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肉芽肿性多血管炎的心脏受累:对31例连续患者的磁共振成像研究

Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients.

作者信息

Pugnet Grégory, Gouya Hervé, Puéchal Xavier, Terrier Benjamin, Kahan André, Legmann Paul, Guillevin Loïc, Vignaux Olivier

机构信息

Service de Médecine Interne, Centre National de Référence Vascularites Nécrosantes et Sclérodermie Systémique, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.

Service de Médecine Interne, Hôpital Purpan, Toulouse.

出版信息

Rheumatology (Oxford). 2017 Jun 1;56(6):947-956. doi: 10.1093/rheumatology/kew490.

Abstract

OBJECTIVES

Specific cardiac involvement in granulomatosis with polyangiitis (GPA) is probably underestimated since many of these conditions are subclinical. The objective of this study was to assess the prevalence and patterns of cardiac abnormalities detected by cardiac MRI (CMRI) in patients with GPA.

METHODS

Thirty-one consecutive patients with newly diagnosed or relapsing GPA underwent CMRI to assess morphological, functional, perfusion at rest and delayed enhancement abnormalities.

RESULTS

At least one abnormality was observed on CMRI for 19 of 31 patients (61%). Four patients (13%) had an impaired left ventricle ejection fraction (LVEF). LV regional wall motion abnormalities were found in 11 patients (35%). Late gadolinium enhancement (LGE) was detected in 10 of 31 patients (32%). LGE was mostly nodular ( n = 9). Myocardial early contrast enhancement was detected in 5 of the 31 patients (16%), which was systematically associated with LGE in the same territory. CMRI detected pericarditis in eight patients (26%). GPA with <18 months duration was associated with a higher LVEF ( P = 0.03), fewer CMRI abnormalities ( P = 0.04) and less LV hypokinesia ( P = 0.04) than GPA with a longer duration. Patients with recent-onset GPA had a higher LVEF ( P = 0.01) and less LV hypokinesia ( P = 0.006) than patients experiencing a relapse ( P = 0.02).

CONCLUSION

CMR is an accurate technique for detecting heart involvement in GPA. This unique non-invasive technique may provide information with important clinical implications for the accurate early assessment of cardiac lesions in GPA patients and for detecting cumulative, irreversible damage. It may also have prognostic implications.

摘要

目的

肉芽肿性多血管炎(GPA)患者的特异性心脏受累情况可能被低估,因为其中许多情况是亚临床的。本研究的目的是评估心脏磁共振成像(CMRI)检测GPA患者心脏异常的患病率和模式。

方法

31例新诊断或复发的GPA患者连续接受CMRI检查,以评估形态学、功能、静息灌注和延迟强化异常情况。

结果

31例患者中有19例(61%)在CMRI上观察到至少一项异常。4例患者(13%)左心室射血分数(LVEF)受损。11例患者(35%)发现左心室局部壁运动异常。31例患者中有10例(32%)检测到延迟钆增强(LGE)。LGE大多为结节状(n = 9)。31例患者中有5例(16%)检测到心肌早期对比增强,且在同一区域与LGE有系统性关联。CMRI检测到8例患者(26%)有心肌炎。病程<18个月的GPA患者比病程较长的GPA患者LVEF更高(P = 0.03),CMRI异常更少(P = 0.04),左心室运动减弱更少(P = 0.04)。与复发患者相比,近期发病的GPA患者LVEF更高(P = 0.01),左心室运动减弱更少(P = 0.006)(P = 0.02)。

结论

CMR是检测GPA患者心脏受累的准确技术。这种独特的非侵入性技术可为GPA患者心脏病变的准确早期评估以及检测累积性、不可逆损伤提供具有重要临床意义的信息。它也可能具有预后意义。

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