Thromboangiitis obliterans

The condition occurs almost exclusively in smokers.

Thromboangiitis obliterans is predominantly a clinical diagnosis, as well as one of exclusion, although sometimes inflammatory venous nodules can be biopsied and a definitive diagnosis established.

Management consists of three modalities:

1. Cessation of smoking
2. Medical management
3. Surgical management

As there is no truly effective medical treatment, immediate and total abstinence from tobacco and cannabis is essential.

This is the only measure that will substantially lessen the likelihood of amputation.

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History

The first medical description was likely by Austrian physician Felix von Winiwarter in 1879.

A detailed pathological description was later provided by Leo Buerger in 1908, after whom the condition is now commonly named.

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Epidemiology

• Most patients present before the age of 45.
• Occurs almost exclusively in smokers.
• Most common in regions with high tobacco use prevalence.

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Pathology

Cause

Strongly associated with tobacco. Cannabis arteritis is clinically and pathologically indistinguishable but less common.

There is no well-documented case in a non-tobacco or non-cannabis user confirmed by toxicology.

Reported in:

• Cigarette smokers
• Cigar smokers
• Marijuana users
• Smokeless tobacco users (e.g. chewing tobacco, nicotine patches)

Pathophysiology

• Non-atherosclerotic, segmental inflammatory disease of distal vessels.
• Immunologic and inflammatory processes implicated (e.g. anti-endothelial antibodies).

Histological features:

• Highly cellular, segmental intraluminal thrombus
• Relative sparing of the internal elastic lamina

Histological stages:

1. Acute Phase
   • Inflammatory occlusive thrombi with polymorphonuclear cells and microabscesses
   • Internal elastic lamina intact
2. Intermediate Phase
   • Progressive thrombus organization with less vessel wall inflammation
3. Chronic Phase
   • Organized thrombus and fibrosis with absent inflammation
   • Indistinguishable from other chronic occlusive arterial diseases

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Clinical Features

Typically presents in smokers < 45 years with distal ischemia, ulcers, or digital gangrene.

Disease Progression

1. Early Small Venous Involvement
   • Migratory superficial thrombophlebitis (tender nodules)
   • May precede ischemia
   • Often parallels arterial activity
   • Raynaud’s in >40%
2. Small Artery Involvement (Digital Ischemia)
   • Pain, cyanosis/rubor (“Buerger’s colour”), ulcers, gangrene
3. Larger Vessel Involvement
   • Less common; always with distal involvement
   • Claudication in hands/arms or feet/legs

Diagnostic Criteria

1. Age < 45
2. Current or recent tobacco use
3. Distal extremity ischemia (confirmed on testing)
4. Exclusion of other causes: autoimmune disease, thrombophilia, diabetes, embolic sources

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Investigations

Blood Tests

Used to exclude differential diagnoses:

• FBE
• CRP / ESR
• U&Es / Glucose
• LFTs
• Coagulation profile
• Toxicology (e.g. cocaine, cannabis)
• Anticardiolipin / anti-endothelial antibodies (may be positive)

Imaging

CT Angiography / MRA
Not ideal for distal anatomy but can provide useful findings.

Characteristic angiographic findings:

1. Segmental arterial occlusions
2. Corkscrew collaterals (not pathognomonic)
3. Involvement of more than one limb
4. Distribution in small/medium arteries (plantar, tibial, radial, ulnar)
5. Sparing of proximal inflow vessels
6. No signs of atherosclerosis or embolism

Biopsy

• Definite diagnosis if biopsy of superficial vein/subcutaneous nodule shows classic findings.

Urine Testing

• Cotinine/nicotine levels useful if smoking cessation is in doubt.

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Management

Management includes:

1. Cessation of smoking
2. Medical therapy
3. Surgical options

Cessation of Smoking

• The only intervention that substantially reduces amputation risk
• Nicotine replacement therapies (e.g. patches, gum) should also be avoided
• Bupropion or varenicline can be used to support cessation
• Continued smoking → up to 50% amputation rate

Medical Management

Primarily symptomatic:

• Vasodilators:
  • Dihydropyridine CCBs (e.g. nifedipine)
  • Alpha-blockers
  • Transdermal nitrates
  • Iloprost (IV prostacyclin analogue)

Surgical Management

• Sympathectomy may help alleviate pain
• Debridement of ulcers and necrotic tissue
• Revascularization rarely feasible (distal disease). May be indicated if concurrent peripheral artery disease present

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References

Publications

• Jorge VC, Araújo AC, Noronha C, Panarra A, Riso N, Vaz Riscado M. Buerger’s disease (Thromboangiitis obliterans): a diagnostic challenge. BMJ Case Rep. 2011 Sep 13;2011
• Piazza G, Creager MA. Thromboangiitis obliterans. Circulation. 2010 Apr 27;121(16):1858-61.

FOAMed

• Leo Buerger (1879-1943) and Buerger disease