Serum angiotensin-converting enzyme activity in lymphangioleiomyomatosis

Introduction: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease, caused by the proliferation of LAM cells leading to destruction and remodeling of the tissues. Renin–angiotensin system and mast cells have been reported to function on cell proliferation and tissue remodeling in LAM (AJRCMB 2006, AJRCCM 2002, Respir Res 2012). Aim of this study is to clarify the role of measurement of serum angiotensin-converting enzyme (ACE) activity as a biomarker for LAM. Subjects and methods: 102 patients of LAM including 15 tuberlous sclerosis complex (TSC)-LAM, 37 healthy volunteers (HV) and 16 other lung disease controls (OLD) were enrolled. Serum ACE activities were measured by Kasahara9s method. Serum VEGF-D levels and clinical measures were compared with serum ACE. Results: Serum ACE significantly elevated in LAM {14.6 (11.7-19.1) median (IQR) IU/L } compared in HV {10.5 (7.6-12.7) IU/L } or OLD {9.9 (7.4-11.6) IU/L } (p Discussion and conclusion: Serum ACE significantly increased in LAM, which was associated with lymphangioleiomyomas and serum VEGF-D, suggesting lymphatic abnormalities. Serum ACE may have accessory role in LAM which differ from VEGF-D. This study was partially supported by the grant from JMHLW (YI).