Buerger Disease: Symptoms, Types, Causes and Treatment

Buerger disease—also known as thromboangiitis obliterans—is a lesser-known but devastating vascular condition that primarily affects young adults, especially among those who smoke. Its impact is profound, often leading to severe pain, chronic ulcers, and even limb loss if not properly managed. In this comprehensive article, we'll explore the symptoms, distinct types, underlying causes, and current treatments for Buerger disease, drawing from up-to-date research and clinical experience.

Symptoms of Buerger Disease

Buerger disease can be insidious at its onset, often presenting with subtle symptoms that escalate over time. Recognizing these symptoms early is vital to minimize irreversible damage and improve patient outcomes.

Symptom	Description	Prevalence/Notes	Source(s)
Claudication	Pain/cramping in limbs during activity	Common initial symptom	2 3 4 5
Chronic Ulcers	Non-healing sores on fingers/toes	Seen in up to 80% of cases	3 5 4
Gangrene	Tissue death, often distal digits	Can require amputation	1 3 5
Rest Pain	Persistent pain even at rest	Indicates disease severity	4 14
Cold Sensation	Coldness in fingers/toes, especially in cold weather	Early presentation	2 4
Raynaud’s Phen.	Color changes in fingers/toes with cold/stress	Frequent in upper limbs	1
Phlebitis	Superficial migrating vein inflammation	Distinctive for Buerger	5 1

Table 1: Key Symptoms

Claudication and Rest Pain

The most common early manifestation of Buerger disease is claudication—pain or cramping in the hands or feet during activity, which subsides with rest. As the disease advances, the pain may persist even when not active (rest pain), typically worsening at night and disrupting sleep 2 3 4.

Chronic Ulcers and Gangrene

A hallmark of advanced Buerger disease is the development of chronic, non-healing ulcers, especially on the toes and fingers. Without intervention, these ulcers can progress to gangrene—tissue death—which may necessitate minor or even major amputations. In some reported series, up to half of patients required amputation at some stage, with lower limbs more frequently affected than upper limbs 3 1 5.

Cold Sensation and Raynaud’s Phenomenon

Many patients notice increased sensitivity to cold, with their fingers or toes feeling unusually cold or numb. Some experience Raynaud’s phenomenon, where the affected digits change color (white, blue, then red) in response to cold or stress, reflecting poor blood supply 2 1.

Phlebitis and Other Signs

A distinctive feature of Buerger disease, setting it apart from other vascular disorders, is superficial, migrating phlebitis—vein inflammation that appears as tender, red streaks under the skin. This can be an early sign and is highly suggestive of the disease 5 1.

Types of Buerger Disease

While Buerger disease is generally considered a single clinical entity, there is notable variability in its presentation and progression. Understanding these variations helps clinicians tailor management to each patient.

Type	Key Features	Severity/Progression	Source(s)
Mild	Intermittent claudication, minor symptoms	Slow, may remain stable	4 3
Moderate	Persistent pain, superficial ulcers	Risk of progression	4 3
Severe	Rest pain, deep ulcers, gangrene	High amputation risk	4 3 1
Upper Limb	Involvement of hands/arms	Less common than legs	1 3
Lower Limb	Involvement of feet/legs	Most frequently affected	3 5 11

Table 2: Clinical Variants and Severity

Disease Severity: Mild, Moderate, Severe

Clinical severity can be grouped into mild (intermittent claudication), moderate (more persistent pain, superficial ulcers), and severe forms (rest pain, deep ulcers, or frank gangrene). Severity grading is important for guiding treatment and prognostication 4.

• Mild: Patients may only notice discomfort on walking or using their hands.
• Moderate: More consistent pain and early tissue changes such as superficial ulcers.
• Severe: Rest pain, extensive ulcers, or tissue death (gangrene). These patients are at high risk for limb loss 4 3 1.

Limb Involvement: Upper vs. Lower

Buerger disease can affect both upper and lower limbs, but the legs and feet are most commonly involved. However, up to 15% of patients may have both upper and lower limb disease. Upper limb involvement often presents with Raynaud’s phenomenon or digital ulcers 1 3 5.

Venous Involvement

A unique aspect of Buerger disease is the involvement of the venous system, manifesting as migrating superficial thrombophlebitis. This is seen in a subset of patients and is helpful diagnostically 5 1.

Causes of Buerger Disease

The precise cause of Buerger disease remains a subject of ongoing research and debate. However, a few key risk factors and mechanisms are consistently implicated.

Factor	Description	Evidence/Mechanism	Source(s)
Tobacco Use	Cigarette or other tobacco product use	Central trigger	1 2 3 8
Genetic Susceptibility	Possible hereditary predisposition	Not fully defined	14 9
Immune Response	Immune-mediated vessel inflammation	Autoimmunity suspected	4 1 12
Endothelial Dysfunction	Damage to vessel lining (endothelium)	Early marker	6 10
Unknown Antigen	Hypothetical trigger inciting immune attack	Still unidentified	1 10

Table 3: Key Causes and Risk Factors

Tobacco Use: The Central Culprit

Tobacco use is the single most significant and consistent risk factor for Buerger disease. Nearly all patients diagnosed with the condition are current or former smokers, and disease activity is closely linked to continued tobacco use. Even small amounts of smoking can trigger or worsen the disease 1 2 3 8 11. Other forms of tobacco, including chewing tobacco, have also been implicated 3.

Genetic and Immune Factors

While not yet fully understood, there appears to be a genetic predisposition in some individuals, possibly related to specific HLA types. The disease is more prevalent in certain populations, such as those in the Middle East and Asia, suggesting environmental and genetic interplay 8 9 14.

Buerger disease is considered an immune-mediated endarteritis. Evidence includes the finding of immune complexes and complement deposits in affected vessels, and elevated inflammatory markers—especially in severe cases 4 1 12. However, the exact antigen (trigger) remains unknown 1 10.

Endothelial and Vascular Changes

Research shows that even vessels not yet clinically affected may have endothelial dysfunction—suggesting a diffuse, underlying process 6. This may be both a result of tobacco-induced injury and abnormal immune responses.

Other Risk Factors

While the vast majority of patients are young to middle-aged men, women can also be affected, especially as smoking rates rise among women 8. Less commonly, opiate use has been reported in some patient populations, but tobacco remains the principal risk factor 3.

Treatment of Buerger Disease

Managing Buerger disease is challenging, but early intervention can dramatically alter the outcome. The mainstay of therapy is complete tobacco cessation, but other approaches exist for advanced or complicated cases.

Treatment	Description/Goal	Efficacy/Notes	Source(s)
Tobacco Cessation	Complete abstinence from all tobacco	Only proven way to halt disease	1 2 6 7 8 12
Wound Care	Local treatment of ulcers/gangrene	Essential for healing	7 3
Pharmacologic Agents	Prostacyclins, prostaglandins, others	IV iloprost helps ulcers/pain	14 7
Surgical Options	Sympathectomy, bypass surgery	Selected cases, variable success	15 5
Therapeutic Angiogenesis	Gene/stem cell therapy	Experimental, promising	13 16 17

Table 4: Treatment Approaches

Tobacco Cessation: The Cornerstone

Quitting all forms of tobacco is the single intervention proven to halt the progression of Buerger disease. This can lead to disease quiescence and prevent new damage, though existing ulcers or gangrene may not reverse. Even small lapses in abstinence can reactivate the disease 1 2 6 7 8 12. Emerging therapies such as cannabinoid receptor antagonists may help patients overcome nicotine addiction 6 7.

Wound and Local Care

Proper local wound care is critical for patients with ulcers or gangrene. This includes meticulous cleaning, dressings, infection prevention, and sometimes limited surgical debridement 7 3.

Pharmacologic Treatments

• Prostacyclin Analogues: IV iloprost is more effective than aspirin for healing ulcers and relieving rest pain, but oral forms are not significantly better than placebo 14. These agents may be used in severe cases or when surgery is not feasible.
• Other Agents: Evidence for other drugs (cilostazol, clopidogrel, pentoxifylline) is lacking or inconclusive 14.

Surgical and Interventional Options

• Sympathectomy: Surgical or chemical interruption of sympathetic nerves can improve blood flow and pain in some patients, though results are variable 15 5.
• Bypass Surgery: Reserved for select patients with suitable target vessels. Success rates are modest due to the diffuse, distal nature of the disease 15.
• Amputation: Sometimes necessary in cases of irreversible gangrene; however, limb salvage is possible with early intervention 1 3 5 15.

Experimental Therapies

• Therapeutic Angiogenesis: Gene therapy using vascular endothelial growth factor (VEGF) and cell-based therapies (stem cell or bone marrow mononuclear cell transplantation) have shown promise in early studies, promoting new blood vessel growth and improved healing 13 16 17. Evidence is still limited, and more research is needed to confirm long-term safety and effectiveness.

Conclusion

Buerger disease is a uniquely challenging vascular disorder, tightly linked to tobacco use and marked by severe, progressive damage to blood vessels in the limbs. Early recognition and aggressive tobacco cessation are essential to prevent limb loss and preserve quality of life.

Key Points:

• Symptoms: Begin with claudication and cold extremities, progressing to ulcers and gangrene if untreated.
• Types: Range from mild to severe, with both upper and lower limb involvement possible.
• Causes: Tobacco use is the primary trigger, with immune and genetic factors playing a role.
• Treatment: Complete tobacco cessation is critical; adjunct therapies include wound care, pharmacologic agents, surgery, and experimental regenerative therapies.

For those at risk—especially smokers—awareness and early action can make all the difference. Continued research offers hope for new treatments, but prevention remains the best medicine for Buerger disease.