Is juvenile dermatomyositis a different disease in children up to three years of age at onset than in children above three years at onset? A retrospective review of 23 years of a single center's experience.
Is juvenile dermatomyositis a different disease in children up to three years of age at onset than in children above three years at onset? A retrospective review of 23 years of a single center's experience.
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DOI：
10.1186/1546-0096-10-34
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发表时间：
2012-09-20
期刊：
影响因子：
--
通讯作者：
Spencer CH
中科院分区：
文献类型：
--
作者：
Patwardhan A;Rennebohm R;Dvorchik I;Spencer CH
我们检验了一个假设，即在一个中心发现的幼年型皮肌炎（JDM）发病年龄在3岁或3岁以下的儿童的病程和结局与发病年龄大于3岁的儿童不同。获得了机构审查委员会的批准，以回顾性审查自1988年1月以来的23年中在哥伦布，俄亥俄州的全国儿童医院儿科风湿病诊所就诊的78例0-18岁JDM患者的病历。诊断是由治疗的儿科风湿病学家作出的。并非所有患者都符合Bohan和Peter标准，因为肌肉活检和EMG并不总是进行，我们使用了修改后的JDM标准。关于病程和结局的数据收集自末次临床随访或至2010年7月1日。在SAS 9.1.3中，我们使用Wilcoxon双样本检验比较两个年龄组之间的数值变量，并使用logistic回归比较两个年龄组之间的分类变量。Minitab-16用于计算各种平均值、中位数、众数、标准差和范围。对于生存分析，我们使用Kaplan-Meier方法和log-rank检验。在全国儿童医院，两组的平均发病年龄分别为27个月和91个月。年轻和老年组从症状发作到诊断的平均时间分别为5.6个月和4.5个月，无统计学显著差异。年轻发病组有更多的女性（p=0.05），他们的疾病发作发生在典型的冬春季节（p=0.031）的频率较低。年轻发病组更可能有发热（p=0.029）和自身免疫性疾病家族史（p=0.012）。年轻发病组不太可能出现天芥菜疹（p=0.04）、Gottron征（p=0.049）、毛细血管袢异常（p=0.010）或肌酸激酶（CK，p=0.022）、天冬氨酸转氨酶（AST，p=0.021）或醛缩酶（p=0.035）升高。年轻发病组在诊断时接受甲基强的松龙冲击治疗的频率较低（p=0.043），接受羟氯喹治疗的频率较低（p=0.035）。两组之间在初始口服类固醇剂量（p=0.8017）、诊断时接受甲氨蝶呤治疗的患者数量（p=0.709）和接受其他免疫抑制剂治疗的患者数量（p=0.323）方面没有差异。平均和最大持续时间（年轻和老年发病组的平均持续时间分别为24.3个月和35.2个月，最长持续时间分别为51个月和124个月）以及口服类固醇治疗的平均和最长持续时间（年轻组和老年组的平均持续时间分别为16.8个月和33.3个月，最长持续时间分别为50个月和151个月），年轻组较短。年轻患者在诊断后5年（9% vs. 35.7%，p=0.015）和10年（9% vs. 45.1%，p=0.011，表7）时活动性疾病的可能性较小。年轻患者发生骨坏死的可能性较小（p=0.023）。年轻组中有两例与疾病相关的死亡，老年组中没有。生存分析结果显示，各年龄组间差异有统计学意义（p < 0.012）。性别和种族差异无统计学意义（分别为p> 0.26和p>0.95）。在我们的中心，与年龄较大的患者相比，发病年龄在3岁或以下的JDM患者之间存在显著差异。在我们的队列中，年轻患者在诊断时的典型表现较少，病程较轻，不需要长时间的皮质类固醇和免疫抑制。发病年龄较轻的患者死亡率较高，但死亡率不常见且数量较少。年轻组的并发症发生率与老年组相似，但骨坏死在老年组中较高。这些发现与之前的报告不同，即JDM的发病年龄较低通常与更严重的疾病相关，因为我们中心的结果表明，患有年轻发病JDM的儿童似乎是非典型的，但与年龄较大的JDM患者相比可能表现良好。
We tested the hypothesis that the course and outcome of juvenile dermatomyositis (JDM) in children seen at one center with the JDM disease onset at or below three years of age is different from that in the children with disease onset at greater than three years of age. Institutional Review Board approval was obtained to retrospectively review the charts of 78 patients from age 0–18 years with JDM seen in the pediatric rheumatology clinic at Nationwide Children’s Hospital in Columbus, Ohio over the past 23 years from January 1988. The diagnosis was made by the treating pediatric rheumatologist. Not all the patients met the Bohan and Peter criteria, as muscle biopsy and EMG were not always performed and we utilized a modified JDM criteria. The data regarding disease course and outcome were collected as of the last clinic follow-up or to July 1, 2010. We used the Wilcoxon Two-Sample test to compare numerical variables between two age groups, and used logistic regression to compare categorical variables between two age groups in SAS 9.1.3. Minitab-16 was used to calculate various mean, median, modes, standard deviations and range. For survival analysis, we used Kaplan-Meier method with log-rank test. The mean age of onset in the two groups at Nationwide Children’s Hospital was 27 months and 91 months. The mean times between onset of symptoms to diagnosis in the younger and older age groups was 5.6 months and 4.5 months, respectively, not a statistically significant difference. The younger onset group had more females (p=0.05) and their disease onset occurred less frequently during the typical winter-spring seasons (p=0.031). The younger onset group was more likely to have a preceding fever (p=0.029) and family history of autoimmune diseases (p=0.012). The younger onset group was less likely to have heliotrope rash (p=0.04), Gottron’s sign (p=0.049), capillary loop abnormalities (p=0.010), or elevations in creatine kinase (CK, p=0.022), aspartate aminotransferase (AST, p=0.021) or aldolase (p=0.035). The younger onset group was treated less often with pulse methylprednisolone at diagnosis (p=0.043) and less often with hydroxychloroquine (p=0.035). There were no differences between the two groups regarding initial oral steroid dose (p=0.8017), number of patients who received methotrexate at diagnosis (p=0.709), and the number who ever received other immunosuppressants (p=0.323). The mean and maximum duration (mean duration 24.3 months vs. 35.2 months, maximum duration 51 vs. 124 months in younger and older onset group respectively) of methotrexate therapy, and the mean and maximum duration of oral steroid therapy (Mean duration 16.8 months vs. 33.3 months, maximum duration 50 vs. 151 months in younger and older onset group respectively), was shorter in the younger group. The younger onset patients were less likely to have active disease at 5 years (9% vs. 35.7%, p=0.015) and 10 years post-diagnosis (9% vs. 45.1%, p=0.011, Table 7). The younger patients were less likely to have osteonecrosis (p=0.023). Two disease-related deaths occurred in the younger group, none in the older group. The results of the survival analysis showed that the difference between the age groups was statistically significant (p < 0.012). The sex and race were not significant (p> 0.26 and p>0.95, respectively). There were significant differences between JDM patients with disease onset at or below age three years at our center, compared to their older counterparts. Younger patients in our cohort had fewer typical findings at diagnosis and a milder disease course without needing as long a duration of corticosteroids and immunosuppression. Patients with a younger onset had a higher mortality rate but mortalities were unusual and numbers small. The younger group had a similar complication rate compared to the older onset patients, except for osteonecrosis which was higher in the older onset group. These findings differ from the previous reports that a younger age of onset in JDM is often associated with a more severe disease, as results at our center suggest that children with younger onset JDM appear to be atypical but may do well compared to the older JDM patients.
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DOI：
10.1016/0190-9622(91)70153-s
发表时间：
1991-06-01
影响因子：
13.8
作者：
EUWER, RL;SONTHEIMER, RD
通讯作者：
SONTHEIMER, RD
影响因子：
5.5
作者：
Manlhiot, C.;Liang, L.;Feldman, B. M.
通讯作者：
Feldman, B. M.
影响因子：
5
作者：
HERNANDEZ, RJ;SULLIVAN, DB;KEIM, DR
通讯作者：
KEIM, DR
DOI：
10.1590/s1516-31802000000200002
发表时间：
2000-03-02
期刊：
Sao Paulo medical journal = Revista paulista de medicina
影响因子：
--
作者：
Hilario, M O;Yamashita, H;Lederman, H
通讯作者：
Lederman, H
影响因子：
5.1
作者：
BITNUM, S;HOPPS, HC;TRAVIS, LB
通讯作者：
TRAVIS, LB