Buerger’s diseaseDz

Synonyms:   Thromboangiitis obliterans (TAO)

ICD-9 code: 443.1
ICD-10 code: I73.1

Definition: Buerger’s disease (also called thromboangiitis obliterans) is an inflammatory, nonatherosclerotic, occlusive, vascular disease characterized by thrombotic occlusions of small and medium sized arteries and veins in the upper and lower extremities. It is closely related to and initiated by tobacco use.

Etiology: Unknown. Tobacco use and periodontal disease are risk factors.

Demographics: TAO predominantly affects young male smokers. It is most prevalent in the Mediterranean, Middle East and Southeast Asia and highest among Ashkenazi jews, but is far less common in western Europe and N. America.  In many countries the prevalence has declined in the last few decades.  As a subset of peripheral vascular disease, (PAD) TAO accounts for few (<6%) of cases in N. America and up to 2/3 of all PAD cases in Korea, Japan and India.

Cardinal Features: Male, heavy smokers  between 20 and 40 years of age present with ischemic symptoms and finding, often without other risk factors for atherosclerotic disease. Distal limb ischemia manifests in the lower extremity as arch or or forefoot claudication, rest pain, distal cyanosis or gangrene. In the upper extremity common findings include asymmetric Raynaud’s phenomenon, digital gangrene or cyanosis, and symptoms of arm or hand claudication. All four limbs may be involved in over 40% of patients. Also common are findings of migratory superficial thrombophlebitis, absent pulses in the  radial, ulnar, dorsalis pedis and posterior tibial arteries. Involved vessels may be tender but bruits are uncommon.  Arterial pressures are normal proximally and depressed distally in affected limbs.

Uncommon Findings: Rarely are coronary, pulmonary, mesenteric or cerebral vessels involved.

Complications: Skip lesions may become confluent or vascular changes may progress proximally but seldom above the knee or elbow. Distal ischemia often leads to ulceration in the toes or digits and may progress to necrosis, infection, gangrene and amputation (25-43%).

Labs: Most laboratory tests, serologies (ANA, ANCA, etc.) and tests for thrombotic disorders (antithrombin III, protein C, protein S) are normal, except the erythrocyte sedimentation rate (ESR) may be abnormal in some, with modest to moderate elevations.

Imaging:  Computed tomographic, magnetic resonance or intravenous contrast angiography may be necessary to demonstrate the absence of atherosclerotic disease in the aorta and the normal appearance of proximal (i.e., brachial or femoral) arteries. Typical findings of TOA are best seen with IV contrast angiography and demonstrate multiple, segmental occlusions, oftent with direct corkscrew collateral vessels following the course of the thrombosed vessel (Mantorell’s sign).

Keys to Diagnosis: A history of heavy tobacco use, onset before 50 years, infrapopliteal arterial occlusions, upper limb involvement or migratory superficial thrombophlebitis, and absent distal pulses is highly suggestive. Typical angiographic findings are helpful but not pathognomonic.

Treatment: Tobacco abstinence is the single most important intervention as cessation lowers the risk of digit/limb amputation from 40% to <5% in those abstaining. Local care, vasodilators and sympathectomy may provide improvement.  Preliminary clinical trials have shown some efficacy with iloprost or bosentan. Vascular reconstruction or bypass grafting is seldom feasible or successful.

BIBLIOGRAPHY

Szuba A1, Cooke JP. Thromboangiitis obliterans. An update on Buerger's disease.  West J Med. 1998;168:255-60. PMID: 9584663

Piazza G, Creager MA. Thromboangiitis obliterans. Circulation. 2010;121:1858-61.  PMID: 20421527