Department of Dermatology, Institute of Medical Sciences, Medical College of Rzeszow University, Rzeszów, Poland.
Biomed Res Int. 2021 Nov 19;2021:5513544. doi: 10.1155/2021/5513544. eCollection 2021.
Juvenile dermatomyositis is a chronic and rare autoimmune disorder classified into the spectrum of idiopathic inflammatory myopathies. Although this entity is mainly characterized by the presence of pathognomonic cutaneous lesions and proximal muscle weakness, the clinical manifestation can be highly heterogeneous; thus, diagnosis might be challenging. Current treatment recommendations for juvenile dermatomyositis, based mainly upon case series, include the use of corticosteroids, immunomodulatory, and immunosuppressive agents. Recently, several specific autoantibodies have been shown to be associated with distinct clinical phenotypes of classic dermatomyositis. There is a need to further evaluate their relevance in the formation of various clinical features. Furthermore, while providing more personalized treatment strategies, one should consider diversity of autoantibody-related subgroups of juvenile dermatomyositis.
幼年特发性皮肌炎是一种慢性且罕见的自身免疫性疾病，属于特发性炎性肌病谱。虽然该疾病的主要特征为存在特征性皮肤损伤和近端肌无力，但临床表现可能高度异质，因此诊断可能具有挑战性。目前基于病例系列的幼年特发性皮肌炎治疗建议包括使用皮质类固醇、免疫调节剂和免疫抑制剂。最近，几项特定的自身抗体已被证明与经典皮肌炎的不同临床表型相关。需要进一步评估它们在形成各种临床特征中的相关性。此外，在提供更个体化的治疗策略时，应考虑幼年特发性皮肌炎的自身抗体相关亚组的多样性。
