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Polyarteritis nodosa

Periarteritis nodosa; PAN; Systemic necrotizing vasculitis

Polyarteritis nodosa is a serious inflammatory 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.

Artery cut section

Artery cut section

The structure of blood vessel walls is important in the regulation of blood flow. The tunica media contains smooth muscle fibers which contract to either dilate or constrict the size of the vessel. These small changes in the vessel lumen can greatly influence blood pressure and blood flow. Therefore, the tunica media plays an important role in maintaining blood pressure and continuous blood circulation.

Symptoms

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

Symptoms include:

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. There are a number of disorders that have features similar to polyarteritis nodosa. These are known as "mimics."

You will have a complete physical exam.

Lab tests that can help make the diagnosis and check for disease mimics include:

  • Complete blood count (CBC) with differential, creatinine, tests for hepatitis B and C, and urinalysis
  • Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
  • Serum protein electrophoresis
  • Serum complement levels
  • HIV test
  • Hepatitis B and hepatitis C tests
  • Cryoglobulins
  • Anti-phospholipid antibodies
  • Blood cultures
  • Other blood tests will be done to check for similar conditions, such as systemic lupus erythematosus (ANA) or granulomatosis with polyangiitis (ANCA)
  • Arteriogram
  • Tissue biopsy

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 medicines 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:

When to Contact a Medical Professional

Contact 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, Awisat A. Polyarteritis nodosa and related disorders. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 95.

Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 10th ed. Philadelphia, PA: Elsevier; 2023:chap 138.

Stone JH. The systemic vasculitides. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 249.

  • Artery cut section

    Artery cut section - illustration

    The structure of blood vessel walls is important in the regulation of blood flow. The tunica media contains smooth muscle fibers which contract to either dilate or constrict the size of the vessel. These small changes in the vessel lumen can greatly influence blood pressure and blood flow. Therefore, the tunica media plays an important role in maintaining blood pressure and continuous blood circulation.

    Artery cut section

    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

  • Artery cut section

    Artery cut section - illustration

    The structure of blood vessel walls is important in the regulation of blood flow. The tunica media contains smooth muscle fibers which contract to either dilate or constrict the size of the vessel. These small changes in the vessel lumen can greatly influence blood pressure and blood flow. Therefore, the tunica media plays an important role in maintaining blood pressure and continuous blood circulation.

    Artery cut section

    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

 

Review Date: 5/9/2024

Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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