Summary

• A non-atherosclerotic vasculitis resulting in segmental occlusions of small and medium-sized arteries.
• Highest incidence is in young men of southeast Mediterranean origin and of Middle and Far Eastern origin who smoke.
• Presents as an acutely ischaemic limb, more commonly affecting the lower limb. Claudication is rarely described.
• Laboratory investigations exclude other vascular disease. Appropriate imaging shows medium and small vessel occlusion. Histological analysis of arterial specimens shows preservation of the internal elastic lamina.
• Best outcomes are associated with smoking cessation.
• Life expectancy is not altered.