Elsevier

Microvascular Research

Volume 82, Issue 3, November 2011, Pages 410-415
Microvascular Research

In systemic sclerosis macrovascular damage of hands digital arteries correlates with microvascular damage

https://doi.org/10.1016/j.mvr.2011.07.009Get rights and content

Abstract

Objective

To assess morphology and blood flow of the proper palmar digital arteries (PPDA) by Color Doppler Ultrasonography (CDUS) and its relationship with nailfold videocapillaroscopy (NVC), skin blood perfusion and digital arteries pulsatility of hands in SSc patients and healthy controls.

Methods

CDUS, NVC, Laser Doppler Perfusion Imaging (LDPI) and photoplethysmography (PPG) were performed in 36 systemic sclerosis (SSc) patients and 20 healthy controls.

Results

CDUS was pathologic in 69% of patients with SSc and in none of healthy controls (p < 0.0001). SSc patients with low vascular damage (early capillaroscopic pattern) have a normal morphology of PPDA, but the blood flow, evaluated by peak systolic velocity (PSV) and end diastolic velocity (EDV), is reduced and vascular resistance, measured by resistive index (RI) and pulsatility index (PI), increased. At this stage the LDPI mean perfusion and digital artery pulsatility, evaluated by PPG, were reduced. The US changes appear with microvasculare damage progression (active and late capillaroscopic patterns), while the PPDA blood flow progressively decreases (PSV and EDV decreased, RI and PI increased). The macrovascular damage correlates with disease duration. Anti-topoisomerase I represents an independent predictive factor for macrovascular damage. We not observed any association between digital ulcer history, pulmonary fibrosis and US findings.

Conclusion

PPDA blood flow dysfunction is already present in early disease. Structural macrovascular damage progresses with worsening of SSc microangiopathy.

Highlights

► Distal peripheral artery disease is present in the digits of systemic sclerosis patients. ► Ultrasonography damage of proper palmar digital arteries appears in late stages of microangiopathy. ► Blood flow dysfunction of digital arteries is already present in early disease. ► Macrovascular damage progresses with worsening of systemic sclerosis microangiopathy.

Introduction

Systemic sclerosis (SSc) is a chronic connective tissue disease characterized by endothelial dysfunction and fibrosis of the skin and internal organs. Vascular dysfunction is one of the hallmarks of SSc and involves both the macro and microvasculature (Campbell and LeRoy, 1975).

In SSc patients ultrasonography (US) of digital arteries is characterized by smaller artery lumen, reduced pulsation, and thickened, slightly hyperechoic artery walls, increased resistive index (RI), low vessel compliance (Bregenzer et al., 2004, Schmidt et al., 2006). Color Doppler Ultrasonography (CDUS) of finger arteries is useful to differentiate between primary and secondary Raynaud's phenomenon (RP). It depicts the same anatomical structures as angiography, but it is cheaper, faster, and noninvasive (Keberle et al., 2000, Schmidt et al., 2008, Singh et al., 1991).

Nailfold videocapillaroscopy (NVC) is the most reliable way to distinguish between primary and secondary RP through identification of an early pattern of SSc (Cutolo et al., 2003, Maricq and LeRoy, 1973). Laser Doppler Perfusion Imaging (LDPI) is not an invasive technique on evaluating skin blood perfusion in patients with primary RP and SSc (Cutolo et al., 2010, Grattagliano et al., 2010, Rosato et al., 2009, Rosato et al., 2010a). Photoplethysmography (PPG) application in RP is restricted to evaluate the response of digital arteries to cold test and drugs. PPG improves the evaluation of vascular damage in patients with PRP and SSc (Rosato et al., 2010b).

Our aim was to assess in SSc patients macrovascular damage of hand digital arteries by CDUS and its relationship with microvascular damage, evaluated by NVC, LDPI and PPG.

Section snippets

Subjects

Thirty six patients (31 female and 5 male; mean age 46.1 ± 11.9 years) fulfilling the American College of Rheumatology preliminary criteria for the classification of SSc were enrolled in this study (ARA, 1980). SSc patients were divided into limited (lcSSc) and diffuse (dcSSc) cutaneous SSc groups (15). Disease activity and disease severity were measured by Valentini Disease Activity Index (DAI) and Medsger Disease Severity Scale (DSS), respectively (Medseger et al., 2003, Valentini et al., 2003).

CDUS

No significant differences of IMT were observed between SSc patients and healthy controls (0.61 ± 0.03 mm vs 0.58 ± 0.06 mm). CDUS of healthy controls showed normal US morphology of PPDA and normal blood flow with homogeneous pattern of waveform analysis. US pathologic findings of PPDA occurred in 69% of patients with SSc: 11 patients (31%) have an US type A pattern, 13 (36%) have an US type B pattern, 12 (33%) an US type C pattern and no one has a type D pattern (Fig. 1). In all ten fingers of each

Discussion

Microvascular dysfunction is already present in early disease. Microvascular abnormalities are well-known as major sites of pathology, but less attention has been paid to macrovascular abnormalities (Rollando et al., 2010).

In SSc macrovascular disease was considered extremely rare and the prevalence of vascular abnormalities has been considered to be inversely proportional to the size of blood vessels studied (Hettema et al., 2008). An increased prevalence of peripheral vascular disease in SSc

Conclusion

PPDA blood flow dysfunction is already present in early disease. Structural macrovascular damage progresses with worsening of SSc microangiopathy. Therefore, our findings should be confirmed in a multicenter study with a larger cohort of patients.

Author contribution

Edoardo Rosato: designed the research, performed the research, analyzed data, wrote the paper. Antonietta Gigante: designed the research, performed the research, analyzed data, wrote the paper; Biagio Barbano: performed the research, analyzed data, wrote the paper; Rosario Cianci: performed research; Ilenia Molinaro: analyzed data, wrote the paper; Simonetta Pisarri: performed the research; Felice Salsano: designed the research, wrote the paper.

Conflict of interest statement

The authors declare no conflicts of interest.

Submission declaration

The authors declare that the work described has not been published previously, that it is not under consideration for publication elsewhere, that its publication is approved by all authors.

    Abbreviations

    SSc

    systemic sclerosis

    US

    ultrasonography

    RI

    resistive index

    CDUS

    Color Doppler Ultrasonography

    RP

    Raynaud's phenomenon

    NVC

    nailfold videocapillaroscopy

    LDPI

    Laser Doppler Perfusion Imaging

    PPG

    photoplethysmography

    IMT

    intima-media thickness

    PIP

    proximal interphalangeal joints

    DIP

    distal interphalangeal joints

    PI

    pulsatility index

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