Abstract：
BACKGROUND: Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known.
METHODS: We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected.
RESULTS: Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control.
CONCLUSIONS: Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
METHODS: We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected.
RESULTS: Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control.
CONCLUSIONS: Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
摘要：
背景：在特发性炎性肌病（IIM）中涉及皮下组织的情况鲜为人知。
方法：我们通过PubMed/Medline对青少年和成人IIM的脂膜炎和脂肪营养不良/脂肪萎缩的文献进行了系统回顾，Embase和Scopus数据库。这项审查包括三项当地观察。流行病学,临床,收集临床和治疗数据。
结果：脂膜炎似乎在成人中比在青少年中更常见。它主要位于上肢和下肢。在大多数类固醇和脂膜炎的病例中，脂膜炎和肌炎的病程相似，在青少年和成年人中，有83.3％和72.2％的病例，分别。脂肪营养不良在青少年中似乎更为频繁，仅在青少年和成年人的皮肌炎中观察到。脂肪营养不良主要在青少年和成年人中部分发生。青少年和成年人从肌炎到诊断脂肪营养不良的中位时间为6年[0-35]和2.5年[0-10]，分别。脂肪营养不良与抗TIF1γ自身抗体阳性相关，肌炎的多环/慢性过程和钙质沉着的发生，可能是疾病控制不良的指标。
结论：脂肪组织受累，特别是脂肪营养不良，几乎只发生在皮肌炎。阴险的发作和对诊断的认识不足可能会低估其患病率。需要更大规模的研究来确定这些患者可能的危险因素，为了更好的潜在潜在病理生理过程，以讨论潜在的治疗靶点。
方法：我们通过PubMed/Medline对青少年和成人IIM的脂膜炎和脂肪营养不良/脂肪萎缩的文献进行了系统回顾，Embase和Scopus数据库。这项审查包括三项当地观察。流行病学,临床,收集临床和治疗数据。
结果：脂膜炎似乎在成人中比在青少年中更常见。它主要位于上肢和下肢。在大多数类固醇和脂膜炎的病例中，脂膜炎和肌炎的病程相似，在青少年和成年人中，有83.3％和72.2％的病例，分别。脂肪营养不良在青少年中似乎更为频繁，仅在青少年和成年人的皮肌炎中观察到。脂肪营养不良主要在青少年和成年人中部分发生。青少年和成年人从肌炎到诊断脂肪营养不良的中位时间为6年[0-35]和2.5年[0-10]，分别。脂肪营养不良与抗TIF1γ自身抗体阳性相关，肌炎的多环/慢性过程和钙质沉着的发生，可能是疾病控制不良的指标。
结论：脂肪组织受累，特别是脂肪营养不良，几乎只发生在皮肌炎。阴险的发作和对诊断的认识不足可能会低估其患病率。需要更大规模的研究来确定这些患者可能的危险因素，为了更好的潜在潜在病理生理过程，以讨论潜在的治疗靶点。
