Polyarteritis Nodosa

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Learning objectives

  • Appropriate settings to look for PAN
  • Supporting evidence to ensure firm diagnosis
  • Treatment options

Introduction

This a systemic necrotising vasculitis of medium-sized arteries without the involvement of smaller vessels. It may be associated with hepatitis virus (HBV/HCV) infection. Only about 20% have any cerebral involvement.


Clinical

Patients have typical systemic illness, myalgia, weight loss, fever, arthritis, peripheral ischaemic changes. They can have a polyneuropathy. Patients may experience both ischaemic and haemorrhagic strokes as well as an encephalopathy. Biopsies classically show necrotising granulomatous inflammation.

Diagnostic criteria

PAN may be diagnosed with three of these ten criteria (82% sensitivity, 86% specificity), if other

vasculitides are excluded
1 Loss of weight >4 kg
2 Livedo reticularis
3 Testicular pain
4 Myalgias
5 Mononeuritis or polyneuritis
6 Blood pressure elevation >90 mmHg
7 Creatinine >1.5 mg/dl
8 Hepatitis B or C virus antibodies
9 Pathologic arteriography (aneurysm, occlusions)
10 Typical histology finding

Management

  • Induction therapy for a confirmed case is usually with Steroids and Cyclophosphamide.
  • Plasma exchange has been used.
  • Antivirals if serology suggests Hepatitis B or C may be given.

References