Thromboangiitis Obliterans (Buerger Disease)

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Thromboangiitis obliterans (TAO), an inflammatory vasculopathy also known as Buerger disease, is characterized by an inflammatory endarteritis that causes a prothrombotic state and subsequent vaso-occlusive phenomena. The inflammatory process is initiated within the tunica intima. It characteristically affects small and medium-sized arteries as well as veins of the upper and lower extremities. The condition is strongly associated with heavy tobacco use, and disease progression is closely linked to continued use. (See Pathophysiology and Etiology.)

Patients often present with moderate-to-severe claudication that can quickly progress to critical limb ischemia featuring rest pain or tissue loss. Features of acute limb ischemia (eg, pain, paresthesia, palor, mottling, poikilothermia, paresis, and pulselessness) are common signs and symptoms encountered in the emergency setting. [1, 2, 3, 4] (See Presentation.)

Pharmacologic therapy is generally ineffective; abstinence from tobacco is the only measure known to prevent disease progression. (See Treatment.) Given the arteritis of the small and medium-sized vessels, surgical or endovascular revascularization may not be possible, because of the absence of a distal target for revascularization. As the disease evolves, amputation may be the only viable option.

As noted, the development of TAO is strongly associated with heavy use of tobacco, and the progression of the disease is closely linked to continued use.

A few observations suggest the existence of an immunologic phenomenon leading to vasodysfunction and inflammatory thrombi. Patients with TAO exhibit hypersensitivity to intradermally injected tobacco extracts, increased cellular sensitivity to collagen types I and III, elevated serum anti–endothelial cell antibody titers, and impaired peripheral endothelium-dependent vasorelaxation. They also show a higher prevalence of human leukocyte antigen (HLA)–A9, HLA-A54, and HLA-B5, suggesting a genetic component to the disease.

The prevalence of TAO has decreased over the past decade, partly because the prevalence of smoking has decreased but also because the diagnostic criteria have become more stringent. In 1947, the prevalence of the disease in the United States was 104 cases per 100,000 population. Since then, the prevalence has fallen to an estimated 12.6-20 cases per 100,000 population.

Most patients with TAO are aged 20-45 years; the disease does not occur in pediatric or elderly patients. TAO is more common in males (male-to-female ratio, 3:1); however, the incidence in women is believed to be increasing, probably as a consequence of the growing frequency of smoking among women. The disease is relatively less common in people of northern European descent; natives of India, Korea, and Japan, along with Israeli Jews of Ashkenazi descent, have the highest incidence of TAO. [5]

Death from TAO is rare. Between 1999 and 2007, according to data from the US Centers for Disease Control and prevention (CDC), TAO (code I73.1 in the International Classification of Diseases, Tenth Revision [ICD-10]) was the underlying cause of 117 deaths in the United States. [6]

A striking dichotomy is observed in the prognosis of patients with TAO, which is dependent on whether absolute avoidance of tobacco is achieved. Among patients who stop using tobacco, 94% avoid amputation; among patients who stop using tobacco before progression to critical limb ischemia, the amputation rate is near 0%. In stark contrast, among patients who continue using tobacco, there is an 8-year amputation rate of 43%.

Patients with TAO must be repeatedly advised to cease all use of or exposure to tobacco products (including chewing tobacco, nicotine patches and gums, and second-hand smoke) and reassured that if they are able to discontinue tobacco use, the disease will remit and amputation will be avoided.

Physicians should counsel patients that the level of tobacco avoidance required to achieve resolution of their disease often necessitates that they rigorously limit their exposure even to secondhand smoke. This can be extremely difficult for patients who live with another smoker, and it is therefore not unreasonable to consider referring such patients (and their loved ones) to multidisciplinary smoking cessation programs.

For patient education resources, see the Healthy Living Center and the Lung and Airway Center, as well as Cigarette Smoking.

von Winiwarter F. Ueber eine eigenthumliche Form von Endarteriitis und Endophlebitis mit Gangran des Fusses. Arch Klin Chir. 1879. 23:202-26.

Buerger L. Thrombo-angiitis obliterans: a study of the vascular lesions leading to presenile spontaneous gangrene. Am J Med Sci. 1908. 136:567-80.

Espinoza LR. Buerger’s disease: thromboangiitis obliterans 100 years after the initial description. Am J Med Sci. 2009 Apr. 337(4):285-6. [Medline].

Małecki R, Zdrojowy K, Adamiec R. Thromboangiitis obliterans in the 21st century–a new face of disease. Atherosclerosis. 2009 Oct. 206 (2):328-34. [Medline].

Salimi J, Tavakkoli H, Salimzadeh A, Ghadimi H, Habibi G, Masoumi AA. Clinical characteristics of Buerger’s disease in Iran. J Coll Physicians Surg Pak. 2008 Aug. 18(8):502-5. [Medline].

ICD10Data.com. 2014 ICD-10-CM Diagnosis Code I73.1. Available at http://www.icd10data.com/ICD10CM/Codes/I00-I99/I70-I79/I73-/I73.1. Accessed: August 7, 2018.

Abyshov NS, Zakirdzhaev EA, Aliev ZM. [Modern aspects of diagnostics and treatment for thromboangiitis obliterans]. Khirurgiia (Mosk). 2009. 75-9. [Medline].

Olin JW, Young JR, Graor RA, Ruschhaupt WF, Bartholomew JR. The changing clinical spectrum of thromboangiitis obliterans (Buerger’s disease). Circulation. 1990 Nov. 82(5 Suppl):IV3-8. [Medline].

Motukuru V, Suresh KR, Vivekanand V, Raj S, Girija KR. Therapeutic angiogenesis in Buerger’s disease (thromboangiitis obliterans) patients with critical limb ischemia by autologous transplantation of bone marrow mononuclear cells. J Vasc Surg. 2008 Dec. 48(6 Suppl):53S-60S; discussion 60S. [Medline].

Kulkarni S, Kulkarni G, Shyam AK, Kulkarni M, Kulkarni R, Kulkarni V. Management of thromboangiitis obliterans using distraction osteogenesis: A retrospective study. Indian J Orthop. 2011 Sep. 45(5):459-64. [Medline]. [Full Text].

Papa MZ, Rabi I, Adar R. A point scoring system for the clinical diagnosis of Buerger’s disease. Eur J Vasc Endovasc Surg. 1996 Apr. 11(3):335-9. [Medline].

Graziani L, Morelli L, Parini F, Franceschini L, Spano P, Calza S, et al. Clinical Outcome After Extended Endovascular Recanalization in Buerger’s Disease in 20 Consecutive Cases. Ann Vasc Surg. 2012 Apr. 26(3):387-95. [Medline].

Fazeli B, Dadgar Moghadam M, Niroumand S. How to Treat a Patient with Thromboangiitis Obliterans: A Systematic Review. Ann Vasc Surg. 2018 May. 49:219-228. [Medline].

Kawarada O, Kume T, Ayabe S, Nakaya T, Nakai M, Nishimura K, et al. Endovascular Therapy Outcomes and Intravascular Ultrasound Findings in Thromboangiitis Obliterans (Buerger’s Disease). J Endovasc Ther. 2017 Aug. 24 (4):504-515. [Medline].

Kim DH, Ko YG, Ahn CM, Shin DH, Kim JS, Kim BK, et al. Immediate and late outcomes of endovascular therapy for lower extremity arteries in Buerger disease. J Vasc Surg. 2018 Jun. 67 (6):1769-1777. [Medline].

Modaghegh MS, Hafezi S. Endovascular Treatment of Thromboangiitis Obliterans (Buerger’s Disease). Vasc Endovascular Surg. 2018 Feb. 52 (2):124-130. [Medline].

Lawrence PF, Lund OI, Jimenez JC, Muttalib R. Substitution of smokeless tobacco for cigarettes in Buerger’s disease does not prevent limb loss. J Vasc Surg. 2008 Jul. 48(1):210-2. [Medline].

Melillo E, Grigoratos C, Sanctis FD, Spontoni P, Nuti M, Dell’Omodarme M, et al. Noninvasive Transcutaneous Monitoring in Long-Term Follow-Up of Patients With Thromboangiitis Obliterans Treated With Intravenous Iloprost. Angiology. 2015 Jul. 66 (6):531-8. [Medline].

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Positive Criterion

Positive Points

Age at onset

< 30 y (+2)

30-40 y (+1)

Foot intermittent claudication

Present (+2)

By history only (+1)

Upper extremity

Symptomatic (+2)

Asymptomatic (+1)

Migrating superficial thrombophlebitis

Present (+2)

By history only (+1)

Raynaud phenomenon

Present (+2)

By history only (+1)

Angiography; biopsy

If typical, both (+2)

Either(+1)

Negative Criterion

Negative Points

Age at onset

45-50 y (−1)

>50 y (−2)

Sex; smoking

Female (−1)

Nonsmoker (−2)

Location

Single limb (−1)

No lower extremity involved (−2)

Absent pulses

Brachial (−1)

Femoral (−2)

Arteriosclerosis, diabetes, hypertension, hyperlipidemia

Discovered 5.1-10 y after diagnosis (−1)

Discovered 2.1-5 y later (−2)

Score

Probability of Diagnosis

0-1

Diagnosis excluded

2-3

Diagnosis suspected (low probability)

4-5

Diagnosis probable (medium probability)

≥6

Diagnosis definite (high probability)

Naiem Nassiri, MD RPVI, Instructor in Vascular Surgery, Rutgers Robert Wood Johnson Medical School; Founder and Director, Vascular Anomalies and Malformations Program, Vascular Center of New Jersey and Bristol Myers Squibb Children’s Hospital Center for Advanced Surgery

Naiem Nassiri, MD is a member of the following medical societies: American College of Surgeons, Society for Vascular Surgery, American Venous Forum, Society for Clinical Vascular Surgery, International Society for the Study of Vascular Anomalies

Disclosure: Nothing to disclose.

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Surgical Association, Pacific Coast Surgical Association, Society for Clinical Vascular Surgery, Society for Vascular Surgery, Western Vascular Society

Disclosure: Nothing to disclose.

Matthew Carpenter, MD Program Director, Department of Internal Medicine, Department of Internal Medicine, Keesler Medical Center; Assistant Clinical Professor, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine

Disclosure: Nothing to disclose.

E Jerry Cohn Jr, MD, FACS Vascular Surgeon, The Vein Center at Savannah Vascular Institute

E Jerry Cohn Jr, MD, FACS is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Eric J Hanly, MD Fellow, Department of Surgery, The Johns Hopkins University School of Medicine

Eric J Hanly, MD is a member of the following medical societies: American Medical Association, Association of Military Surgeons of the US, MedChi, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Ozanan R Meireles, MD Instructor in Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School

Ozanan R Meireles, MD is a member of the following medical societies: American College of Surgeons, American Society for Gastrointestinal Endoscopy, and Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Brian D Peyton, MD Chief of Vascular and General Surgery, Keesler Medical Center; Assistant Professor, Department of Surgery, Associate Program Director, Department of General Surgery, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Thromboangiitis Obliterans (Buerger Disease)

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