Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the presence of proliferative lesions throughout the body. Management of TSC is challenging because patients have a multifaceted systemic illness with prominent neurological and developmental impact as well as potentially severe kidney, heart and lung phenotypes; however, every organ system can be involved. Adequate care for patients with TSC requires a coordinated effort involving a multidisciplinary team of clinicians and support staff. This clinical practice recommendation was developed by nephrologists, urologists, paediatric radiologists, interventional radiologists, geneticists, pathologists, and patient and family group representatives, with a focus on TSC-associated kidney manifestations. Careful monitoring of kidney function and assessment of kidney structural lesions by imaging enable early interventions that can preserve kidney function through targeted approaches. Here, we summarize the current evidence and present recommendations for the multidisciplinary management of kidney involvement in TSC.

结节性硬化症（TSC）是一种常染色体显性遗传疾病，其特征是全身存在增殖性病变。TSC 的治疗具有挑战性，因为患者患有多方面的系统性疾病，具有显着的神经和发育影响，以及潜在的严重肾脏、心脏和肺表型；然而，每个器官系统都可能参与其中。对 TSC 患者的充分护理需要临床医生和支持人员的多学科团队的协调努力。该临床实践建议由肾脏科医生、泌尿科医生、儿科放射科医生、介入放射科医生、遗传学家、病理学家以及患者和家庭团体代表制定，重点关注 TSC 相关的肾脏表现。通过成像仔细监测肾功能并评估肾脏结构病变，可以进行早期干预，通过有针对性的方法保护肾功能。在这里，我们总结了当前的证据，并提出了对 TSC 肾脏受累的多学科管理的建议。