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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:

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 polyarthritis nodosa. These are known as "mimics."

You will have a complete physical exam.

Lab tests that can help make the diagnosis and rule out 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, cryoglobulins
  • Serum complement levels
  • Arteriogram
  • Tissue biopsy
  • Other blood tests will be done to rule out similar conditions, such as systemic lupus erythematosus (ANA) or granulomatosis with polyangiitis (ANCA)
  • Test for HIV
  • Cryoglobulins
  • Anti-phospholipid antibodies
  • Blood cultures

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:

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.

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. 10th ed. Philadelphia, PA: Elsevier; 2017: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.

Puéchal X, Pagnoux C, Baron G, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg-Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: a randomized, controlled trial. Arthritis Rheumatol. 2017;69(11):2175-2186. PMID: 28678392 www.ncbi.nlm.nih.gov/pubmed/28678392.

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.

Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 137.

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

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  • 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 - 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

  • 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 - 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: 4/12/2018

Reviewed By: Gordon A. Starkebaum, MD, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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