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Polyarteritis Nodosa

Katherine S. Upchurch, MD, MACR and Stephen Morais, MD, MBA, MS Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Polyarteritis nodosa (PAN) is an antineutrophil cytoplasmic antibody (ANCA)-negative necrotizing arteritis of medium-sized muscular arteries and (occasionally) small arteries. Arteri...

DIAGNOSIS

  • There are no formal diagnostic criteria for PAN (1),(2).

  • Suspect PAN with:

    • Acute, sometimes fulminant multisystem disease with a relatively short prodrome (i.e., weeks to months)

    • Vasculitic ski...

TREATMENT

GENERAL MEASURES

Treat HTN aggressively to prevent complications (stroke, myocardial infarction, heart failure). 

MEDICATION

First Line

  • Severe (life-threatening) disease: corticosteroids (CS) (h...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • CBC, urinalysis, renal and hepatic function tests

  • Acute-phase reactants (e.g., ESR, CRP) may help monitor disease activity.

  • The revised 2009 Five ...

REFERENCES

1
De Virgilio A, Grego A, Magliulo G, et al. Polyarteritis nodosa: a contemporary overview. Autoimmun Rev.  2016;15(6):564–570. [View Abstract on OvidMedline]
2
Pagnoux C, Seror R, Henega...

ADDITIONAL READING

Samson M, Puéchal X, Mouthon L, et al; for French Vasculitis Study Group. Microscopic polyangiitis and non-HBV polyarteritis nodosa with poor-prognosis factors: 10-year results of t...

CODES

ICD10

  • M30.0 Polyarteritis nodosa

  • M30.1 Polyarteritis with lung involvement [Churg-Strauss]

  • M30.8 Other conditions related to polyarteritis nodosa

  • M31.7 Microscopic polyangiitis

ICD9

446.0 Polyarteriti...

CLINICAL PEARLS

  • PAN is a necrotizing vasculitis of small- to medium-sized muscular arteries with lack of granuloma formation that spares veins and pulmonary arteries.

  • Clinical features of PAN depend on...

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