Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy.
Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy.
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青少年皮肌炎：诊断和治疗的新线索。
DOI：
10.1007/s40674-020-00168-5
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发表时间：
2021-03
影响因子：
1.2
通讯作者：
Khojah AM
中科院分区：
文献类型：
--
作者：
Pachman LM;Nolan BE;DeRanieri D;Khojah AM
确定疾病活动的线索并讨论治疗方案。诊断评估包括通过检查和 MRI 记录对称性近端肌肉损伤，以及升高的肌酶（醛缩酶、肌酸磷酸激酶、LDH 和 SGOT），这些酶通常会随着疾病未经治疗的持续时间较长而恢复正常。超声波可识别持续性、隐匿性的肌肉炎症。肌炎特异性抗体（MSA）和肌炎相关抗体（MAA）与特定的病程变异相关。抗 NXP-2 存在于年幼的儿童中，并与钙质沉着有关；抗TIF-1γ+青少年皮肌炎病程较长。诊断性皮疹（涉及眼睑、手、膝盖、面部和上胸部）是最持久的症状，与微血管损害相关，表现为甲襞（甲周）端排毛细血管消失。这种损失与口服泼尼松的生物利用度降低有关；还应考虑其他口服药物的生物利用度。诊断时，至少 3 天静脉注射甲基泼尼松龙可能有助于控制 HLA 限制性和 1/2 型干扰素驱动的炎症过程。避免紫外线照射的要求要求补充维生素 D。这种慢性疾病通常针对心血管系统；使用皮质类固醇后，死亡率从 30% 降低至 1-2%。新的血清学生物标志物表明隐匿性炎症：CXCL-10 预测病程更长。一些生物疗法似乎很有前景。
To identify clues to disease activity and discuss therapy options. The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation. This often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.
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影响因子：
3.4
作者：
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通讯作者：
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