International Journal of

cardiologyInternational Journal of Cardiology 54 SuppJ. (1996) S185-S187

Thromboangiitis obliterans (Buerger's disease)

Yoshio Mishima

Tokyo Medical and Dental University 5-45. Yushima. l-chome. Bunkyo-ku Tokyo. Japan

Abstract

Buerger disease is an chronic vasculitis mainly involving lower extremities. This disease is sometimes contrasted withTakayasu arteritis because both are speculated autoimmune mechanisms as an important factor in their etiology. Fur-thermore both are frequently encountered in Asian countries. Howerver, patients with Takayasu arteritis were mostlyyoung women in Japan and, on the contrary, patients with Buerger disease were mainly middle aged men. Recent HLAstudies on Buerger disease are revealing the interesting results in comparison with those on Takayasu arteritis. Theoutline of Buerger disease will be described herein.

Keywords: Thromboangiitis obliterans; Buerger disease; Autoimmune disease; HLA; Smoking

1. Introduction

In 1908, Leo Buerger published a paper onthromboangiitis obliterans: a study of the vascularlesions leading to presenile gangrene [1,2J. Thiswas not the first description of the condition, thefirst being by von Winiwarter [3J. The lesion is thesegmental, inflammatory and obliterative diseaseof medium-sized and small arteries, affectingmainly the distal upper and lower extremities ofyoung adult male smoker, often associated withmigratory thrombophlebitis.

Although Wessler [4J questioned the realexistence of Buerger's disease as a clinical entity in1960, Samuels [5J emphasized that there was anintense inflammatory reaction in the arteries, veinsand nerves in Buerger's disease. McKusick [6Jreported on Buerger's disease in 28 Korean malepatients. There are also many reports that suffi-ciently distinctive clinical and pathologicalcharacteristics are evident in a relatively small

Corresponding author, Fax: +81 3 58030138.

group of cases to permit classification of them ascases of thromboangiitis obliterans [7,8J. In 1962,we analyzed the patients suffering from chronic ar-terial occlusion of the extremities and concludedthat Buerger's disease does exist. Several reportshave laid stress on its prevalence in the Orient[10-14J.

2. Etiology

The specific etiology is not known. Secondaryetiologic factors which have a positive effect on thedisease include age, sex, race, hereditary factors(HLA antigen), autoimmune process, occupation,changes in the blood and smoking. Smoking isthought to be the strongest secondary etiologicfactor. Buerger's disease has rarely been reportedin non-smokers, and the condition improves fre-quently after abstinence from smoking. In ourseries [16J, smokers were 91.1% in Buerger'sdisease, whereas the ratio in the control group was79%.

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SI86 y. Mishima Iintenrational Journal of Cardiology 54 Suppl. (1996) S185-S187

3.Patbology

Most authors agree that inflammatory changesin all three layers of the involved vessel walls andthrombotic occlusion of the affected segments arecharacteristic, followed by recanalization.

In the acute stage, there may be fibroblasts andlymphocytes, and sometimes a giant cell. The oc-cluding thrombus is very cellular and containsmany nuclei of fibroblasts. Usually, the diseaseaffects primarily medium-sized and small arteriesin segments, with relatively normal portionbetween involved segments.

In the old lesion, the involved artery was occlud-ed by well organized thrombi with recanalization.Therefore, it is generally difficult to determine itsreal etiology by the resected specimen obtainedfrom these old lesions.

The migratory thrombophlebitis recurredfrequently in extremities and observed before,during or after the onset of arterial lesions. Thehistopathologic changes in the involved superficialvein is similar with those observed in the involvedarteries. Consequently, it is different from thesimple venous thrombosis.

4. Pathopbysiology

The symptoms are those which arise from thearterial occlusion, those which depend upon theinflammatory nature of the lesions, and thoseresulting from the breakdown of the tissuerendered ischemia.

As to initial symptoms, incidence of gangrenewas high. Intermittent claudication occurs, but isless frequent than in arteriosclerosis obliterans,because the disease involves mostly the smallervessels, such as anterior or posterior tibial artery,producing extensive tissue damage in many casesbefore claudication develops.

The symptoms due to inflammatory nature arethose of ischemic neuritis producing rest pain andthe assosiated thrombophlebitis.

The occlusive pattern and the age distribution ofBuerger's disease is characteristic and is differentfrom those of arteriosclerosis obliterans. In Japan,the typical cases of Buerger's disease have decreas-ed in number and arteriosclerosis obliterans has

increased, depending upon the progress of thediagnostic procedures and the europeanization ofthe living style and the dietary habits after the Sec-ond World War.

5. Diagnosis

The diagnostic criteria used are as follows [16].(I) Asymmetrical abnormal coldness of the skin inthe extremities, (2) impairment or absence ofperipheral arterial pulsations, (3) excluding thecases with hypertension, hyperlipidemia, album-inuria, glycosuria, calcification, abnormal ECG,retinal atherosclerosis, etc., (4) arteriographicfindings: tapering, abrupt occlusion, corkscrewappearance of collaterals, and (5) excluding thecases with atheroma formation.

As diagnostic aids, (l) chronic arterial occlusionof limbs involving young males age 15-40 years,(2) usually cigarette smoker, and (3) association ofmigratory thrombophlebitis are recommended.

The above mentioned criteria are thought to besufficient in order to establish the clinical diagno-sis, although they may include in part simplethrombosis or some type of non-manifestedatherosclerotic occlusions.

6. Prognosis

In our series [I6J, the patients with Buerger'sdisease had a practically normal life survival ascompared to a normal population of the same ageand sex distribution. The survival curves in thisseries resemble those reported by McPharson [I5Jand both curves are distinctly better than those ofthe patients with arteriosclerosis obliterans.

7. Treatment

Suspicion of Buerger's disease is the signal forcomplete abstinence from smoking and the institu-tion of a variety of other supportive measures,although none of them is specific. Current conser-vative measures consist of antiplatelet agents,vasodilators, chemical sympathectomy, and physi-cal exercise. Sympathectomy is helpful to promotewound healing and to make amputation as distalas possible.

Y. Mishima / International Journal of Cardiology 54 Supp/. (/996) SI85-SJ87 SI87

The arterial reconstructive surgery seems to berarely indicative, but it should be performedactively in the indicated cases selected by profoundstudies [69].

References

[I] Buerger L. Thrombo-angiitis obliterans: A study of thevascular lesions leading to presenile spontaneousgangrene. Am J Med Sci 1909;136:567.

[2] Buerger L. The veins in thromboangiitis obliterans. J AmMed Assoc 1909;111:1320.

[3] von Winiwarter F. Ueber eine eigentuemliche Form vonEndarteritis und Endophlebitis mit Gangraen des Fusses.Arch Klin Chir 1879;23:202.

[4] Wessler S, Ming SC, Gurewich V, Freimann BG. A criti-cal of thromboangiitis obliterans. The case againstBuerger's disease. New Eng! J Moo 1960;262:1149.

[5] Samuels SS. Buerger's disease. Angiology 1960;11:213.[6] McKusick VA, Harris WS, Ottesen OE, Goodman RM,

Shelly WM, Bloodwell RD. Buerger's disease: A distinctclinical and pathologic entity. J Am Moo Assoc1962;181:5.

[7] Schatz 11, Fine G, Eyler WR. Thromboangiitisobliterans. Br Heart J 1966;28:84.

[8] Brown H, Sellwood RA, Harrison CV, Martin P. Throm-boangiitis obliterans. Br J Surg 1969;56:59.

[9) Ishikawa K, Kawase S, Mishima Y. Occlusive arterialdisease in extremities, with special reference to Buerger'sdisease. Angiology 1962;13:399.

[10) Goodman RM, Elian B, Mozes M, Deutsch V. Buerger'sdisease in Israel. Am J Moo 1965;39:601.

[II) Kradjian R, Bowels LT, Edwards WS. Peripheral arterialdisease in Ceylon. Surgery 1971;69:523.

[12] Hill GL, Smith AH. Buerger s disease in Indonesia: Clini-cal course and prognostic factors. J Chron Dis1974;29:205.

[13] Reddi HTV. Thromboangiitis obliterans and/orBuerger's disease in South India: A review of 70 cases.Int Surg 1974;59:555.

[14) Wong J, Lam STK, Ong GB. Buerger's disease - areview of 105 patients. Aust NZ J Surgery 1978;48:382.

[15) McPharson JR, Juergens JL, Gifford RW Jr. Throm-boangitis obliterans and arteriosclerosis obliterans:Clinical and prognostic differences. Ann Intern Med1963;59:288.

[16) Annual reprots of Research Committee on Buerger'sdisease - Ministry of Health and Welfare Japan;1985-1995 (in Japanese).