title: Kidney damage due to tuberous sclerosis complex: management recommendations.
abstract: OBJECTIVE
  To deduce recommendations from the literature on the management of kidney damage caused by tuberous sclerosis complex (TSC).
  
  
  METHODS
  Five practitioners have written up recommendations after reviewing the literature. They were evaluated by 14 experts using a 9 level scale (1: complete disagreement; 9: complete agreement), then reworded until each item received a median score of greater than or equal to 8.
  
  
  RESULTS
  Forty-eight to 80% of patients with TSC have kidney disease with the presence of angiomyolipomas (AML), cysts, cancers and/or progression towards renal insufficiency. An abdominal ultrasound (and serum creatinine level if there is an abnormality) is recommended as soon as the TSC is diagnosed. The evaluation should be repeated every 3 to 5 years if it is normal. Numerous and voluminous cysts are suggestive of associated polycystosis. After 20 years of age, the monitoring should be based on CT scan or MRI, which are more precise in the monitoring of AML. The biopsy of a renal mass should be discussed if there are calcifications, central necrosis or rapid growth. Lymphangioleiomyomatosis should be screened for in women via pulmonary CT scan at 18 and 30 to 40 years of age. Haemorrhagic rupture of an AML should be treated in first-line by embolisation. Asymptomatic AMLs that cumulate risk factors for bleeding (size >80 mm, predominant vascular contingent, micro-aneurisms) should be preventively treated, if possible by embolisation. The role of mTOR inhibitors remains to be defined.
  
  
  CONCLUSIONS
  Monitoring and a standardised treatment are necessary to improve the treatment of renal damage caused by TSC.
authors: Urinary and Vascular Imaging Department, Hospices Civils de Lyon, Hôpital Edouard Herriot, 5, place d'Arsonval, 69437 Lyon cedex 03, France. olivier.rouviere@netcourrier.com
authors: O
authors: O
authors: Rouvière
authors: H
authors: H
authors: Nivet
authors: N
authors: N
authors: Grenier
authors: L
authors: L
authors: Zini
authors: E
authors: E
authors: Lechevallier
mesh_terms: D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D014402:Tuberous Sclerosis / Q000150:complications*