Polyarteritis Nodosa

Description:Polyarteritis nodosa (or periarteritis nodosa) is a vasculitis of medium-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Meier disease

The first description of this disease dates back to 1866 when Kussmaul and Maier identified a condition that consisted of “focal, inflammatory, arterial nodules”. They termed this disorder “periarteritis nodosa” because of the inflammation they observed around the blood vessel wall. The name was changed to polyarteritis nodosa (PAN) to underscore the fact that inflammation throughout the entire arterial wall – not just around the wall – is a major disease feature. Polyarteritis nodosa is sometimes termed “systemic necrotizing vasculitis”, but this term is non-specific as other forms of vasculitis also have systemic and necrotizing features.

Most cases of PAN occur in the 4th or 5th decade, although it can occur at any age. Men are twice as likely to be affected than women. A minority of patients with PAN have an active hepatitis B infection. In the rest of the cases, the cause(s) is presently unknown, and the disease is said to be “idiopathic” in nature.

Causes and risk factors:
Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues. It occurs when certain immune cells attack the affected arteries. One hypothesis is that this condition is caused by antibodies against HBV, via a type III hypersensitivity reaction.

Hepatitis B causes a minority of cases of PAN. With the availability of hepatitis B vaccine now, cases of PAN caused by hepatitis B are now rare in the developed world. It is possible that other infections contribute to other cases of PAN, but links between other infections and this disease remain conjectural at the present time.

Incidence:
The condition affects adults more frequently than children. 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 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).

Fatigue
Weakness
Fever
Abdominal pain
Decreased appetite
Unintentional weight loss
Muscle aches
Joint aches


    Nerve:
    .
    * Peripheral neuropathies are very common (50 to 70%). This includes tingling, numbness and/or pain in the hands, arms, feet, and legs.
    * Central nervous system (CNS) lesions may occur 2 to 3 years after the onset of PAN and may lead to cognitive dysfunction, decreased alertness, seizures and neurologic deficits.

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Skin:
*Skin abnormalities are very common in PAN and may include purpura, livedo reticularis, ulcers, nodules or gangrene.
*Skin involvement occurs most often on the legs and is very painful.

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    Kidney:
    * Renal artery vasculitis may lead to protein in the urine, impaired kidney function, and hypertension.
    * Small percentage of patients go on to require dialysis.
    .
    Gastrointestinal Tract:
    * Abdominal pain, gastrointestinal bleeding (occasionally is mistaken for inflammatory bowel disease)
    * Hemorrhage, bowel infarction, and perforation are rare, but very serious
    Heart:* Clinical involvement of the heart does not usually cause symptoms.
    * However, some patients develop myocardial infarctions (heart attacks) or congestive heart failure.

    Eye:

    * Scleritis or inflammation in the sclera (white part of the eye)

    Genitals:

    * Testicular infarction

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    Signs and tests for 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:

    *CBC (may demonstrate an elevated white blood count)

    *ESR (elevated)

    *Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) – not associated with “classic” polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis.

    *Tissue biopsy (reveals inflammation in small arteries, called arteritis)

    *Elevated c reactive protein

    Routine laboratory tests may provide important clues to PAN, but there is no single blood test that is diagnostic of this disease. Most patients with PAN have elevated ESRs. Proteinuria (protein in the urine) is common among those with kidney involvement.

    A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs:

    1. Weight loss greater than/equal to 4 kg.
    2. Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso).
    3. Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis).
    4. Muscle pain, weakness, or leg tenderness.
    5. Nerve disease (either single or multiple).
    6. Diastolic blood pressure greater than 90mmHg (high blood pressure).
    7. Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine greater than 1.5 mg/dl).
    8. Hepatitis B virus tests positive (for surface antigen or antibody).
    9. Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation.
    10. Biopsy of tissue showing the arteritis (typically inflamed arteries).

    Treatment and Prognosis:
    Treatment of PAN has improved dramatically in the past couple of decades. Before the availability of effective therapy, untreated PAN was usually fatal within weeks to months. Most deaths occurred as a result of kidney failure, heart or gastrointestinal complications. However, effective treatment is now available for PAN. After diagnosis, patients are treated with high doses of corticosteroids. Other immunosuppressive drugs are also added for patients who are especially ill. In most cases of PAN now, if diagnosed early enough the disease can be controlled, and often cured.

    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. Without treatment, the outlook is poor.

    Potassium citrate was able to prevent arterial lesions in sodium chloride loaded hypertensive dahl rats without lowering the blood pressure. Potassium chloride had a somewhat lesser protective affect.

    Complications:

    *Stroke

    *Kidney failure

    *Heart attack

    *Intestinal necrosis and perforation

    Prevention:
    This disease cannot be currently prevented, but early treatment can prevent some damage and symptoms.

    Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

    Resources:
    http://en.wikipedia.org/wiki/Polyarteritis_nodosa
    http://vasculitis.med.jhu.edu/typesof/polyarteritis.html

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