Polyarteritis nodosa

Polyarteritis nodosa, also known as Panarteritis nodosa,^[1] Periarteritis nodosa,^[1]), Kussmaul disease or Kussmaul-Maier disease.,^[2] is a vasculitis of medium and small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Infantile polyarteritis nodosa is a type of PAN restricted to infants.^[3] In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary,^[4] therefore making "rosary sign" an important diagnostic feature of the vasculitis.^[5]

With treatment, 5-year survival is 80%; without treatment, 5-year survival is 13%. Death is often a consequence of renal failure, myocardial infarction, or stroke.^[6]

Signs and symptoms

Polyarteritis nodosa: Macroscopic specimen of the heart with abundant adipose tissue and nodular thickened coronary vessels.

In this disease, symptoms result from ischemic 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.^{[citation needed]} Palpable purpura and livedo reticularis can occur in some patients.

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy). Central nervous system involvement may cause strokes or seizures.

Kidney involvement can produce varying degrees of renal failure, such as hypertension, edema, oliguria and uremia.^{[citation needed]}

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^{[citation needed]}

Complications

• Stroke^{[citation needed]}
• Kidney failure^{[citation needed]}
• Heart attack^{[citation needed]}
• Intestinal necrosis and perforation^{[citation needed]}

Causes

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. ^{[citation needed]}

Diagnosis

Microscopic findings in polyarteritis nodosa: nodular thickened and branched arteries from small bowel mucosa (Fig. 1). Flexor digitorum superficialis artery with early diffuse nuclear proliferation (X155; Fig. 2). Nodular thickened and aneurysmal expanded artery: (a) tunica intima, (b) tunica media, (c) tunica adventitia, (d) newly formed connective tissue and fat (Fig. 3; X155)

There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help 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

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)^[7] 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 creatinine greater than 1.5 mg/dl).
• Hepatitis B or Hepatitis C 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).^[8] Sural nerve is a frequent location for the biopsy.

In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an diagnostic feature of the vasculitis.

In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary,^[4] therefore making "rosary sign" an important diagnostic feature of the vasculitis.^[5]

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 discriminative 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 as a screening tool for PAN diagnosis in patients suspected of systemic vasculitis.^[9]

Treatment

Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. In some cases methotrexate or leflunomide may be helpful.^[10] Some patients have also noticed a remission phase when a 4 dose infusion of rituximab is used before the leflunomide treatment is begun. 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.^[11]

In case of remission, approximately 60% experience relapse within five years.^[12] In cases caused by hepatitis B virus, however, recurrence rate is only approximately 6%.^[13]

Epidemiology

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. ^{[citation needed]}

Polyarteritis nodosa is more common in people with hepatitis B infection.^[14]

See also

• List of cutaneous conditions

References

External links

• -1408892916 at GPnotebook
• Polyarteritis Nodosa on ThirdAge.com