Thromboangiitis Obliterans Disease

DESCRIPTION

Occlusion of small and medium sized arteries and veins caused by inflammatory changes in these vessels. It primarily occurs in men who smoke.

System(s) affected: Cardiovascular
Genetics: Greater prevalence of HLA-A54, HLA-A9 and HLA-B5. Familial cases reported rarely.
Incidence/Prevalence in USA: 13/100,000
Predominant age: 20 to 40 years
Predominant sex: Male > Female (3:1). Increasing numbers of women are being diagnosed, presumably due to increased smoking.

SIGNS AND SYMPTOMS

Symptoms tend to wax and wane in early disease and are often asymmetric. Symptoms may be gradual or have a sudden onset related to impaired vasculature.

Ulceration of digits; pain may be disabling
Coldness in feet and/or fingers
Cold sensitivity
Paresthesias (numbness, tingling, burning, hypoesthesia) of feet and/or fingers
Intermittent claudication in arch of foot or leg (rarely hand, forearm)
Persistent extremity pain (may be worse at rest)
Paroxysmal "electric shock" pain of ischemic neuropathy
Raynaud's phenomenon
Postural color changes (pallor on elevation; rubor on dependency)
"Buerger's color" – cyanosis of hands and feet
Migratory superficial phlebitis
Tender skin nodules on extremities
Impaired distal pulses; proximal pulses normal
Foot edema
Gangrene

CAUSES

(Postulated)

Smoking
Genetic factors
Autoimmune disorder with cell-mediated sensitivity to types I and III human collagens (both are constituents of blood vessels)
Impaired peripheral endothelium-dependent vasodilation

RISK FACTORS

Smoking tobacco
Incidence higher in Israel, Eastern Europe, Japan, India, Far East

LABORATORY

Routine laboratory studies show no changes characteristic of this disorder. Auto-antibodies to collagen and circulating immune complexes may be present, but are considered a research tool only.

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Segmental nonsuppurative panarteritis or panphlebitis with thrombosis
Histologic findings may vary between acute, subacute, and chronic stages of the disease
Histologic sine qua non – granulomas with collections of neutrophils in the organizing thrombus. Inflammatory reaction permeates the entire thickness of the vessel wall.
Chronic lesions show recanalized thrombus and perivascular fibrosis

SPECIAL TESTS

Doppler ultrasound (not specific)
Point scoring systems may help clarify clinical diagnosis

IMAGING

Arteriogram or digital-subtraction angiography (DSA)
- Multiple areas of segmental occlusion of small to medium arteries of arms and legs
- "Skip" areas may be demonstrated
- Numerous collateral vessels around occluded segments may give a characteristic "corkscrew" appearance
- Larger arteries are spared

DIAGNOSTIC PROCEDURES

History and physical examination
Studies of nerve conduction velocity (to exclude neuropathy)

APPROPRIATE HEALTH CARE

Outpatient
Inpatient if surgery needed for gangrene
Inpatient for dorsal or lumbar sympathectomy if indicated

GENERAL MEASURES

Stop smoking (mandatory)
Protect against trauma (poor fitting shoes)
Protect against infections
Protect against vasoconstriction from cold or drugs
Eliminate exposure to thermal damage
Eliminate exposure to chemical damage (iodine, carbolic acid, salicylic acid)
Thrombolytic therapy of occlusive thrombus and angioplasty are experimental

SURGICAL MEASURES

Amputation for non-healing ulcers, gangrene, or intractable pain. Should preserve as much limb as possible. Rarely required.
Omental autotransplantation has been successful in treating ulcers
In severe disease, a lumbar sympathectomy to increase blood supply to the skin
Direct revascularization of distal arteries is not practical unless coexistent atherosclerotic disease is present or patients stop smoking

ACTIVITY

Restricted by symptoms. Use a bed cradle (non-heated) to prevent pressure from bed linens.

DIET

No restrictions

PATIENT EDUCATION

Must stop smoking
Remove possibilities of exposure to others in the environment who smoke
Use heel pads or foam rubber boots
See General Measures

PREVENTION/AVOIDANCE

Never smoke

POSSIBLE COMPLICATIONS

Ulcerations
Gangrene
Need for amputation
Rare occlusion of cerebral, coronary, renal, splenic, or mesenteric arteries

EXPECTED COURSE AND PROGNOSIS

Occasional remissions
Unremitting progression if patient continues to smoke
Death rare; normal survival curve

ASSOCIATED CONDITIONS

N/A

AGE-RELATED FACTORS

Pediatric: Not a problem in this age group
Geriatric: Not common in this age group, but diagnosis in the elderly is increasing
Others: N/A

PREGNANCY

N/A

OTHER NOTES

May be difficult to differentiate from some types of atherosclerosis, systemic emboli or idiopathic peripheral thromboses

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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