- Definition
- A non-atherosclerotic, segmental, inflammatory vasculitis primarily affecting small- and medium-sized arteries and veins. It is strongly associated with tobacco use.
- Epidemiology
- Occurs mostly in young males aged 20–45 years and has a higher prevalence in smokers and regions with common tobacco use.
- Pathophysiology
- Chronic inflammation of vessel walls leads to thrombosis, ischemia, and tissue necrosis, predominantly involving distal extremities and leading to gangrene in advanced cases.
- Risk factors
- Tobacco use, including smoking or chewing, is the major trigger, and genetic predisposition may play a role in some populations.
- Clinical
- Early symptoms include pain in affected limbs, claudication, or Raynaud’s phenomenon, while advanced symptoms include rest pain, ulceration, and gangrene in fingers or toes.
- Superficial thrombophlebitis may also occur.
- Diagnosis
- Involves Shionoya’s clinical criteria: onset before age 50, tobacco use history, involvement of distal vessels, exclusion of other diseases like atherosclerosis or autoimmune conditions, and positive angiographic findings showing “corkscrew” collateral vessels in distal extremities.
- Investigations
- Angiography to show segmental occlusion and collateral formation and blood tests to exclude autoimmune or hypercoagulable conditions.
- Management
- Includes complete cessation of tobacco, which is the cornerstone of treatment and halts disease progression.
- Smoking cessation programs or nicotine replacement therapy may be required.
- Vasodilators like calcium channel blockers are used for ischemic symptoms, and antiplatelets reduce thrombus formation. Pain is controlled with analgesics.
- Sympathectomy may improve symptoms in severe ischemia, while amputation is reserved for nonviable tissue or uncontrolled infection.
- Prognosis
- Depends on tobacco cessation, as continued smoking leads to recurrent thrombosis, severe ischemia, and limb loss.