Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity
Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity
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DOI：
10.1097/md.0b013e31816bc604
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发表时间：
2008-03-01
期刊：
影响因子：
1.6
通讯作者：
Rider, Lisa G.
中科院分区：
文献类型：
--
作者：
Bingham, April;Mamyrova, Gulnara;Rider, Lisa G.
我们描述了 28 名青少年皮肌炎 (JDM) 患者和 1 名成人皮肌炎 (DM) 患者的临床特征，所有这些患者都出现了脂肪营养不良 (LD)，根据脂肪减少分布的模式，可将其分为 3 种表型中的 I：全身型、部分型或局灶性。 LD 的发病通常较晚，平均在诊断 DM 后 4.6 年开始。钙质沉着、肌肉萎缩、关节挛缩和面部皮疹是被发现与 LD 相关的 DM 疾病特征。脂膜炎与局灶性脂肪萎缩相关，而抗 p155 自身抗体（一种新描述的肌炎相关自身抗体）与全身性 LD 更相关。特定的 LD 特征，如黑棘皮病、多毛症、脂肪重新分布和脂肪变性/非酒精性脂肪性脂肪炎，在 LD 患者中很常见，且 3 种亚表型之间的频率和严重程度存在梯度。代谢研究经常发现全身性和部分性 LD 患者存在胰岛素抵抗和高甘油三酯血症。大腿区域性脂肪减少（大腿内侧脂肪减少相对较少）比部分 LD 更常见，并且在没有 LD 的 DM 患者中不存在。细胞因子多态性、C3 肾炎因子、胰岛素受体抗体和核纤层蛋白突变似乎在我们患者的 LD 发展中没有发挥致病作用。 LD 是 JDM 的一种未被充分认识的后遗症，某些具有严重、长期临床病程和高钙质沉着频率的 DM 患者出现这种并发症的风险似乎更大。高危 JDM 患者应筛查代谢异常，代谢异常在全身性和部分 LD 中很常见，并导致大部分 LD 相关发病率。需要进一步研究 DM 患者获得性 LD 的发病机制。
We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into I of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. LD onset was often delayed, beginning a median of 4.6 years after diagnosis of DM. Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD. Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, a newly described myositis-associated autoantibody, was more associated with generalized LD. Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatoliepatitis were frequent in patients with LD, in a gradient of frequency and severity among the 3 sub-phenotypes. Metabolic studies frequently revealed insulin resistance and hypertriglyceridemia in patients with generalized and partial LD. Regional fat loss from the thighs, with relative sparing of fat loss from the medial thighs, was more frequent in generalized than in partial LD and absent from DM patients without LD. Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in our patients. LD is an under-recognized sequela of JDM, and certain DM patients with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development of this complication. High-risk JDM patients should be screened for metabolic abnormalities, which are common in generalized and partial LD and result in much of the LD-associated morbidity. Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM.