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Polyarteritis nodosa

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Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.

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What is polyarteritis nodosa?

This was the first non-infectious vasculitis to be described and studied in detail,1 with Kussmaul and Maier's initial case report dating to 1866.2 The term polyarteritis nodosa (PAN) was adopted in 1992.3 The current definition of PAN was agreed at the 2012 Chapel Hill Conference:

  • PAN is necrotising arteritis of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and not associated with antineutrophil cytoplasmic antibodies (ANCAs).4

It can affect any organ but, for unknown reasons, it spares the pulmonary and glomerular arteries.5

A less severe form called cutaneous polyarteritis nodosa (CPAN) has also been described. Its features include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis.6 It is often associated with streptococcal infection. Although progress to classical PAN at a later stage has been reported, generally it is thought to be unlikely.7

How common is polyarteritis nodosa? (Epidemiology)

  • PAN's prevalence is approximately 3.1 per 100,000 people.8

  • It is seen in all ethnic groups and appears to be present throughout the world, although the incidence is higher in areas where hepatitis B is endemic.

  • Primary, idiopathic, or 'classical' polyarteritis nodosa is most common.9

  • Hepatitis B infection is an important cause of secondary polyarteritis nodosa.

  • Associations have also been reported with other infectious agents such as group A streptococcus, hepatitis C, HIV and human T cell leukaemia virus 1, but there is a lack of consistent evidence for any specific microbial involvement in idiopathic polyarteritis nodosa.2 10

  • A genetic condition, deficiency of adenosine deaminase-2 (DADA2), causes a condition that mimics polyarteritis nodosa, and this is sometimes described as a subtype of the disease. Screening for ADA2 mutations in patients initially considered to have idiopathic polyarteritis nodosa has identified DADA2 in 7% to 31%.10

  • The average age of onset is approximately 50 years.2 It can occur, rarely, in childhood, with a mean age at diagnosis of 10 years.11 12

  • In both adults and children, males appear to be more commonly affected than females. 10 12

Polyarteritis nodosa seems to have become an even rarer disease over time. Historically, polyarteritis nodosa was used to describe any systemic vasculitis of unknown cause; tightening of the diagnostic criteria have contributed to fewer diagnoses being made.2 Hepatitis B vaccination campaigns and blood transfusion safety measures have substantially reduced the rates of hepatitis B-associated polyarteritis nodosa.10

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Polyarteritis nodosa symptoms (presentation)2

Diagnosis is not easy, as PAN often presents in a vague manner with symptoms including fever, weight loss, headache and myalgia. There is a spectrum of organ involvement ranging from a single organ to multisystem disease.

  • Peripheral nerves and skin are the most frequently affected tissues.

  • The skin can demonstrate a range of lesions, including purpura, livedoid, subcutaneous nodules and necrotic ulcers.5

  • Neurologically, mononeuritis multiplex is the most common presentation.

  • Involvement of the gastrointestinal tract, kidneys, heart and central nervous system is associated with a higher mortality.9

  • If there is renal involvement, patients may present with hypertension or acute kidney injury. Renal infarction may produce micro- or macrohaematuria and mild to moderate proteinuria.

  • Gastrointestinal symptoms occur in 14-65% of patients and postprandial abdominal pain from ischaemia is the most common symptom.13 Bowel necrosis and perforation are associated with a poor prognosis.

  • Myalgia is reported in 72% of childhood patients.14

  • The typical presentation in children is one- or two-organ involvement, with constitutional symptoms, and the diagnosis is often based on pathology.15

Diagnostic criteria

Diagnosis of polyarteritis nodosa requires the integration of clinical, biopsy, and angiographic findings.


Historically, these have included the American College of Rheumatology (ACR) and Chapel Hill Consensus criteria.4 16 The ACR criteria (10 factors) for classifying a patient with vasculitis within a specific disease entity are useful in clinical practice; however, they were developed before microscopic polyangiitis was reclassified as a separate condition, and make no reference to ANCAs. ANCAs are absent in polyarteritis nodosa, and their presence rules it out.10

According to the ACR criteria, polyarteritis nodosa can be diagnosed in a patient with vasculitis if three or more of the following features are present:16

  • Weight loss greater than 4 kg.

  • Livedo reticularis.

  • Testicular pain or tenderness.

  • Myalgias.

  • Mononeuropathy or polyneuropathy.

  • New-onset diastolic blood pressure greater than 90 mm Hg.

  • Renal dysfunction (blood urea greater than 14.3 mmol/L or creatinine greater than 133 µmol/L).

  • Evidence of hepatitis B infection.

  • Arteriogram showing the arteries that are dilated or constricted by the blood vessel inflammation.

  • On biopsy, presence of granulocyte or mixed leukocyte infiltrate in the wall of a small or medium-sized artery.


Classification of childhood PAN requires a systemic inflammatory disease with evidence of necrotising vasculitis or angiographic abnormalities of medium- or small-sized arteries (mandatory criterion) plus one of five criteria:14

  • Skin involvement.

  • Myalgia/muscle tenderness.

  • Hypertension.

  • Peripheral neuropathy.

  • Renal involvement.

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Differential diagnosis

As PAN presents with nonspecific symptoms, numerous alternative diagnoses must be considered:


  • Serological testing for hepatitis B, hepatitis C, and HIV should be carried out.

  • ANCAs are negative in PAN, and a positive ANCA in the context of necrotising vasculitis strongly suggests an alternative (ANCA-associated) diagnosis, such as microscopic polyangiitis, granulomatosis with polyangiitis, or Churg-Strauss syndrome.2

  • Inflammatory markers (including ESR and CRP) are elevated - this is nonspecific.

  • Biopsy of small arteries will show evidence of necrotising inflammation. Suitable biopsy sites include the skin (including subcutaneous fat with medium-sized arteries); the sural, superficial peroneal, or superficial radial nerves; and muscle. Renal and liver biopsies are best avoided due to a risk of rupture and haemorrhage.10

  • Arteriography shows microaneurysms in the small-sized and medium-sized arteries of the kidneys and abdominal viscera.17 Selective renal angiography shows aneurysms in 40% of children.18

  • FDG-PET/CT is emerging as a potentially useful non-invasive imaging technique for diagnosis.19

Associated diseases

Hepatitis B-associated PAN is an important subtype of PAN. The pathogenesis is attributed to immune-complex deposition with antigen excess.20

Polyarteritis nodosa treatment and management

Modern trial data are limited, and most current guidelines are based on expert opinion.21 Therapy depends on the disease's manifestations and severity. The following are taken from the 2021 American College of Rheumatology/Vasculitis Foundation recommendations:22

  • Newly diagnosed active, severe PAN should be treated with high-dose glucocorticoids plus cyclophosphamide.

  • Newly diagnosed, active, nonsevere PAN should be treated with glucocorticoids and other immunosuppressive agents, such as azathioprine or methotrexate.

  • Once remission has been obtained, non-glucocorticoid immunosuppressive agents should be continued for 18 months, alongside tapering of glucocorticoid therapy.

  • Tumour necrosis factor (TNF) inhibitors appear to be particularly useful in the DADA2 subtype, substantially reducing the risk of stroke.

Management options for other forms of PAN include:

  • In patients with active hepatitis B, antivirals and plasma exchanges prevent the development of long-term hepatic complications of hepatitis B viral infection.20

  • Intravenous immunoglobulin (IV-Ig) and aspirin are effective in childhood PAN but, in resistant cases, either steroid or infliximab has a role.23

  • In cutaneous polyarteritis nodosa:6

    • Mild cases may require only non-steroidal anti-inflammatory drugs (NSAIDs) or colchicine.

    • Prednisolone 30 mg daily or less is often effective in more severe cases but a dosage of 1 mg/kg/day may be required. Unfortunately, exacerbations occur with the tapering of the corticosteroids and adverse effects limit their long-term use.

    • Immunosuppressive agents are frequently effective in CPAN resistant to high-dose corticosteroids and should be reserved for these severe relentless forms.


Serious complications of polyarteritis nodosa include:24

  • Stroke.

  • Bowel infarction.

  • Renal failure.

  • Heart failure.

  • Encephalopathy.

  • Complications of immunosuppressive therapy, such as secondary cancers and opportunistic infections.


Untreated polyarteritis nodosa has a poor prognosis, with a 5-year survival of 13%.

Treatment improves this substantially, with current 5-year survival rates of approximately 80%.2

Once remission has been obtained, relapse is relatively uncommon (affecting fewer than 20% of patients).2 10


Measures to reduce the incidence of hepatitis B infection, such as vaccination and blood transfusion safety protocols, have made hepatitis B-associated polyarteritis nodosa significantly less common.10

Further reading and references

  1. Matteson EL; Historical perspective of vasculitis: polyarteritis nodosa and microscopic polyangiitis. Curr Rheumatol Rep. 2002 Feb;4(1):67-74. doi: 10.1007/s11926-002-0026-9.
  2. De Virgilio A, Greco A, Magliulo G, et al; Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016 Jun;15(6):564-70. doi: 10.1016/j.autrev.2016.02.015. Epub 2016 Feb 13.
  3. Sunderkotter C, Sindrilaru A; Clinical classification of vasculitis. Eur J Dermatol. 2006 Mar-Apr;16(2):114-24.
  4. Jennette JC, Falk RJ, Bacon PA, et al; 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11. doi: 10.1002/art.37715.
  5. Howard T, Ahmad K, Swanson JA, et al; Polyarteritis nodosa. Tech Vasc Interv Radiol. 2014 Dec;17(4):247-51. doi: 10.1053/j.tvir.2014.11.005. Epub 2014 Nov 13.
  6. Morgan AJ, Schwartz RA; Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010 Jul;49(7):750-6.
  7. Nakamura T, Kanazawa N, Ikeda T, et al; Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res. 2008 Sep 19.
  8. Mohammad AJ, Jacobsson LT, Mahr AD, et al; Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford). 2007 Aug;46(8):1329-37. Epub 2007 Jun 6.
  9. Hernandez-Rodriguez J, Alba MA, Prieto-Gonzalez S, et al; Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014 Feb-Mar;48-49:84-9. doi: 10.1016/j.jaut.2014.01.029. Epub 2014 Jan 28.
  10. Puechal X; Polyarteritis Nodosa: State of the art. Joint Bone Spine. 2022 Jul;89(4):105320. doi: 10.1016/j.jbspin.2021.105320. Epub 2021 Dec 11.
  11. Ozen S, Besbas N, Saatci U, et al; Diagnostic criteria for polyarteritis nodosa in childhood. J Pediatr. 1992 Feb;120(2 Pt 1):206-9. doi: 10.1016/s0022-3476(05)80428-7.
  12. Iudici M, Quartier P, Pagnoux C, et al; Childhood- versus Adult-Onset Polyarteritis Nodosa Results from the French Vasculitis Study Group Registry. Autoimmun Rev. 2018 Oct;17(10):984-989. doi: 10.1016/j.autrev.2018.08.001. Epub 2018 Aug 14.
  13. Ebert EC, Hagspiel KD, Nagar M, et al; Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol. 2008 Sep;6(9):960-6. Epub 2008 Jun 27.
  14. Ozen S, Pistorio A, Iusan SM, et al; EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010 May;69(5):798-806.
  15. Ozen S; Juvenile polyarteritis: is it a different disease? J Rheumatol. 2004 Apr;31(4):831-2.
  16. Lightfoot RW Jr, Michel BA, Bloch DA, et al; The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug;33(8):1088-93.
  17. Stanson AW, Friese JL, Johnson CM, et al; Polyarteritis nodosa: spectrum of angiographic findings. Radiographics. 2001 Jan-Feb;21(1):151-9.
  18. Brogan PA, Davies R, Gordon I, et al; Renal angiography in children with polyarteritis nodosa. Pediatr Nephrol. 2002 Apr;17(4):277-83.
  19. Fagart A, Machet T, Collet G, et al; Fluorodeoxyglucose positron emission tomography-computed tomography findings in a first series of 10 patients with polyarteritis nodosa. Rheumatology (Oxford). 2022 Apr 11;61(4):1663-1668. doi: 10.1093/rheumatology/keab591.
  20. Guillevin L, Mahr A, Callard P, et al; Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005 Sep;84(5):313-22.
  21. Springer JM, Byram K; Polyarteritis nodosa: an evolving primary systemic vasculitis. Postgrad Med. 2023 Jan;135(sup1):61-68. doi: 10.1080/00325481.2022.2088940. Epub 2022 Jun 22.
  22. Chung SA, Gorelik M, Langford CA, et al; 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Rheumatol. 2021 Aug;73(8):1384-1393. doi: 10.1002/art.41776. Epub 2021 Jul 8.
  23. Dillon MJ, Eleftheriou D, Brogan PA; Medium-size-vessel vasculitis. Pediatr Nephrol. 2010 Sep;25(9):1641-52. Epub 2009 Nov 28.
  24. Jardel S, Puechal X, Le Quellec A, et al; Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry. Autoimmun Rev. 2018 Jul;17(7):653-659. doi: 10.1016/j.autrev.2018.01.022. Epub 2018 May 3.

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