Lymphœdèmes associés à la prise de sirolimus : à propos de 15 patients

Abstract

BackgroundSirolimus is a mammalian target of rapamycin (mTOR) inhibitor used after organ transplantation and to treat vascular malformations. Among its adverse effects, limb lymphedema has been described. ObjectiveThe aim of this study was to analyze the clinical features, lymphoscintigraphy and lymphedema outcome in patients treated with sirolimus. Patients and methodsMonocentric retrospective study from January 2008 to September 2017 analyzing all consecutive patients having lymphedema occurring with sirolimus. ResultsFifteen patients (7 men, 8 women), mean age at the first visit, 56 years (range: 38–76), had a kidney transplant (n=12), liver transplant (n=1), or lymphangioleiomyomatosis (n=2) treated with sirolimus at a mean daily dose of 1.8mg were included. Lymphedema involved one (n=4), or both (n=1) lower limbs, upper limb (n=9), lower limbs and upper limb (n=1). Lymphedema affected the whole limb (n=10), or the distal part (n=5). The median time between lymphedema onset and the beginning of sirolimus was 52 weeks (range: 8–232). Lymphoscintigraphy in 7 patients (lower limb: 3, superior: 4) showed no inguinal or axillary nodal fixation (n=6) or decreased uptake (n=1). Sirolimus was discontinued in 7 cases without lymphedema improvement with a median follow-up of 12 months and maintained in 8 cases. ConclusionSirolimus is associated with upper and/or lower limb lymphedema, without predominance of sex, and without disappearance after sirolimus discontinuation. Pathophysiological mechanisms remain unclear. Lymphedema management is based on low-stretch bandages and compression.