Suppr超能文献

下肢脂肪性水肿、上肢脂肪营养不良及严重钙质沉着症并发幼年皮肌炎。

Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis.

作者信息

Pavlov-Dolijanovic Slavica R, Vujasinovic Stupar Nada Z, Gavrilov Nikola, Seric Srdjan

机构信息

Institute of Rheumatology Belgrade, Resavska 69, Belgrade, Serbia,

出版信息

Rheumatol Int. 2014 Nov;34(11):1615-8. doi: 10.1007/s00296-014-3031-1. Epub 2014 May 1.

Abstract

Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.

摘要

幼年皮肌炎(JDM)是一种罕见但复杂且可能危及生命的儿童自身免疫性疾病。相当一部分患者存在残留肌无力、肌肉萎缩、关节挛缩和钙质沉着。最近,有报道出现了一些新的临床发现,如伴有某些部位脂肪沉积增加的脂肪营养不良。到目前为止,尚不清楚身体脂肪重新分布是否可能是下肢脂肪性水肿的类型。我们描述了一名39岁女性,她7岁时被诊断为JDM。后来她出现了上肢对称性脂肪营养不良和下肢对称性脂肪性水肿(分别占全身脂肪量的2%和58.3%),皮下组织有多个钙化结节。尽管进行了治疗,这些结节的大小仍逐渐增大。毛细血管镜检查结果显示硬皮病样异常。抗核抗体(ANA)和抗U1RNP抗体呈阳性。迄今为止,尚未发表过同时出现下肢脂肪性水肿、上肢脂肪营养不良以及严重钙质沉着并使JDM复杂化的类似病例。我们发现钙质沉着和脂肪营养不良与活动性疾病的病程较短有关。此外,我们展示的病例提出了一个问题,即在随访期间是否可能存在重叠的自身免疫性疾病。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验