Polyarteritis nodosa
Encyclopedia
Polyarteritis nodosa is a vasculitis
of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul
disease or Kussmaul-Maier
disease. Infantile polyarteritis nodosa is a type of PAN restricted to infants.
Polyarteritis nodosa is more common in people with hepatitis B infection.
Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.
Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal failure.
Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis
).
A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs known as the 1990 ACR (American College of Rheumatology) criteria:
It should be underlined that the 1990 ACR criteria were designed for classification purposes only. Nevertheless their good discriminatory performances, indicated by the initial ACR analysis, suggested their potential usefulness for diagnostic purposes also. Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminant abilities on the prevalence of the various vasculitides in the analyzed populations. Recently an original study, combining the analysis of more than 100 items used to describe patients characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively PAN discriminating items to be used a screening tool for PAN diagnosis in patients suspected of systemic vasculitis.
and cyclophosphamide
. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal hemorrhage, infection, myocardial infarction and/or renal failure.
In case of remission, approximately 60% experience relapse within 5 years. In cases caused by hepatitis B virus, however, recurrence rate is only approximately 6%.
Vasculitis
Vasculitis refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis...
of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul
Adolph Kussmaul
Adolph Kussmaul was a German physician and a leading clinician of his time. He was born as the son and grandson of physicians at Graben near Karlsruhe and studied at Heidelberg. He entered the army after graduation and spent two years as an army surgeon...
disease or Kussmaul-Maier
Rudolf Robert Maier
Rudolf Robert Maier was a German pathologist who was a native of Freiburg im Breisgau.He studied medicine at the University of Freiburg, where one of his instructors was orthopedist Louis Georg Friedrich Stromeyer...
disease. Infantile polyarteritis nodosa is a type of PAN restricted to infants.
Causes and risk factors
Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry blood from the heart to organs and tissues. A minority of people (about 30%) diagnosed have an active Hepatitis B infection, and men are also twice as likely to get the disease as women.Incidence
The condition affects adults more frequently than children and males more frequently than females. Most cases occur between the ages of 30 and 49. It damages the tissues supplied by the affected arteries because they don't receive enough oxygen and nourishment without a proper blood supply.Polyarteritis nodosa is more common in people with hepatitis B infection.
Symptoms
In this disease, symptoms result from ischaemic damage to affected organs, often the skin, heart, kidneys, and nervous system.Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.
Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal failure.
Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis
Pericarditis
Pericarditis is an inflammation of the pericardium . A characteristic chest pain is often present.The causes of pericarditis are varied, including viral infections of the pericardium, idiopathic causes, uremic pericarditis, bacterial infections of the precardium Pericarditis is an inflammation of...
).
- FatigueFatigue (physical)Fatigue is a state of awareness describing a range of afflictions, usually associated with physical and/or mental weakness, though varying from a general state of lethargy to a specific work-induced burning sensation within one's muscles...
- WeaknessWeaknessWeakness is a symptom represented, medically, by a number of different conditions, including: lack of muscle strength, malaise, dizziness, or fatigue. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a...
- FeverFeverFever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
- Abdominal painAbdominal painAbdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...
- Decreased appetite
- Unintentional weight lossWeight lossWeight loss, in the context of medicine, health or physical fitness, is a reduction of the total body mass, due to a mean loss of fluid, body fat or adipose tissue and/or lean mass, namely bone mineral deposits, muscle, tendon and other connective tissue...
- Muscle aches
- Joint aches
Diagnosis
There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based upon the physical examination and a few laboratory studies that help to confirm the diagnosis:- CBCComplete blood countA complete blood count , also known as full blood count or full blood exam or blood panel, is a test panel requested by a doctor or other medical professional that gives information about the cells in a patient's blood...
(may demonstrate an elevated white blood count) - ESRErythrocyte sedimentation rateThe erythrocyte sedimentation rate , also called a sedimentation rate or Biernacki Reaction, is the rate at which red blood cells sediment in a period of 1 hour...
(elevated) - Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCAANCAANCA Pty Ltd is an Australia company which designs and manufactures CNC grinding machines. The company was founded in 1974 by Pat Boland and Pat McCluskey in Melbourne, Australia....
) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitisMicroscopic polyangiitisMicroscopic polyangiitis is an ill-defined autoimmune disease characterized by pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.-Presentation:Because many different organ systems may be involved, a wide range of...
or leukocytoclastic angiitis. - Tissue biopsy (reveals inflammation in small arteries, called arteritisArteritisArteritis is inflammation of the walls of arteries, usually as a result of infection or auto-immune response.-Types:Some disorders have arteritis as their main feature...
) - Elevated C-reactive proteinC-reactive proteinC-reactive protein is a protein found in the blood, the levels of which rise in response to inflammation...
A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs known as the 1990 ACR (American College of Rheumatology) criteria:
- Weight loss greater than/equal to 4.5 kg.
- Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso).
- Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis).
- Muscle pain, weakness, or leg tenderness.
- Nerve disease (either single or multiple).
- Diastolic blood pressure greater than 90mmHg (high blood pressure).
- Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinineCreatinineCreatinine is a break-down product of creatine phosphate in muscle, and is usually produced at a fairly constant rate by the body...
greater than 1.5 mg/dl). - Hepatitis B virus tests positive (for surface antigen or antibody).
- Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation.
- Biopsy of tissue showing the arteritis (typically inflamed arteries). Sural nerve is a frequent location for the biopsy.
It should be underlined that the 1990 ACR criteria were designed for classification purposes only. Nevertheless their good discriminatory performances, indicated by the initial ACR analysis, suggested their potential usefulness for diagnostic purposes also. Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminant abilities on the prevalence of the various vasculitides in the analyzed populations. Recently an original study, combining the analysis of more than 100 items used to describe patients characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively PAN discriminating items to be used a screening tool for PAN diagnosis in patients suspected of systemic vasculitis.
Treatment and Prognosis
Treatment involves medications to suppress the immune system, including prednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
and cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal hemorrhage, infection, myocardial infarction and/or renal failure.
In case of remission, approximately 60% experience relapse within 5 years. In cases caused by hepatitis B virus, however, recurrence rate is only approximately 6%.
Complications
- StrokeStrokeA stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
- Kidney failure
- Heart attackMyocardial infarctionMyocardial infarction or acute myocardial infarction , commonly known as a heart attack, results from the interruption of blood supply to a part of the heart, causing heart cells to die...
- Intestinal necrosisNecrosisNecrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
and perforation