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Polyarteritis nodosa

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of polyarteritis nodosa are prepared by our editorial team based on guidelines from the Vasculitis Foundation (VF/ACR 2021) and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019). ...
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Diagnostic investigations

Diagnostic imaging: as per ACR/VF 2021 guidelines, consider obtaining abdominal vascular imaging to aid in establishing the diagnosis and determining the extent of disease in patients with suspected PAN.
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Diagnostic procedures

Biopsy
As per ACR/VF 2021 guidelines:
Consider performing deep-skin biopsy (reaching the medium-sized vessels of the dermis) over superficial skin punch biopsy to aid in establishing the diagnosis in patients with suspected PAN involving the skin.
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Consider performing combined nerve and muscle biopsy over nerve biopsy alone to aid in establishing the diagnosis in patients with suspected PAN and peripheral neuropathy (motor and/or sensory).
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Medical management

Induction of remission: as per ACR/VF 2021 guidelines, consider initiating IV pulse corticosteroids over high-dose oral corticosteroids in patients with newly diagnosed active, severe PAN.
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  • Maintenance of remission

  • Management of refractory disease

Nonpharmacologic interventions

Physical therapy: as per ACR/VF 2021 guidelines, consider offering physical therapy in patients with PAN with nerve and/or muscle involvement.
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Therapeutic procedures

Plasma exchange: as per ACR/VF 2021 guidelines, avoid performing plasmapheresis combined with cyclophosphamide and corticosteroids over cyclophosphamide and corticosteroids alone in patients with newly diagnosed active, severe PAN.
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Specific circumstances

Pediatric patients, diagnosis: as per SHARE initiative 2019 guidelines, recognize that there are two different classification criteria for PAN:
ACR criteria
EULAR/PRINTO/PReS endorsed Ankara 2008 criteria.
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  • Pediatric patients (laboratory tests)

  • Pediatric patients (biopsy)

  • Pediatric patients (diagnostic imaging)

  • Pediatric patients (treatment)

Follow-up and surveillance

Follow-up
As per ACR/VF 2021 guidelines:
Consider obtaining follow-up abdominal vascular imaging in patients with a history of severe PAN with abdominal involvement becoming clinically asymptomatic.
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Consider performing serial neurologic examinations instead of repeated EMG/nerve conduction studies (such as every 6 months) to monitor disease activity in patients with a history of peripheral motor neuropathy secondary to PAN.
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