Updated: 1/29/2019

Polyarteritis Nodosa

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Snapshot
  • A 52-year-old man presents to the emergency department with abdominal pain and rectal bleeding. His symptoms are associated with malaise, fever, and weight loss. He does not have a family history of malignancy and his last colonoscopy was unremarkable. His blood pressure is 155/98 mmHg (his blood pressure is usually 125/74 mmHg). On physical exam, he has abdominal tenderness, livedo reticularis, and cutaneous ulcers. Laboratory testing is notable for a newly elevated creatinine of 2.6 mg/dL, erythrocyte sedimentation rate, and C-reactive protein. He is also seropositive for hepatitis B virus, ANCA-negative, and guaiac positive.
Introduction
  • Clinical definition
    • medium vessel systemic necrotizing vasculitis
      • not associated with ANCA antibodies 
  • Epidemiology
    • demographics
      • 50-70 years of age
    • risk factors
      • hepatitis B virus
  • Etiology
    • idiopathic (most common)
    • secondary polyarteritis nodosa can result from
      • hepatitis B infection 
      • hepatitis C infection
      • hairy cell leukemia
  • Pathogenesis
    • type III hypersensitivity reaction leading to artery occlusion or rupture which can result in organ ischemia and hemorrhage
  • Prognosis
    • in untreated cases, it is associated with a poor prognosis
    • hepatitis B-associated disease has a worse prognosis
Presentation
  • Symptoms/physical exam
    • systemic symptoms
      • fever
      • weight loss
      • malaise
    • neurologic
      • mononeuritis multiplex
      • polyneuropathy
      • stroke
      • confusion
    • musculoskeletal
      • articular pain
      • extremity pain
    • skin
      • ulcers
      • livedo reticularis
      • purpura
    • renal
      • glomerulonephritis
      • hematuria
    • new onset hypertension
    • pulmonary
      • typically spares lungs, though may involve bronchial arteries
    • gastrointestinal
      • abdominal pain
      • rectal bleeding
Imaging
  • Angiography 
    • indication
      • considered in patients suspected of having polyarteritis nodosa in the setting of being unable to obtain a histologic diagnosis 
    • findings
      • arterial microaneurysms (saccular or fusiform) with stenotic lesions typically affecting arterial branches of the
        • kidney
        • mesentery
        • liver
Studies
  • Studies
    • ↑ erythrocyte sedimentation rate
    • ↑ C-reactive protein
    • may be positive for
      • hepatitis B virus (~30% of patients)
      • hepatitis C virus
      • parvovirus B19 virus
      • HIV
  • Biopsy
    • confirms the diagnosis 
      • must biopsy the clinically affected organ
    • demonstrates medium vessel vasculitis
Differential
  • Granulomatosis with polyangiitis
    • differentiating factors
      • ANCA-positive vasculitis
      • affects the upper and lower respiratory tracts and kidneys
      • affects small vessels
  • Microscopic polyangiitis
    • differentiating factors
      • ANCA-positive vasculitis
      • similar to granulomatosis with polyangiitis without nasopharyngeal involvement
      • affects small vessels
Treatment
  • Medical
    • high-dose corticosteroids with cyclophosphamide
      • indication
        • in patients with moderate to severe disease
Complications
  • Thrombosis
  • Hemorrhage
  • Ulcers

 

 

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