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Updated: Dec 28 2021

Polyarteritis Nodosa

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https://upload.medbullets.com/topic/120732/images/renal microaneurysm pan.jpg
  • 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
  • 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
  • Prognosis
    • In untreated cases, it is associated with a poor prognosis
    • Hepatitis B-associated disease has a worse prognosis
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