Arterial Imaging in Digital Gangrenes Associated with Scleroderma-Spectrum Disorders

Abstract

Objectives: Digital refractory gangrene is rarely found in collagen diseases, including systemic sclerosis and is possibly caused by similar underlying vascular damage in peripheral arterial disease (PAD) such as arteriosclerosis obliterans (ASO) and/or thromboangiitis obliterans (TAO) by unclarified mechanisms other than vasculitis and thrombosis. This study evaluated the radiological imaging in patients with digital gangrene associated with collagen disease and compared the images with those of PAD based on the results of laboratory and histopathological examinations. Methods: Angiography, MR angiography and/or CT angiography were performed on 6 patients with refractory gangrene or extensive ulcers accompanied by scleroderma-spectrum disorders; 3 with diffuse systemic sclerosis, 1 with limited systemic sclerosis, 1 with overlap syndrome and 1 with Sjögren's syndrome. Results: Although the vascular alterations in collagen diseases were similar to those in PAD, the abnormal image findings (occlusion or stenosis of the arteries with smooth vessel walls) found in collagen diseases did not include atheromatous plaque, which are worm-like vessels that are characteristic of those observed in PAD. Conclusions: Some cases of digital gangrene seen in collagen diseases show similar vascular imaging patterns to those of PAD and comprehensive examinations including arterial imaging can be useful for the diagnosis of these unrecognized vascular changes other than vasculitis or digital thrombosis.

Vasculopathie artérielle dans la sclérodermie systémique. Caractéristiques micro- et macrovasculaire de l’angioscanner des artères des membres supérieurs

“…Bien que les ulcérations digitales surviennent fréquemment chez les patients ayant une ScS, peu d'observations de l'atteinte macrovasculaire ont été rapportées. On a signalé que les vaisseaux de taille moyenne en distalité du coude et du genou pouvaient être occasionnellement intéressés dans la sclérodermie systémique [6][7][8][9][10]. Les anomalies angioscannographiques caractéristiques telle que l'occlusion et/ou la sténose sont habituellement observées dans la maladie artérielle sclérodermique indépendamment du site de l'affection, mais les plaques d'athérome qui sont des vaisseaux vermiculaires caractéristiques des artériopathies périphériques (AP) sont absentes [10].…”

Arterial vasculopathy in systemic sclerosis: Computerized tomography (CT) angiographic features of macrovascular and microvascular upper limbs arteries

“…Although digital ulcerations frequently occur in patients with SSc, there have been few reports on the macrovascular involvement. Previous reports suggested that medium -sized vessels distal from the elbow or knee are occasionally involved in SSc [6][7][8][9][10]. Characteristically CT angiographic features like occlusion and/or stenosis are usually observed in SSc arterial disease irrespective to the site of affection, yet atheromatous plaques, which are wormlike vessels that are characteristic of those observed in peripheral arterial disease (PAD), are absent [10].…”