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Polyarteritis nodosa

Periarteritis nodosa; PAN; Systemic necrotizing vasculitis

 

Polyarteritis nodosa is a serious blood vessel disease. The small and medium-sized arteries become swollen and damaged.

Causes

 

Arteries are the blood vessels that carry oxygen-rich blood to organs and tissues. The cause of polyarteritis nodosa is unknown. The condition occurs when certain immune cells attack the affected arteries. The tissues that are fed by the affected arteries DO NOT get the oxygen and nourishment they need. Damage occurs as a result.

More adults than children get this disease.

People with active hepatitis B or hepatitis C may develop this disease.

 

Symptoms

 

Symptoms are caused by damage to affected organs. The skin, joints, muscle, gastrointestinal tract, heart, kidneys, and nervous system are often affected.

Symptoms include:

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

If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures.

 

Exams and Tests

 

No specific lab tests are available to diagnose polyarteritis nodosa. You will have a physical exam.

Lab tests that can help make the diagnosis include:

  • Complete blood count (CBC), creatinine, tests for hepatitis B and C, and urinalysis.
  • Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP).
  • Serum protein electrophoresis, cryoglobulins.
  • Serum complement levels.
  • Arteriogram tissue biopsy .
  • Other blood tests may be done to rule out similar conditions, such as systemic lupus erythematosus (ANA) or granulomatosis with polyangiitis (ANCA).
  • Test for HIV.

 

Treatment

 

Treatment involves medicines to suppress inflammation and the immune system. These may include steroids, such as prednisone. Similar medicines, such as azathioprine, methotrexate or mycophenolate that allow for reducing the dose of steroids are often used as well. Cyclophosphamide is used in severe cases.

For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and antiviral medicines.

 

Outlook (Prognosis)

 

Current treatments with steroids and other drugs that suppress the immune system (such as azathioprine or cyclophosphamide) can improve symptoms and the chance of long-term survival.

The most serious complications most often involve the kidneys and gastrointestinal tract.

Without treatment, the outlook is poor.

 

Possible Complications

 

Complications may include:

  • Heart attack
  • Intestinal necrosis and perforation
  • Kidney failure
  • Stroke

 

When to Contact a Medical Professional

 

Call your health care provider if you develop symptoms of this disorder. Early diagnosis and treatment may improve the chance of a good outcome.

 

Prevention

 

There is no known prevention. However, early treatment can prevent some damage and symptoms.

 

 

Open References

References

Luqmani R. Polyarteritis nodosa and related disorders. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley's Textbook of Rheumatology . 9th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 90.

Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum . 2010;62(2):616-626. PMID: 20112401 www.ncbi.nlm.nih.gov/pubmed/20112401 .

Ribi C, Cohen P, Pagnoux C, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum . 2010;62(4):1186-1197. PMID: 20131268 www.ncbi.nlm.nih.gov/pubmed/20131268 .

Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 270.

Warrington KJ, Cooper LT. Vasculitis and other uncommon arteriopathies. In: Cronenwett JL, Johnston KW, eds. Rutherford's Vascular Surgery . 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 78.

 
  • Polyarteritis, microscopic on the shin

    Polyarteritis, microscopic on the shin - illustration

    This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).

    Polyarteritis, microscopic on the shin

    illustration

  • Microscopic polyarteritis 2

    Microscopic polyarteritis 2 - illustration

    Polyarteritis nodosa, seen here on a lower extremity, produces painful, erythematous nodules. Arteries in the deep dermis and upper fat show neutrophilic infiltration.

    Microscopic polyarteritis 2

    illustration

  • Circulatory system

    Circulatory system - illustration

    Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.

    Circulatory system

    illustration

    • Polyarteritis, microscopic on the shin

      Polyarteritis, microscopic on the shin - illustration

      This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).

      Polyarteritis, microscopic on the shin

      illustration

    • Microscopic polyarteritis 2

      Microscopic polyarteritis 2 - illustration

      Polyarteritis nodosa, seen here on a lower extremity, produces painful, erythematous nodules. Arteries in the deep dermis and upper fat show neutrophilic infiltration.

      Microscopic polyarteritis 2

      illustration

    • Circulatory system

      Circulatory system - illustration

      Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.

      Circulatory system

      illustration

    Tests for Polyarteritis nodosa

     
       

      Review Date: 5/14/2016

      Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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