lower muscle enzyme levels at the time of JDM diagnosis, especially CK and ALT with statistical significance. This could reflect delay in diagnosis and treatment.
钙质沉着症是少年型皮肌炎(JDM)的后遗症,具有显著的发病率。在一家三级儿科医学中心,对 JDM 钙质沉着症的危险因素进行了回顾性研究,包括初始磁共振成像(MRI)中皮下和筋膜水肿强度与钙质沉着症发展之间的可能关联。获得了过去 20 年来在 JDM 诊断时具有 MRI 的 JDM 患者的数据。两名儿科肌肉骨骼放射科医生分别对 MRI 进行了评估,他们对水肿强度进行了 0-4 级的 Likert 评分。比较了发生钙质沉着症和未发生钙质沉着症的患者的临床数据和水肿评分。确定了 43 名患者(14 名有钙质沉着症,29 名无钙质沉着症)。钙质沉着症组包含了更多的种族和少数民族,JDM 发病年龄更小,达到 JDM 诊断的时间更长。钙质沉着症组的 JDM 诊断时肌肉酶水平较低,尤其是肌酸激酶(CK)(p=0.047)和丙氨酸氨基转移酶(ALT)(p=0.015)。两组的水肿中位数评分均为 3(p=0.39),组内观察者间的可靠性为 95%。JDM 诊断时 MRI 中皮下和筋膜水肿增加与钙质沉着症的发展之间没有关联。JDM 发病年龄较小、种族和少数民族以及 JDM 诊断延迟可能是发生钙质沉着症的危险因素。钙质沉着症组在 JDM 诊断时的肌肉酶水平较低,尤其是 CK 和 ALT,具有统计学意义。这可能反映了诊断和治疗的延迟。