Open Access Research article

Prognostic impact of coronary microcirculation abnormalities in systemic sclerosis: a prospective study to evaluate the role of non-invasive tests

Alessandra Vacca1*, Roberta Montisci2, Pietro Garau1, Paolo Siotto3, Matteo Piga1, Alberto Cauli1, Massimo Ruscazio2, Luigi Meloni2, Sabino Iliceto4 and Alessandro Mathieu1

Author affiliations

1 University and A.O.U. of Cagliari, Chair and Unit of Rheumatology, S.S. 554 bivio per Sestu, Monserrato 09042, Italy

2 University and A.O.U. of Cagliari, Chair and Unit of Cardiovascular Diseases, Via Ospedale, Cagliari 09100, Italy

3 Azienda Ospedaliera Brotzu, Radiology Service, Piazzale Ricchi 1, Cagliari 09121, Italy

4 University and Azienda Ospedaliera of Padova, via Giustiniani 2, Padova 35128, Italy

For all author emails, please log on.

Citation and License

Arthritis Research & Therapy 2013, 15:R8  doi:10.1186/ar4136

Published: 9 January 2013

Abstract

Introduction

Microcirculation dysfunction is a typical feature of systemic sclerosis (SSc) and represents the earliest abnormality of primary myocardial involvement. We assessed coronary microcirculation status by combining two functional tests in SSc patients and estimating its impact on disease outcome.

Methods

Forty-one SSc patients, asymptomatic for coronary artery disease, were tested for coronary flow velocity reserve (CFR) by transthoracic-echo-Doppler with adenosine infusion (A-TTE) and for left ventricular wall motion abnormalities (WMA) by dobutamine stress echocardiography (DSE). Myocardial multi-detector computed tomography (MDCT) enabled the presence of epicardial stenosis, which could interfere with the accuracy of the tests, to be excluded. Patient survival rate was assessed over a 6.7- ± 3.5-year follow-up.

Results

Nineteen out of 41 (46%) SSc patients had a reduced CFR (≤2.5) and in 16/41 (39%) a WMA was observed during DSE. Furthermore, 13/41 (32%) patients showed pathological CFR and WMA. An inverse correlation between wall motion score index (WMSI) during DSE and CFR value (r = -0.57, P <0.0001) was observed; in addition, CFR was significantly reduced (2.21 ± 0.38) in patients with WMA as compared to those without (2.94 ± 0.60) (P <0.0001). In 12 patients with abnormal DSE, MDCT was used to exclude macrovasculopathy. During a 6.7- ± 3.5-year follow-up seven patients with abnormal coronary functional tests died of disease-related causes, compared to only one patient with normal tests.

Conclusions

A-TTE and DSE tests are useful tools to detect non-invasively pre-clinical microcirculation abnormalities in SSc patients; moreover, abnormal CFR and WMA might be related to a worse disease outcome suggesting a prognostic value of these tests, similar to other myocardial diseases.