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Introduction:
Vascular involvement is a cardinal feature of systemic sclerosis with early and diffuse microcirculation impairment. The available methods to assess microcirculation are limited; the main techniques are capillaroscopy for digital capillary assessment and color-Doppler imaging which is under development. However, magnetic resonance angiography (MRA) has strikingly improved the assessment of vascular lesions such as for vascular malformations of the hand. We thus aimed to evaluate the vascular lesion of the hand in patients with SSc using MRA.
Patients and Methods:
23 successive patients were evaluated with MRA for right hand involvement after disruption since 3 days of usual treatments; mean±SD age was 51±13 years, mean±SD disease duration was 7±9 years (14 with less than 5 years), 14 had a diffuse cutaneous form, 7 had digital ulcers, 5 had ever been treated by intra-venous prostacyclin therapy, 10 had abnormal lung carbon monoxide diffusion capacity. MRA consisted in 4 successive acquisitions of 50 sec each with 3 dimension coronal cross-sectional images after gadolinium injection. The primary pre-defined criteria were: quality of arterial opacification and venous flux (from proper digital arteries of finger 2 to 5), presence of tissular damning up, avascular areas. Secondary criteria were: synovitis, tenosynovitis, erosions. MRA analyses were performed by 2 independent readers with a consensus made by a third investigator.
Results:
19 (83 %) of the patients had 1 proper digital artery which did not reach the first phalanx at the initial arterial analysis and 14 (61 %) had 4 or more proper digital artery with such disturbance. All the patients had abnormal venous flux which was considered as very altered in 14 cases (61 %). A global venous damning up was found in 7 patients (30 %). Seventeen (74 %) patients had thin arteries and 10 (43 %) had more than 1 avascular areas. Eleven (48 %) patients had at least one synovitis, 2/23 at least one erosion and 3/23 at least one tenosynovitis. Patients with thin arteries had a longer disease duration than patients without thin arteries (p = 0.006), and this was also the case for patients with more than 4 disturbed proper digital arteries (p = 0.04). Altered venous reflux was associated with high value of plasma von Willebrand factor concentrations (p = 0.015).
Conclusion:
All the patients had at least one abnormality on MRA; lesions are generally diffuse and involve both arterial and venous systems. No difference was established among the 2 cutaneous sub-types but lesions seem to increase with disease duration; however, some lesions may be found as early as the initial months after diagnosis. A comparison with patients having primary Raynaud’s syndrome is ongoing but MRA seems a very interesting tool to evaluate the vascular lesions of SSc and potentially the effects of vascular drugs.
Vascular involvement is a cardinal feature of systemic sclerosis with early and diffuse microcirculation impairment. The available methods to assess microcirculation are limited; the main techniques are capillaroscopy for digital capillary assessment and color-Doppler imaging which is under development. However, magnetic resonance angiography (MRA) has strikingly improved the assessment of vascular lesions such as for vascular malformations of the hand. We thus aimed to evaluate the vascular lesion of the hand in patients with SSc using MRA.
Patients and Methods:
23 successive patients were evaluated with MRA for right hand involvement after disruption since 3 days of usual treatments; mean±SD age was 51±13 years, mean±SD disease duration was 7±9 years (14 with less than 5 years), 14 had a diffuse cutaneous form, 7 had digital ulcers, 5 had ever been treated by intra-venous prostacyclin therapy, 10 had abnormal lung carbon monoxide diffusion capacity. MRA consisted in 4 successive acquisitions of 50 sec each with 3 dimension coronal cross-sectional images after gadolinium injection. The primary pre-defined criteria were: quality of arterial opacification and venous flux (from proper digital arteries of finger 2 to 5), presence of tissular damning up, avascular areas. Secondary criteria were: synovitis, tenosynovitis, erosions. MRA analyses were performed by 2 independent readers with a consensus made by a third investigator.
Results:
19 (83 %) of the patients had 1 proper digital artery which did not reach the first phalanx at the initial arterial analysis and 14 (61 %) had 4 or more proper digital artery with such disturbance. All the patients had abnormal venous flux which was considered as very altered in 14 cases (61 %). A global venous damning up was found in 7 patients (30 %). Seventeen (74 %) patients had thin arteries and 10 (43 %) had more than 1 avascular areas. Eleven (48 %) patients had at least one synovitis, 2/23 at least one erosion and 3/23 at least one tenosynovitis. Patients with thin arteries had a longer disease duration than patients without thin arteries (p = 0.006), and this was also the case for patients with more than 4 disturbed proper digital arteries (p = 0.04). Altered venous reflux was associated with high value of plasma von Willebrand factor concentrations (p = 0.015).
Conclusion:
All the patients had at least one abnormality on MRA; lesions are generally diffuse and involve both arterial and venous systems. No difference was established among the 2 cutaneous sub-types but lesions seem to increase with disease duration; however, some lesions may be found as early as the initial months after diagnosis. A comparison with patients having primary Raynaud’s syndrome is ongoing but MRA seems a very interesting tool to evaluate the vascular lesions of SSc and potentially the effects of vascular drugs.
Y. Allanore, None.