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Polyarteritis Nodosa G027

Last amended 
5 May 2020
Current RMA Instruments
Reasonable Hypothesis SOP33 of 2020
Balance of Probabilities SOP 34 of 2020
Changes from previous Instruments

SOP bulletin 214

ICD Coding

  • ICD-9-CM Codes: 446.0
  • ICD-10-AM Codes: M30.0
Brief description

Polyarteritis nodosa is a systemic, necrotising, inflammation (vasculitis) of medium-sized or small arteries.  The condition does not involve veins and mostly spares arterioles, capillaries, and venules. 

Confirming the diagnosis

This diagnosis is complex.  The condition needs to be distinguished from multiple other types of vasculitis and related disorders, but particularly microscopic polyangiitis.  Characteristic clinical findings and laboratory and radiological results may indicate the diagnosis, but confirmation really requires histology from biopsy of an affected organ.

There is no specific specialist physician for this disease with the relevant specialty being related to the site or sites of the vasculitis.

Additional diagnoses covered by SOP
  • Cutaneous polyarteritis nodosa
  • Necrotising angiitis
  • Panarteritis or periarteritis (nodosa)
  • Disseminated necrotising periarteritis
Conditions not covered by SOP
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)#
  • Giant cell or temporal arteritis*
  • Hypersensitivity vasculitis#
  • Kawasaki disease#
  • Microscopic polyangiitis*
  • Polyangiitis overlap syndrome#
  • Polyarteritis with lung involvement#
  • Thromboangiitis obliterans (Buerger’s disease)*
  • Wegner’s granulomatosis#

* another SOP applies

# non-SOP condition

Clinical onset

The condition typically presents with systemic symptoms (fatigue, weight loss, weakness, fever, arthralgias) and signs of multisystem involvement (skin lesions, hypertension, renal insufficiency, neurologic dysfunction, abdominal pain).  It is a rare disease, presenting mostly in middle aged or older adults.

Clinical worsening

Treatment depends on the severity and extent of disease.  Glucocorticoids and other immunosuppressive drugs are used.  The condition is moderately responsive to treatment.  Remission occurs in a percentage of patients but the condition may relapse or progress.  Impaired renal and cardiac function and cerebral infarction may all occur and the condition has a significant mortality rate.