Buerger disease in an elderly man.

Abstract: Buerger disease is characterized by progressive distal extremity ischemia in persons with recent tobacco consumption. Typically, affected persons are young men. There have been case reports of Buerger disease in older men. Almost all of the reported cases were characterized with progressive, severe disease requiring amputation. We report a case of an older man with features of Buerger disease without significant proximal progression despite active smoking. Arteriographic and pathologic studies confirmed the diagnosis.

Key Words: Buerger disease, tobacco consumption, older age, inflammatory venous and arterial thrombosis, small- or medium-sized vessels, angiographic features, autoimmune and hypercoagulable diseases

Case Report

A 76-year-old man presented to clinic due to bilateral foot pain and numbness for several years with recent worsening. He had previously been seen by other physicians and had been placed on several pain medications, including gabapentin, that did not help. His foot pain was not exertional, although the patient was not very active due to significant exertional dyspnea. He denied calf pain, orthopnea, paroxysmal nocturnal dyspnea (PND), syncope, and chest pain. His past medical history was remarkable for chronic obstructive pulmonary disease (COPD) and osteoarthritis in his knees. There was no history of diabetes, cerebrovascular accident (CVA), or known heart disease.

He smoked two packs per day for 50 years. He did not take any medications at the time of presentation.

Examination revealed an older man in no distress at rest. There were no head or neck abnormalities. There was no carotid bruit or jugular venous distention (JVD). Lungs revealed no wheezes or signs of consolidation. Cardiac auscultation revealed a 3/6 systolic ejection murmur over the left sternal border with a normal aortic component of the second heart sound with wide splitting heard over the pulmonic area. Abdomen revealed no masses or bruit. Hands revealed diminished radial and ulnar pulses bilaterally without significant skin changes. Signs of healed ulcers were noted on the tips of his right index and middle finger. Legs revealed blanching dark erythema in both feet below the ankles with signs of multiple healing ulcers on the dorsum of the feet and multiple toes. Several of his toes were cyanotic (Fig. 1). He had normal pulses over both femoral and popliteal arteries. Pulses were absent over both posterior tibial and dorsalis pedis arteries. Neurologic examination was significant for a remarkable decrease in sensation in both feet and lower legs.

Sedimentation rate, antinuclear antibodies (ANA), rheumatoid factor (RF), cryoglobulins, anticentromere antibody, Scl-70, antiphospholipid and anticardiolipin antibodies were all normal or negative. A transthoracic echocardiogram revealed mild to moderate aortic valve stenosis but revealed no cardiac source of emboli. A skin biopsy was obtained from the dorsum of the left foot. The biopsy revealed normal skin with changes in the subcutaneous tissue consistent with fresh venous thrombosis. Inflammatory cell infiltration was also noted in the subcutaneous tissue. An arteriogram of the lower extremities revealed widely patent vessels until the level of the lower calf where blood flow disappeared in both legs (Figs. 2-3). Segmental narrowing was noted in the left superficial femoral and popliteal arteries with little col-lateralization. These pathologic and radiographic findings were diagnostic of Buerger disease.

Discussion

Buerger disease, known as thromboangiitis obliterans, is more prevalent in the Middle and Far East than it is in the United States. (1) The incidence of Buerger disease in the US is 12.6 per 100,000 as of 1986. (1,2) Although there are reports of increasing prevalence of the disease in women, it remains primarily a disease of young men. The typical age of onset is below 40 years. (3,4) Thromboangiitis obliterans is a nonathero-sclerotic, segmental, inflammatory disease that affects the veins, nerves, and small- and medium-sized arteries of the distal lower and upper extremities. (3) Pathologically, Buerger disease is characterized by inflammatory thrombus without inflammatory involvement of the blood vessel wall. Tobacco use seems to be the main association and/or cause of the disease initiation and progression. (3,5,6) Although there are reports of Buerger disease in nonsmokers or users of smokeless tobacco or snuff, (7) it is believed that Buerger disease occurs predominantly in smokers. It is more common in countries where tobacco is heavily used, especially where cigarettes are handmade with raw tobacco. (8)

[FIGURE 1 OMITTED]

Buerger disease is manifested as pain and/or claudication of the hands and feet and ischemic ulcerations of the fingers and/or toes with accompanying ischemic pain at rest. It usually begins with ischemia of the distal small arteries and veins, followed by more proximal arterial involvement as the disease advances. (3)

[FIGURE 2 OMITTED]

Buerger disease should be suspected in young smokers who present with distal ischemia of the hands and/or feet. Autoimmune and hypercoagulable diseases must be ruled out. A definitive diagnosis (although not always required) is made when the histopathological examination identifies an acute-phase lesion of recent inflammatory venous and/or arterial thrombosis in small- or medium-sized vessels in a patient with a clinical history of active smoking or tobacco consumption. Typical arteriographic features of involvement of the distal vessels of the upper and lower extremities are supportive of the diagnosis. (9-12)

[FIGURE 3 OMITTED]

Our patient met the diagnostic criteria for Buerger disease except for the older age at onset. He also differed from patients with classic Buerger disease in that there was no noted clinical proximal progression of the disease, despite active smoking. However, he did have active disease. There have been rare case reports of thromboangiitis obliterans in older male smokers involving the upper and lower extremities. Almost all the reported cases were characterized with severe disease requiring amputation. (13,14) The disease and the pathologic findings have been reported in cerebral, coronary, renal, mesenteric, and internal thoracic arteries. (15-18) There are rare reports of large vessel involvement, such as the aorta, pulmonary arteries, and iliac arteries. (19,20) Thrombolytic therapy, surgical revascularization, and sympathectomy are suggested treatments but they all show minimal effects. (21-24) Smoking cessation and discontinuation of tobacco intake are the only effective procedures to halt the disease. Intravenous Iloprost (a prostaglandin analog) given for up to six months has shown total relief of pain at rest and complete healing of all trophic changes of limb ulcers in a small randomized trial. (25) Our patient failed smoking cessation counseling and treatment. He continued to smoke and continued to have active disease and symptoms.

Conclusion

Age less than 40 years, current or recent history of tobacco use, distal extremity ischemia, and exclusion of autoimmune and hypercoagulable diseases are the criteria for diagnosing Buerger disease. Old age, however, does not exclude Buerger disease. If other diagnostic criteria are met, it is recommended that histopathological and angiographic studies be performed. If these studies are consistent with Buerger disease, the diagnosis can be made. Smoking cessation is currently the most effective treatment. (9-12)

References

1. Lie JT. The rise and fall and resurgence of thromboangiitis obliterans (Buerger's disease). Acta Pathol Jpn 1989;39:153-155.

2. Lie JT. Thromboangiitis obliterans (Buerger's disease) in women. Medicine (Baltimore) 1987;66:65-72.

3. Olin JW. Thromboangiitis obliterans (Buerger's disease). N Engl J Med 2000;343:864-869.

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5. Mills JL, Porter JM. Buerger's disease (thromboangiitis obliterans). Ann Vasc Surg 1991;5:570-572.

6. Papa M, Bass A, Adar R, et al. Autoimmune mechanisms in thromboangiitis obliterans (Buerger's disease): the role of tobacco antigen and the major histocompatibility complex. Surgery 1992;111:527-531.

7. Lie JT. Thromboangiitis obliterans (Buerger's disease) and smokeless tobacco. Arthritis Rheum 1988;31:812-813.

8. Grove WJ, Stansby GP. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl 1992;74:115-117.

9. Papa MZ, Rabi I, Adar R. A point scoring system for the clinical diagnosis of Buerger's disease. Eur J Vase Endovasc Surg 1996;11:335-339.

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12. Shionoya S. What is Buerger's disease? World J Surg 1983;7:544-551.

13. Du Toit DF, Maritz J, Klompje J, et al. Buerger's disease: a case report and review of the literature. S Afr Med J 1984;66:701-702.

14. Akimoto N, Akutsu H, Tsuchida H, et al. An aged case of peripheral arterial obstructive disease with progressive limb necrosis. Nippon Geka Gakkai Zasshi 1989;90:622-625.

15. Donatelli F, Triggiani M, Nascimbene S, et al. Thromboangiitis obliterans of coronary and internal thoracic arteries in a young woman. J Thorac Cardiovasc Surg 1997;113:800-802.

16. Lie JT. Visceral intestinal Buerger's disease. Int J Cardiol 1998;66(Suppl 1):S249-S256.

17. Hoppe B, Lu JT, Thistlewaite P, et al. Beyond peripheral arteries in Buerger's disease: angiographic considerations in thromboangiitis obliterans. Catheter Cardiovasc Interv 2002;57:363-366.

18. Kurata A, Nonaka T, Arimura Y, et al. Multiple ulcers with perforation of the small intestine in Buerger's disease: a case report. Gastroenterology 2003;125:911-916.

19. Harten P, Muller-Huelsbeck S, Regensburger D, et al. Multiple organ manifestations in thromboangiitis obliterans (Buerger's disease): a case report. Angiology 1996;47:419-425.

20. Michail PO, Filis KA, Delladetsima JK, et al. Thromboangiitis obliterans (Buerger's disease) in visceral vessels confirmed by angiographic and histological findings. Eur J Vasc Endovasc Surg 1998;16:445-448.

21. Hussein EA, el Dorri A. Intra-arterial streptokinase as adjuvant therapy for complicated Buerger's disease: early trials. Int Surg 1993;78:54-58.

22. Sasajima T, Kubo Y, Inaba M, et al. Role of infrainguinal bypass in Buerger's disease: an eighteen-year experience. Eur J Vasc Endovasc Surg 1997;13:186-192.

23. Lau H, Cheng SW. Buerger's disease in Hong Kong: a review of 89 cases. Aust N Z J Surg 1997;67:264-269.

24. Sayin A, Bozkurt AK, Tuzun H, et al. Surgical treatment of Buerger's disease: experience with 216 patients. Cardiovasc Surg 1993;1:377-380.

25. Fiessinger JN, Schafer M. Trial of iloprost versus aspirin treatment for critical limb ischaemia of thromboangiitis obliterans: the TAO Study. Lancet 1990;335:555-557.

Hassan M. Ismail, MD, MPH, FACP 

From East Tennessee State University, James Quillen College of Medicine, Division of Cardiovascular Disease, 2 Professional Park, suite 15, Johnson City, TN.

Reprint requests to Dr. Hassan M. Ismail, East Tennessee State University, James Quillen College of Medicine, 325 N. State of Franklin Road, 2nd Floor, Johnson City, TN 37604. Email: [e-mail]

Accepted July 17, 2006.

RELATED ARTICLE: Key Points

* Buerger disease is a nonatherosclerotic, segmental, inflammatory disease that affects the veins, nerves, and small- and medium-sized arteries of the distal lower and upper extremities.

* Buerger is a disease of young men who consume tobacco. Old age, however, does not rule out the disease.

* If the diagnostic criteria for Buerger disease are met, the diagnosis is confirmed by the typical histopathological and angiographic findings.