更新的S2k指南涉及局限性硬皮病(LoS)的诊断和治疗。LoS代表了一系列硬化性皮肤病,根据子类型和本地化,脂肪组织等结构,肌肉,接头,骨骼也可能受到影响。不会发生内脏器官受累或进展为系统性硬化症。LoS可以分为四种主要形式:有限,广义的,线性,和混合形式,有一些额外的亚型。对于皮肤受累有限的病例,该指南主要推荐外用糖皮质激素治疗.也可以推荐UV疗法。在严重皮肤或肌肉骨骼受累的亚型中,建议使用甲氨蝶呤进行全身治疗.在疾病的活跃阶段,全身性糖皮质激素可以额外使用。在甲氨蝶呤和类固醇难治性疗程的情况下,禁忌症,或不宽容,霉酚酸酯,霉酚酸,或abatacept可被视为二线全身治疗。在线性LoS的情况下,自体脂肪干细胞移植也可用于修复软组织缺损.
The updated S2k
guideline deals with the diagnosis and therapy of localized scleroderma (LoS). LoS represents a spectrum of sclerotic skin diseases in which, depending on the subtype and localisation, structures such as adipose tissue, muscles, joints, and bones may also be affected. Involvement of internal organs or progression to systemic sclerosis does not occur. LoS can be classified into four main forms: limited, generalized, linear, and mixed forms, with some additional subtypes. For cases of limited skin involvement, the
guideline primarily recommends therapy with topical corticosteroids. UV therapy can also be recommended. In subtypes with severe skin or musculoskeletal involvement, systemic therapy with methotrexate is recommended. During the active phase of the disease, systemic glucocorticosteroids can be used additionally. In cases of methotrexate and steroid refractory courses, contraindications, or intolerance, mycophenolate mofetil, mycophenolic acid, or abatacept can be considered as second-line systemic therapies. In the case of linear LoS, autologous adipose-derived stem cell transplantation can also be performed for correcting soft tissue defects.