Chapter 119

Upper Extremity Arterial Disease

General Considerations

Matthew J. Alef, Allen Hamdan

Based on a chapter in the seventh edition by James M. Edwards, Sean P. Roddy and R. Clement Darling, III

Arterial disease of the upper extremity differs from that in the lower extremity. First, upper extremity ischemia is relatively rare in that it accounts for less than 5% of patients presenting for evaluation of limb ischemia.¹ In fact, a Dutch study examining the distribution of peripheral vascular disease identified only 2.3% of diabetic patients and no nondiabetic patients with abnormal Doppler waveforms of the arteries of a single arm.² In addition, a plethora of disease processes affect the arteries of the upper extremity whereas atherosclerosis is accountable for the majority of vascular disease within the lower extremity. This chapter provides an overview of upper extremity arterial diseases. Ensuing chapters will review in detail the diagnosis, natural history, and treatment of specific diseases that affect the upper extremity circulation.

Epidemiology

The examination of upper extremity arterial disease epidemiology is problematic due to the low incidence and vast number of etiologies. One exception is Raynaud’s phenomenon, which is characterized by episodic vasospastic attacks involving the digits (see Chapter 122). Among people living in cold climates, up to 20% to 30% will complain of cold-induced digital pain.³ In the United States, population-based studies suggest that the prevalence of Raynaud’s phenomenon in women is approximately 9% in the Northeast and 4% in the South. The prevalence in men is approximately 6% in the Northeast and 3% in the South.⁴^,⁵

Most of these patients have primary (idiopathic) Raynaud’s disease, which is caused by emotional stress, caffeine, or cold exposure, and will not develop severe finger ischemia. Secondary Raynaud’s phenomenon is suspected when a local or systemic disease appears to be the precipitating factor for this complex of symptoms. These patients are more likely to progress to severe occlusive lesions within the digital arteries, rest pain, and ulceration. Such diseases include scleroderma, mixed collagen vascular disease, systemic lupus erythematosus, and rheumatoid arthritis. With the exception of scleroderma, the prevalence of Raynaud’s phenomenon is quite low.⁶ In fact, in 10 years of prospective surveillance in Austria, only 11% of all Raynaud’s patients had secondary Raynaud’s.⁷ However, scleroderma, with Raynaud’s phenomenon being present in 80% to 97% of these patients, is present in approximately 50,000 patients, with an incidence of about 10 cases per million population in the United States.⁸^,⁹

Etiology and Pathogenesis

Anatomic location and etiology are the two essential ways to categorize upper extremity arterial disease. The anatomic location is subdivided by the small or large arteries, and etiology is subdivided by either occlusive disease or vasospasm.¹ The initial clinical findings of small and large artery may be similar; however, the etiologies, pathophysiologies, and treatments differ. Although the clinical appearance of occlusive and vasospastic disease may be similar, delineating between them is essential for appropriate treatment. Processes leading to upper extremity arterial disease are vast. Arterial vasospasm may occur with ergotism, vinyl chloride exposure, and idiopathic Raynaud’s phenomenon.

The pathophysiology of Raynaud’s phenomenon remains poorly understood, despite the fact that it has been more than a century since it was first described by Maurice Raynaud.¹⁰ Primary Raynaud’s phenomenon is characterized by digital artery vasospasm, which is often linked to sympathetic nervous system overactivity. However, sympathetic nerve activity recorded by microelectrodes, normal sympathetic reflex response, and incomplete therapeutic responses to surgical or medical sympathectomy suggest that sympathetic nervous system abnormalities alone are insufficient to cause Raynaud’s phenomenon (see Chapter 122).¹¹^,¹²

Small Vessel Arteriopathies

Numerous small vessel arteriopathies in the upper extremity, defined as those distal to the wrist, may lead to hand ischemia. The diseases leading to the most severe upper extremity arterial ischemia are autoimmune or connective tissue diseases such as scleroderma, rheumatoid arthritis, systemic lupus, and others.⁶ Scleroderma is the most common connective tissue disorder. It is a generalized disorder of connective tissue, the microvasculature, and the small arteries.¹³ Cutaneous involvement is almost universal, and the clinical course is characterized by progressive scarring and small vessel occlusions in the skin, gastrointestinal tract, kidneys, lungs, and heart. Scleroderma is thought to cause arterial damage via cytotoxic antibodies to the endothelium.¹⁴

Rheumatoid arthritis is a chronic inflammatory joint disease; a subgroup of patients with rheumatoid arthritis have extra-articular involvement of the skin, eyes, lungs, spleen, and arteries.¹⁵ Sjögren’s syndrome is characterized by dry eyes and mouth and may be primary or secondary to another connective tissue disease. Sjögren’s syndrome; this syndrome may be associated with small vessel arteriopathy, which can then be subdivided into acute necrotizing, leukocytoclastic, and lymphocytic vasculitis.¹⁶ Systemic lupus erythematosus is an immune complex–mediated disease that causes fevers, arthralgias, skin rashes, nephritis, and Raynaud’s phenomenon.

Buerger’s disease (thromboangiitis obliterans) describes a clinical syndrome characterized by segmental thrombotic occlusions of the small- and medium-sized arteries (see Chapter 79).¹⁷^,¹⁸ Although the lower extremities are more often involved, approximately 50% of patients also have upper extremity involvement with subsequent digital ischemia. Smoking tobacco or marijuana is a key risk factor.¹⁹ Hypersensitivity angiitis describes patients that present with acute onset of severe digital ischemia but have no demonstrable underlying abnormality.²⁰ These acute episodes tend to be isolated, generally not recurring, and fortunately their clinical course is progressive improvement. Frostbite freeze injury of the small vessels of the digits results in Raynaud’s phenomenon. Mild cases usually result in vasospastic disease, whereas severe frostbite may result in occlusive disease of the digital arteries.

Raynaud’s phenomenon resulting from long-term use of vibrating tools was first reported over a century ago.²¹^,²² Initially these patients were stonecutters, but other groups of laborers in similar professions have subsequently presented. Shipyard welders or grinders, lumberjacks, and most recently, windshield replacement technicians in the auto glass industry have all presented with this syndrome.²³ This condition has been termed hand-arm vibration syndrome (HAVS) or vibration-induced white finger. Patients with HAVS will first have Raynaud’s phenomenon, but in the late stage, usually after decades of using vibrating tools, digital artery occlusive disease is seen (see Chapter 123).²⁴^,²⁵

Large Vessel Arteriopathies

The most common large vessel arteriopathy in the upper extremity is atherosclerosis. Arterial occlusive disease risk factors in the upper extremity are the same as those for arterial disease elsewhere in the body. Tobacco is the most significant risk factor, with hyperlipidemia, hypertension, diabetes, male gender, and age also being important (see Section 3: Atherosclerotic Risk Factors—Chapters 26 to 30). Atherosclerosis may cause upper extremity ischemia through a variety of mechanisms. The most common is occlusive disease, with involvement of the origin of the left subclavian artery being the most common location in patients with significant atherosclerosis. Aneurysms of the upper extremity arteries are rare but can occur in the subclavian or axillary arteries. Patients with diabetes or renal failure, or both (particularly those undergoing dialysis) may have accelerated atherosclerosis and not infrequently have involvement of both large and small arteries of the upper extremity that can lead to significant hand ischemia.²⁶

Large and small artery disease may also occur in arterial thoracic outlet syndrome. Chronic trauma from the thoracic outlet may result in subclavian artery stenosis, which is sometimes accompanied by digital ischemia due to embolic phenomenon (see Chapter 127).

Fibromuscular disease typically affects the renal arteries in young women. However, rare cases of fibromuscular dysplasia involving the upper extremity arteries have been described.²⁷ Takayasu’s arteritis is a chronic vasculitis mainly involving the aorta and its branches (see Chapter 80). Upper extremity ischemia may result in severe disease within the subclavian arteries.²⁸ Giant cell arteritis is a spontaneous vasculitic syndrome specifically involving the walls of medium and large arteries. Although this syndrome is most frequently recognized with symptomatic involvement of the temporal arteries, upper extremity arteries are occasionally involved (see Chapter 78).²⁹ Embolic occlusion from atrial fibrillation or other sources is classically seen in the brachial artery before the bifurcation of the radial and ulnar arteries.

Upper extremity ischemia may also occur immediately after placement of dialysis access. Distal hypoperfusion ischemic syndrome, commonly referred to as “steal” after dialysis access placement, occurs in 5% to 10% of cases when the brachial artery is used.³⁰ It is typically seen in elderly women with diabetes and may carry severe morbidity, including tissue or limb loss if not recognized and treated (see Chapter 77).

Iatrogenic injury to the radial and brachial arteries has increased in conjunction with the increasing incidence of coronary and peripheral catheterization access from these upper extremity arteries. Significant brachial access complications were initially reported to be as high as 17% but this percentage has since declined to approximately 1% to 5%.³¹^–³³ Because of the proximity of the median nerve and confined space from the covering fascia, hematomas must be monitored carefully and evacuated if symptomatic. The radial has become the access site of choice for coronary catheterization. Occlusion of the radial artery following these procedures is 3% to 10%, depending on the series.³³^,³⁴ An Allen test should be performed before all radial access procedures because an intact ulnar artery and palmer arch allow collateral flow to the hand. Thrombosis, pseudoaneurysms, and occlusions can all occur following catheter instrumentation. A number of less commonly seen causes of upper extremity arterial ischemia are possible, such as malignancy and myeloproliferative disorders.³⁵^–³⁷ Finally, penetrating and blunt injury are important considerations when viewing upper extremity ischemia (see Chapter 159).

Box 119-1 lists the causes of hand ischemia, and Table 119-1 details the portion of the arterial system affected by these etiologies. Nine disease processes from this table are discussed in other chapters, including Chapter 5: Atherosclerosis, Chapter 77: Hemodialysis Access: Nonthrombotic Complications, Chapter 78: Vasculitis and Other Arteriopathies, Chapter 79: Thromboangiitis Obliterans, Chapter 80: Takayasu’s Disease, Chapter 123: Occupational Vascular Problems, Chapter 122: Raynaud’s Syndrome, Chapter 127: Thoracic Outlet Syndrome: Arterial, and Chapter 140: Upper Extremity Aneurysms.