Background Scleroderma Spectrum Diseases (SSDs) are characterized by alterations of the microarchitecture of small arteries. Recently, gadolinium enhanced MRA has been used as a safe and accurate technique for the evaluation of vascular pathologies of the small arteries of the hands1.
Objectives the aims of the present study were to: 1. evaluate the feasibility of unenhanced MRA in healthy controls (HC) and in SSDs. 2. outline differences between HC and SSDs 3. assess the capability of unenhanced MRA to measure the severity of vascular damage in SSDs.
Methods MRA was performed both in HS and in consecutive SSDs patients: both hands were assessed with a 3 tesla system (Discovery MR750, GE) using 3D delta flow MRA. All of the patients performed a standardized clinical-instrumental assessment to evaluate organ involvement as well as capillaroscopy, with classification of the pattern according to Cutolo et al.
Results 10 HC and 7 consecutive SSDs were enrolled in the period from September to December 2011. 3 patients were classified, according to ACR criteria, as limited cutaneous SSc (Lc-SSc), 2 as diffuse cutaneous SSc (Dc-Ssc), 1 patient fulfilled very early diagnosis of systemic sclerosis (VEDOSS) criteria and 1 was classified as mixed connective tissue disease (MCTD). All of the patients were female with a mean age at the evaluation of 46 years [30-71], mean disease duration 5.9 years [1-9]. Five patients had a history of digital ulcers (DUs), 2 had active DUs, all had esophageal involvement. Five patients were affected by interstitial lung disease and no case of pulmonary arterial hypertension was recorded. Table 1 summarizes the clinical data of the patients. Whereas in HC proper digital arteries (PDAs) were depictet in 100% of the cases (fully depicted in 7/10, partially depicted in 3/10), in SSDs these vessels were depicted in only 57% of the cases (fully: 3 partially: 1).
In SSDs patients, abnormalities of MRA findings were correlated to Allen test alteration, moreover, 3/5 of the patients with a history of DUs had major alteration of proper digital arteries.
Conclusions vascular involvement is important in the pathogenesis of digital skin ulcers in SSDs. Unhenanced MRA seems to be feasible and able to improve the assessment of vascular involvement in these disorders. However, the unenhanced MRA technique needs to be validated on larger number of patients in order to establish if it can measure the severity of vascular involvement and predict the development of digital ulcers and if it could replace contrast-enhanced MRA.
Allanore Y, Seror R et al. Hand vascular involvement assessed by magnetic resonance angiography in Systemic Sclerosis. Arthritis & Rheumatism 2007; 56(8):2747-54
Disclosure of Interest None Declared