Thromboangiitis obliterans involving the temporal artery
Article first published online: 31 MAY 2011
Copyright © 2011 by the American College of Rheumatology
Arthritis Care & Research
Volume 63, Issue 6, pages 918–920, June 2011
How to Cite
Prokesch, B., Law, K. and Conn, D. L. (2011), Thromboangiitis obliterans involving the temporal artery. Arthritis Care Res, 63: 918–920. doi: 10.1002/acr.20457
- Issue published online: 31 MAY 2011
- Article first published online: 31 MAY 2011
- Accepted manuscript online: 25 FEB 2011 11:15AM EST
- Manuscript Accepted: 7 FEB 2011
- Manuscript Received: 18 AUG 2010
Thromboangiitis obliterans (TO) is a nonatherosclerotic, inflammatory segmental vascular disease typically seen in young male smokers, involving primarily small- and medium-sized vessels of the distal extremities (1). The first case of TO was described by von Winiwarter in 1879, and, in 1908, Leo Buerger explained the pathologic findings in 11 amputated extremities. Although more than 100 years have passed since the vasculitis was recognized and the pathology fully explained, the cause of TO remains unclear. Nonetheless, the use of or exposure to tobacco has been found to be essential to the onset and progression of the disease. Diagnosis is often one of exclusion, but can be made based on bilateral extremity digital necrosis in a heavy smoker and in the absence of scleroderma. Typically, echocardiography is done to rule out a proximal source of emboli, and angiography is done to look for segmental occlusive lesions, or “corkscrew collaterals”; however, these findings are not pathognomonic for TO and may also be seen in atherosclerosis. The physical examination findings, along with the clinical history of tobacco use, an examination with negative results for other sources of thrombi, and a suggestive angiographic picture allow for a diagnosis of TO. Rarely is a biopsy performed. In the case described below, TO was confirmed by temporal artery biopsy showing the classic pathologic features in a patient presenting with typical signs and symptoms of the disease.
A 50-year-old man who was human immunodeficiency virus (HIV)–positive presented to the clinic with 3 weeks of progressive burning pain and numbness of his fingers. He stated that 3 fingertips on each hand began to feel as though they were “on fire,” then they started to turn black and cold. He reported no fevers, headaches, rashes, or vision changes. He had also noticed a small, painless bulging near his left temple that had developed 1 month prior to presentation.
The patient's past medical history was significant for HIV, with a recent CD4 cell count of 18. He had recently started antiretroviral therapy. He did not have any other medical problems, such as hypertension, diabetes mellitus, or hyperlipidemia, and had no family history of cardiovascular or autoimmune diseases. The patient had a 15-pack per year history of cigarette smoking.
A physical examination revealed cool, tender, and blackened fingertips with distal peeling of the deadened skin and more proximal areas of duskiness (Figure 1). A painless, nonpulsating cord-like vessel measuring ∼8 mm at the right temple was also noted. The findings of a cardiopulmonary examination were within normal limits, including symmetric pulses in all 4 extremities without bruit.
The laboratory data revealed an erythrocyte sedimentation rate (ESR) of 91 mm/hour and a C-reactive protein level of 1.0 mg/liter. Antinuclear antibody was negative. Given the clinical and laboratory findings, TO and giant cell arteritis (GCA) were considered. The patient's temporal artery was biopsied, the findings of which revealed an enlarged, inflamed temporal artery with an in situ thrombus. An inflammatory infiltrate including lymphocytes, histiocytes, and some giant cells was present in the intima and media adjacent to the thrombus, suggesting an underlying thromboembolic disorder with secondary adventitial inflammation consistent with TO (Figure 2). Due to his limited financial resources, the patient elected not to pursue an angiogram.
The patient was told to stop smoking and was prescribed nifedipine and aspirin. His ischemic symptoms began to improve with smoking cessation, and his affected fingers showed evidence of reperfusion at the 3-week followup. Because of his dramatic improvement after smoking cessation, no additional interventions were considered.
TO is most often seen in young male smokers, with the onset of symptoms before age 45 years. While the patient described was slightly older than the typical patient with TO, he was a male with a strong smoking history. As it did with our patient, the disease usually begins with involvement of the distal small arteries and veins, resulting in ischemia. Although TO most commonly remains a disease of small- and medium-sized arteries and veins in the extremities, there are case reports describing the disease in many other vascular beds (2–6). There are no specific laboratory tests that are indicative of TO, although, as in this patient's case, an elevated ESR can indicate active inflammation and tissue damage.
Several criteria have been proposed to assist with the diagnosis of TO. The Shionoya criteria, all 5 of which must be met for a definitive diagnosis, include smoking history, onset before age 50 years, infrapopliteal arterial occlusive lesions, upper extremity involvement of phlebitis migrans, and the absence of other atherosclerotic risk factors. When all but thrombophlebitis is present, the patient is said to have probable TO (7). The Olin criteria, described in 2000, includes smoking history, onset before age 45 years, the presence of distal extremity ischemia indicated by claudication, pain at rest, or ulceration (1). Concomitant autoimmune diseases, hypercoaguable states, and diabetes mellitus must be excluded. Echocardiography and angiography are used to exclude a proximal source of embolization and to document consistent arteriographic findings. Both criteria propose invasive studies to exclude other causes of digital ischemia; however, as in the patient presented here, a prompt response to tobacco withdrawal in the appropriate clinical setting can obviate the need for further costly evaluations in order to make a diagnosis.
In the early 1900s, Buerger first described the histopathologic findings of TO after studying the extremities of patients with TO. The affected vessels had a highly cellular and inflammatory thrombus that relatively spared the blood vessel walls, which was strikingly different from other forms of vasculitis. These findings vary according to the duration of the disease. The hallmark of the acute-phase lesion is an occlusive, extremely cellular, and inflammatory thrombus with the presence of polymorphonuclear leukocytes, microabscesses, and multinucleated giant cells at the junction of the thrombus with the vessel wall. The internal elastic lamina remains intact. The intermediate- and late-stage lesions are nonspecific, showing only varying degrees of thrombus organization, fibrosis, and a paucity of inflammatory cells (8). These findings are consistent with multiple etiologies, including various inactive vasculitides or thromboembolic disease. The temporal progression of the TO lesion has 2 important implications. First, in most cases, biopsies are not helpful in the diagnosis of TO, since once the patient seeks clinical attention for ischemia, digital necrosis has already occurred and the patient is no longer in the acute thrombotic phase of the disease. Second, when TO occurs in unusual locations, including the temporal artery, the diagnosis should be made only when the histopathologic findings are consistent with an acute-phase lesion, since intermediate- and late-stage findings in TO are nonspecific.
In this patient, biopsy results of the temporal artery revealed distinguishing features consistent with the acute phase lesion of TO. Although this location is more typical of involvement with GCA, a main distinguishing feature is that, unlike in the acute phase of TO, GCA does not have an occluding thrombus. In GCA, luminal occlusion occurs due to transmural inflammation and intimal hyperplasia, not in situ thrombus formation. Furthermore, in GCA, the internal elastic lamina is generally disrupted, and multinucleated giant cells are found throughout the intima and media, not at the junction of the thrombus and vessel wall as is seen in TO (9). The general pathologic impression in GCA is one of intense vessel wall inflammation inducing narrowing and occlusion of the lumen, instead of an inflammatory occlusive thrombus inducing secondary vessel wall changes as seen in TO.
TO involvement of the temporal artery is rare and cited only once previously in the literature. Lie and Michet describe a series of 3 cases of TO diagnosed by temporal artery biopsy (10). All cases were heavy cigarette smokers, and all received a temporal artery biopsy due to localized swelling. Two patients went on to develop digital ischemic lesions characteristic of TO.
In summary, confirmation of the diagnosis of TO via temporal artery biopsy is extremely uncommon, with only 3 previous cases noted in the literature. In patients suspected of having TO, the temporal artery should be carefully examined; if it is clinically involved, a biopsy can potentially result in a pathologic diagnosis.
Since TO is commonly a diagnosis of exclusion following multiple invasive procedures, a careful physical examination including assessment of the temporal arteries should be emphasized, and if positive, a biopsy should be considered given its relative accessibility for definitive diagnosis.
All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be submitted for publication. Dr. Law had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Study conception and design. Prokesch, Law, Conn.
Acquisition of data. Prokesch, Law.
Analysis and interpretation of data. Prokesch, Law, Conn.
- 2Involvement of the coronary artery in a patient with thromboangiitis obliterans: a case report. Heart Vessels 2002; 15: 201–3., , , .