A major hallmark of Buerger disease is its confinement to the extremities. The initial symptoms may be nonspecific pains in the calf, foot, or toes. The progression of thrombosis and vasculitis can lead to horrific pain in the digits and limbs and ultimately to gangrene and tissue loss, through either autoamputation or elective amputation. For unknown reasons, however, other vascular beds (eg, the cardiac, pulmonary, renal, and mesenteric vasculature) are nearly always spared in Buerger disease.
Although Buerger disease has a predilection for the feet and toes, the hands and fingers may also be affected prominently. More than 60% of patients have abnormal Allen tests, indicating compromise of circulation to the hand; many demonstrate obliterations of the radial or ulnar artery pulses on physical examination. In contrast to atherosclerosis, which is a disease of the proximal vasculature, Buerger disease is characterized by inflammation and thrombosis of medium-sized, distal blood vessels (both arteries and veins), most intense at the levels of the ankles and wrists.
The earliest lesion may be a superficial thrombophlebitis. This complaint is often disregarded by the patient or misdiagnosed as deep varicosities. Histologic examination of these lesions reveals an acute thrombophlebitis with marked perivascular infiltration. This herald lesion is then followed by progressive occlusion of the deeper veins and arteries, leading the patient to seek medical attention. Patients with Buerger disease may have splinter hemorrhages, arousing suspicions of infective endocarditis. Most cutaneous features of disease are those of a process involving the medium-sized vessels exclusively (purpura, for example, a manifestation of small-vessel disease, is absent).
Gangrene occurs in the most distal tissues, ie, the toes and fingers, first (Figure 41–1). If the process remains undiagnosed or if the patient continues to smoke even after the diagnosis, larger portions of the extremities become compromised. In advanced cases, the major arterial supplies to the hands and feet may become occluded, leading to coolness and pain of the entire distal extremity, necessitating amputation (Figure 41–2).
Digital ischemia with gangrene in Buerger disease.
As a consequence of failure to stop smoking, this patient required multiple amputations, including fingers on both hands and bilateral below-the-knee amputations.
Early in the disease, nonspecific pains in the calf, foot, or toes may recall a primary neuropathic process. These sensory symptoms may result from thickening of the tissues immediately surrounding the veins and arteries, leading to connective tissue proliferation around the nerve bundles that are intimately connected with the vasculature. True vasculitic neuropathy, however, does not occur in Buerger disease.
Gastrointestinal Tract and Other Organs
Extremely rare cases of Buerger disease involving the gastrointestinal tract and central nervous system have been reported.
There is no single diagnostic test for Buerger disease. The demonstration of “corkscrew collaterals” (Figure 41–3) on angiography is highly characteristic but not pathognomonic. Such vessels may also be observed in polyarteritis nodosa and other forms of medium-vessel vasculitis. Laboratory and radiologic investigations are important in Buerger disease, both to identify the typical vascular lesions and to exclude conditions that require other approaches to management. Table 41–2 lists the results of routine laboratory tests and specialized assays that are done to rule out disorders masquerading as Buerger disease.
Angiographic findings in Buerger disease. A: Attenuation of the anterior tibial artery in the mid-calf. This artery forms a collateral at the site of occlusion with the peroneal artery. The posterior tibial artery is occluded superiorly. B: Abrupt arterial cut-offs several centimeters above the ankle, with minimal blood flow distal to the cut-offs.
Table 41–2. Laboratory and Radiologic Evaluation in Possible Buerger Disease. ||Download (.pdf)
Table 41–2. Laboratory and Radiologic Evaluation in Possible Buerger Disease.
|Complete blood cell count||Normal. Mild elevations of the white blood cell and platelet count would not be unexpected.|
|Renal and hepatic function||Normal|
|Urinalysis with microscopy||Normal|
|Erythrocyte sedimentation rate (ESR)/C-reactive protein||Mild to moderate elevations in patients with severe digital ischemia. Dramatically elevated acute phase reactants (eg, an ESR >100 mm/h) unusual.|
|Hepatitis B and C serologies||Negative|
|Antiphospholipid antibodies||Negative rapid plasma reagin and anticardiolipin antibody assays. Normal Russell viper venom time (for lupus anticoagulant).|
|Echocardiography (or TEE)||No cardiac valvular vegetations. Normal aortic root.|
|Angiography||Corkscrew collaterals (see Figure 41–3). Abrupt cutoffs of medium-sized arteries at levels of the ankles and wrists, and often higher. Segmental areas of involvement, with diseased regions interspersed with normal-appearing arterial stretches.|
The erythrocyte sedimentation rate and C-reactive protein levels are generally lower than observed in many other types of diffuse systemic vasculitis, but most patients have at least moderate elevations of these acute phase reactants. Routine hematology, serum chemistry, and urinalysis studies are normal in Buerger disease; abnormalities in these tests suggest other diagnoses. Markers of hypercoagulable states that may be associated with widespread arterial thromboses, eg, antiphospholipid antibodies, should be investigated.
Echocardiography (possibly including a transesophageal study) should examine the heart valves and aortic root. Comprehensive angiographic studies that define the vasculature of the extremities, proximal aorta, gastrointestinal tract, and renal arteries should be considered. Such studies are critical in identifying vascular involvement typical of Buerger disease and excluding atheroembolic sources as well as findings more typical of other vasculitides (eg, microaneurysms). The arterial involvement in Buerger disease is highly segmental, with abrupt vascular occlusions interspersed with regions of vessels that appear angiographically normal (Figure 41–3). In advanced cases, the thready appearance of vessels distal to the wrists and ankles may resemble a disorganized spider web. The most commonly involved vessels are the digital arteries of the fingers and toes as well as the palmar, plantar, tibial, peroneal, radial, and ulnar vessels.