Background: Sirolimus 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.
Objective: The aim of this study was to analyze the clinical features, lymphoscintigraphy and lymphedema outcome in patients treated with sirolimus.
Patients and methods: Monocentric retrospective study from January 2008 to September 2017 analyzing all consecutive patients having lymphedema occurring with sirolimus.
Results: Fifteen 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.
Conclusion: Sirolimus 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.
Keywords: Inhibiteur de mTOR; Lymphedema; Lymphœdème; MTOR inhibitor; Sirolimus.
Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.