Risk factors associated with calcinosis of juvenile dermatomyositis
Risk factors associated with calcinosis of juvenile dermatomyositis
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DOI：
10.2223/jped.1746
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发表时间：
2008-02-01
影响因子：
3.3
通讯作者：
Silva, Clovis A. A.
中科院分区：
文献类型：
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作者：
Sallum, Adriana M. E.;Pivato, Francine C. M. M.;Silva, Clovis A. A.
目的：目的：探讨儿童和青少年皮肌炎患者钙质沉着的危险因素。方法：对54例儿童皮肌炎患者的病历资料进行回顾性分析。收集的数据包括人口统计学特征、临床特征、肌肉力量（医学研究理事会量表I至V级），肺部受累（存在或不存在抗Jo 1抗体的限制性肺病）、胃肠道问题（胃食管反流）和/或心脏病（心包炎和/或心肌炎）;实验室检查：血清肌酶水平升高（肌酸磷酸激酶、天冬氨酸转氨酶、丙氨酸转氨酶和/或乳酸脱氢酶）;以及在给予的治疗中：单独或与羟氯喹和/或免疫抑制剂联合的皮质激素治疗。患者被分为两组，根据存在或不存在的钙质沉着症和数据进行了评估，单变量和多变量analysis.Results：钙质沉着症被确定在23（43%）例，并在6（26%）的患者出现在诊断前，而在17（74%），这是诊断后。单变量分析显示，心脏（p = 0.01）和肺（p = 0.02）受累以及需要一种或多种免疫抑制剂（甲氨蝶呤、环孢霉素A和/或静脉环磷酰胺冲击治疗）治疗幼年型皮肌炎（p = 0.03）均与钙质沉着症发病率增加相关。多变量分析表明，只有心脏受累（OR = 15.56; 95%CI 1.59-152.2）和使用一种或多种免疫抑制剂（OR = 4.01; 95%CI 1.08-14.87）与钙质沉着症的存在独立相关。钙质沉着症是一个常见的发展，这些青少年皮肌炎案件，一般出现的疾病进展。钙质沉着症与更严重的病例相关，这些病例也有心脏受累，治疗中必须包括免疫抑制剂。
Objective: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis.Methods: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses.Results: Calcinosis was identified in 23 (43%) patients, and in six (26%) patients it had emerged prior to diagnosis while in 17 (74%) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor(methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87) were independently associated with the presence of calcinosis.Conclusions: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.