| Polyarteritis nodosa | |
|---|---|
| Classification and external resources | |
| ICD-10 | M30.0 |
| ICD-9 | 446.0 |
| DiseasesDB | 10220 |
| MedlinePlus | 001438 |
| MeSH | D010488 |
Polyarteritis nodosa (also known as "Panarteritis nodosa,"[1] and "Periarteritis nodosa"[1]) 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.[2] Infantile polyarteritis nodosa is a type of PAN restricted to infants.[3]
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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.
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.[4]
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).
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:
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)[5] 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.[7]
Treatment involves medications to suppress the immune system, including prednisone 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.[8]
In case of remission, approximately 60% experience relapse within 5 years.[9] In cases caused by hepatitis B virus, however, recurrence rate is only approximately 6%.[10]
This disease cannot be currently prevented, but early treatment can prevent some damage and symptoms.
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