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 Sj?gren’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.
Highlights
Digital gangrene or digital ulcers related to Raynaud’s phenomenon or cutaneous vasculitis are the most refractory clinical manifestations in collagen vascular diseases, and are occasionally accompanied by impaired quality of life and/or life-threatening systemic infections
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
Summary
Digital gangrene or digital ulcers related to Raynaud’s phenomenon or cutaneous vasculitis are the most refractory clinical manifestations in collagen vascular diseases, and are occasionally accompanied by impaired quality of life and/or life-threatening systemic infections. Digital gangrene is rarely found in collagen diseases, including systemic sclerosis and it is considered to show similar underlying vascular damage to that seen in PAD; it is often misdiagnosed as aggravated-digital ulcers related to Raynaud’s phenomenon, especially in case of scleroderma-spectrum disorders [1]. Treating these gangrenous conditions with medications intended for Raynaud’s phenomenon usually leads to unsatisfactory results and increasing the dosage of systemic steroids sometimes exacerbates gangrene due to the possible induction of hyperviscosity of plasma flow and platelet aggregation [2]. The radiological imaging findings obtained in this study were compared with those of PAD
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