Hypertension in adult Fabry's disease: is cardiotrophin-1 a diagnostic biomarker?

Monica Gioia Marazzi,Elena Vianello,Emanuela Galliera,Guido Tettamanti,Andrea Stella,Lorenza Tacchini,Massimiliano M Corsi Romanelli,Elena Dozio

doi:10.1186/s12979-014-0027-3

Abstract

BackgroundCardiotrophin-1 (CT-1), a cytokine produced by cardiomyocytes and non-cardiomyocytes in conditions of stress, can be used as a biomarker of left ventricular hypertrophy and dysfunction in hypertensive patients. Hypertension is one of the main adverse events in the third and last phase of Fabry’s disease (FD). We measured CT-1 in order to examine its correlation with the vascular and cardiac alterations at different ages and assess its potential for use as a biomarker of hypertension in FD.FindingsThe level of CT-1 was clearly higher in hypertensive adults than in adult FD patients. FD patients show a small, non-significant decrease in plasma CT-1 with age, while in hypertensive patients CT-1 in plasma rises strongly and highly significantly with age.ConclusionsCT-1 can be considered a good biomarker of the progression of hypertension with age, but particular care is needed when following hypertension in FD patients, since CT-1 does not correlate the same way with this disease.

Highlights

Cardiotrophin-1 (CT-1), a cytokine produced by cardiomyocytes and non-cardiomyocytes in conditions of stress, can be used as a biomarker of left ventricular hypertrophy and dysfunction in hypertensive patients
CT-1 can be considered a good biomarker of the progression of hypertension with age, but particular care is needed when following hypertension in Fabry’s disease (FD) patients, since CT-1 does not correlate the same way with this disease
CT-1 was measured in FD and hypertensive patients matched by age (Figure 1)

Summary

Introduction

Cardiotrophin-1 (CT-1) is a member of the interleukin-6 superfamily and activates gp130-dependent signaling, stimulating the (JACK/STAT) pathway and cardiac hypertrophic myocytes [1]. CT-1 activates different pathways in cardiac hypertrophic myocytes, leading to myocardial fibrosis, and contributing to the pathogenesis of hypertensive heart disease [2]. A recent study indicated that circulating CT-1 correlates with cardiac hypertrophy and vascular damage in hypertensive patients so it could serve as a biomarker of left ventricular hypertrophy and dysfunction in these cases [2,3]. FD nephropathy progresses with the severity of the disease, eventually resulting in chronic kidney disease, leading to hypertension. The findings do indicate that CT-1 could be a good biomarker to monitor the progression of hypertension with age, but particular care is needed in FD patients because its levels do not correlate the same way with this disease

Patients and methods

Results

Discussion