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Polyarteritis nodosa, also known as panarteritis nodosa, periarteritis nodosa,), Kussmaul disease or Kussmaul-Maier disease, is a vasculitis of medium and small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Infantile polyarteritis nodosa is restricted to infants. In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an important diagnostic feature of the vasculitis.
With treatment, five-year survival is 80%; without treatment, five-year survival is 13%. Death is often a consequence of renal failure, myocardial infarction, or stroke.
Signs and symptoms
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. 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. Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis).
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.
No specific lab tests exist 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 three of the 10 signs known as the 1990 American College of Rheumatology (ACR) 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 90 mmHg (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): The 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 important diagnostic feature of the vasculitis. 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 discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis.
Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. In some cases, methotrexate or leflunomide may be helpful. Some patients have also noticed a remission phase when a four-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.
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 do not receive enough oxygen and nourishment without a proper blood supply. Polyarteritis nodosa is more common in people with hepatitis B infection.
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