狼疮性肾炎的循证治疗:现状与挑战。

Frontiers in nephrology Pub Date : 2024-08-06 eCollection Date: 2024-01-01 DOI:10.3389/fneph.2024.1417026
Efstathios Xagas, Konstantinos Drouzas, George Liapis, Sophia Lionaki
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摘要

系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,因其个体间的高度异质性而闻名,会影响包括肾脏在内的多个器官。狼疮性肾炎(LN)是该病的一种常见表现,可危及生命,多达50%的患者会出现肾脏受累。狼疮肾脏受累的分类基于特定的组织病理学发现,为治疗决策提供指导。免疫抑制疗法,特别是糖皮质激素联合环磷酰胺或霉酚酸酯,多年来一直是治疗的主要手段,而完全缓解率却没有显著变化。尽管治疗取得了进展,但仍有相当一部分 LN 患者会导致终末期肾病(ESKD)。包括贝利木单抗、voclosporin 和 obinutuzumab 在内的新兴疗法在改善肾脏预后方面取得了可喜的成果,尤其是在难治性或复发性疾病中。维持治疗对于预防疾病复发和保护肾功能至关重要。如今,包括改变生活方式和非免疫抑制性药物干预在内的支持性措施对于治疗 LN 也至关重要。这篇综述强调了治疗的最新进展,以及在优化治疗和改善长期疗效方面所面临的挑战。
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Evidence based treatment for lupus nephritis: present perspectives and challenges.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease known for its high heterogeneity among individuals, which affects various organs including the kidneys. Lupus nephritis (LN) is a frequent and life-threatening manifestation of the disease, with up to 50% of patients developing kidney involvement. Classification of renal involvement in lupus is based on specific histopathological findings, guiding therapeutical decisions. Immunosuppressive therapy, particularly glucocorticoids combined with cyclophosphamide or mycophenolate mofetil, has been the mainstay of treatment for many years, while rates of complete remission have not changed dramatically. Despite advancements in therapy, in an important proportion of patients LN leads to end-stage kidney disease (ESKD). Emerging therapies including belimumab, voclosporin, and obinutuzumab offer promising results in improving renal outcomes, especially in refractory or relapsing disease. Maintenance therapy is crucial to prevent disease flares and preserve renal function. Supportive measures including lifestyle modifications and non-immunosuppressive pharmacological interventions are nowadays also essential in managing LN. This review emphasizes recent advances of therapy and challenges regarding treatment optimization with strategies to improve long-term outcomes.

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