eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases
Polyarteritis Nodosa
Updated: Sep 28, 2006
Introduction
Background
Polyarteritis nodosa (PAN) is a rare systemic vasculitis necrotizing vasculitis characterized by necrotizing inflammation of small- and medium-sized arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules. PAN was first described by Kussmaul and Maier in 1866. In 1948, the term microscopic polyarteritis was introduced into the literature by Davson. Subsequently, the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitis held in 1992 officially adopted the term polyarteritis nodosa.
Clinically, PAN affects multiple systems, including renal, musculoskeletal, nervous, gastrointestinal, integumentary, cardiac, and genitourinary. However, the lungs are usually spared with PAN. Signs and symptoms of this disease are primarily attributable to diffuse vascular inflammation and ischemia of affected organs.
Pathophysiology
Characteristic of PAN is a necrotizing inflammation of small and medium-sized muscular arteries. Lesions are segmental and tend to involve bifurcations and branches of arteries. Involvement of venules is not seen with classic PAN.
Acute stage: Polymorphonuclear neutrophils infiltrate all layers of the vessel wall.
Subacute stage: Infiltration of mononuclear cells becomes more prominent.
Chronic stage: Fibrinoid necrosis of the vessels causes thrombosis and tissue infarction. Aneurysmal dilatations of the involved arteries, as large as 1 cm in size, are characteristic findings of PAN.
Kidney lesions show predominant arteritis without glomerulonephritis; however, in patients with severe hypertension, glomerulosclerosis may be superimposed with glomerulonephritis. Pulmonary arteries are not involved and bronchial artery involvement is uncommon.
Frequency
United States
Incidence is about 3-4.5 cases per 100,000 population per year.
International
Incidence is in the range of 1.8-6.3 cases per 100,000 per year in most populations; however, it is as high as 7.7 cases per 100,000 population in certain groups in which hepatitis B is endemic.
Mortality/Morbidity
When left untreated, the 5-year survival rate of PAN is 13%. Nearly half of patients die within the first 3 months of onset. Corticosteroid treatment improves the 5-year survival rate to 50-60%. When the steroid is combined with other immunosuppressants, the 5-year survival rate may increase to greater than 80%.- Cause of death: Renal failure is the most common cause, followed by CNS disease. Other causes include GI complications (bowel infarcts and perforations) and cardiovascular pathology.
- Intractable hypertension contributes to morbidity and mortality rates.
- Fever, weight loss, and malaise are present in 50% of patients; renal failure and hypertension in 60%; arthritis, arthralgia, and myalgia in 64%; peripheral neuropathy and mononeuritis multiplex in 51%.
Race
No racial predisposition is known.
Sex
Men are affected more frequently than women (male-to-female ratio 1.6:1).
Age
Onset is usually in adults aged 40-60 years.
Clinical
History
Patients typically present with nonspecific signs and symptoms such as fever, weakness, headache, abdominal pain, weight loss, and malaise. PAN may involve any organ system.
- Renal system: About 60% of patients with PAN have renal involvement. Ischemic changes in the glomeruli can cause renal failure or hypertension.
- Musculoskeletal system: Symptoms manifest as arthritis, arthralgia, or myalgia.
- Central nervous system: Transient symptoms of cerebral ischemia, including typical spells of transient monocular blindness, are the most commonly presenting neurologic deficit of polyarteritis nodosa. Cerebral arteritis usually presents late in the course of the disease, usually in the second to third year of the vasculitis. Cerebral arteritis may cause arterial thrombosis with cerebral ischemia or intraparenchymal or subarachnoid hemorrhage. Global CNS dysfunction with encephalopathy and seizures results from metabolic derangements secondary to multiple organ failure. Acute or subacute myelopathy with paraparesis can occur at any cord level. Myelopathy may result, although rarely, from cord compression by an extramedullary hematoma secondary to a ruptured spinal aneurysm.
- Peripheral nervous system: Peripheral neuropathy develops in as many as 60% of patients. Vasculitic neuropathy is often asymmetric and presents as (1) mononeuritis multiplex, (2) distal polyneuropathy, or (3) cutaneous neuropathy. It often takes the form of mononeuritis multiplex or of a pure motor, sensory, or mixed sensorimotor polyneuropathy.
- GI tract: GI involvement presents as specific and nonspecific symptoms and signs such as abdominal pain, nausea and vomiting, bleeding, bowel infarction and perforation, cholecystitis, hepatic infarction, or pancreatic infarction.
- Skin: About 40% of patients manifest dermatologic symptoms and signs including rash, purpura, nodules, cutaneous infarcts, livido reticularis, and Raynaud phenomenon.
- Cardiovascular system: Cardiac disease affects 35% of patients with PAN. The symptoms and signs include congestive heart failure, myocardial infarction, and pericarditis.
- Genitourinary system: Patients may develop pain over the testicular or ovarian area.
Physical
Since PAN is a systemic disease, a complete systemic and neurologic examination is essential for diagnosis.
- Systemic examination
- Skin rash or nodules
- Raynaud phenomenon
- Congestive heart failure
- Pericarditis
- Bowel infarction
- Cholecystitis
- Arthritis
- Hypertension
- Renal failure
- Neurologic examination
- Peripheral neuropathy (sensorimotor)
- Encephalopathy
- Sensory deficit with cord level
- Focal weakness or hemiparesis
- Occasionally, a "locked-in syndrome" develops in a patient with PAN, caused by infarction of the basis pontis following occlusion of the proximal and middle segments of the basilar artery. The patient is quadriplegic and unable to move the lower face or speak, but the sparing of the pontine tegmentum leaves the patient conscious, with preserved vertical eye movements and blinking.
Causes
- Approximately 30% patients with PAN are positive for hepatitis B surface antigen.
- In these patients, hepatitis B antigen and circulating hepatitis B antigen-antibody aggregate in the serum and in vascular lesions.
- This finding suggests that PAN results from complexes of antibodies and exogenous antigen, such as hepatitis B antigen.
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References
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Further Reading
Keywords
PAN, microscopic polyangiitis, systemic necrotizing vasculitis, arteritis nodosa, Kussmaul disease, Kussmaul's disease, periarteritis nodosa, microscopic polyarteritis, polyarteritis nodosa
