Tuberous sclerosis complex (TSC) is an autosomal dominant neuroectodermic disorder that can affect brain, kidneys, heart, lungs and skin.
Because the pathogenesis of the disorder results in protein kinase mTOR (mammalian target of rapamycin) hyperactivity, mTOR inhibitors such as Everolimus have an important therapeutic role.
We report a case of a patient (female, age 37) diagnosed with TSC who underwent kidney transplantation in 2001.
Since transplantation, the patient underwent anti-rejection therapy consisting of Everolimus (2 mg 2 times a day) in combination with Cyclosporin microemulsion and steroids. Changes in the volume of renal angiomyolipomas in patients treated with Sirolimus and Everolimus were reported in the literature in the meantime.
Over 11 years, the Everolimus dosage was reduced. Since 2008 the patient takes 1 mg Everolimus 2 times a day (blood levels between 5 and 9 ng / ml), which is much lower than the 10 mg per day proposed by specific studies on TSC.
We observed a significant regression of renal angiomyolipoma in the native kidney after treatment with low dosage of Everolimus.
