Vasculitis

Vasculitis is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

  • Vasculitis is characterized by inflammation of blood vessels, leading to tissue damage and necrosis. This diagnosis includes a broad spectrum of disorders with various causes that involve vessels of different types, sizes, and locations. The immunopathologic process may involve immune complexes.
  • Although in most cases the inciting agent has not been identified, some are associated with chronic hepatitis B and C.
  • Vasculitis “mimics” should be considered, including bacterial endocarditis, HIV infection, atrial myxoma, paraneoplastic syndromes, cholesterol emboli, and cocaine and amphetamine use.
  • Vasculitides are classified by vessel sizes (Table 25-7).
  • Vasculitis should be suspected whenever a patient with systemic symptoms has organ dysfunction. Symptoms are associated with the size of the affected vessel (Table 25-8).
  • We will review some of the most common vasculitis in the following sections.
Table 25-7: Vasculitis Classification
Vessel SizeTypes of VesselTypes of Vasculitis
Large vesselAorta and main branches
  • Takayasu arteritis
  • Giant cell arteritis
Medium vesselMain visceral arteries and veins and their initial branches
  • Polyarteritis nodosa
  • Kawasaki disease
Small vesselArterioles, venules, and capillariesANCA-associated vasculitis:
  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
  • Microscopic polyangiitis
Immune complex mediated:
  • Cryoglobulinemic vasculitis
  • Henoch-Schönlein purpura (HSP)
  • Hypocomplementemic urticarial vasculitis
Variable vesselCan involve any vessel size
  • Behcet disease
  • Cogan syndrome
Table 25-8: Vasculitis Features by Vessel Size
Organ SystemSmall VesselMedium VesselLarge Vessel
DermatologicPalpable purpuraLivedo reticularisCyanosis
RenalHematuria with RBC casts, proteinuriaHematuria, flank painHypertension
GastrointestinalGI bleedingBowel perforationBowel infarction
NeurologicPolyneuropathyPolyneuropathy, strokesStrokes

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General Principles

  • Vasculitis is characterized by inflammation of blood vessels, leading to tissue damage and necrosis. This diagnosis includes a broad spectrum of disorders with various causes that involve vessels of different types, sizes, and locations. The immunopathologic process may involve immune complexes.
  • Although in most cases the inciting agent has not been identified, some are associated with chronic hepatitis B and C.
  • Vasculitis “mimics” should be considered, including bacterial endocarditis, HIV infection, atrial myxoma, paraneoplastic syndromes, cholesterol emboli, and cocaine and amphetamine use.
  • Vasculitides are classified by vessel sizes (Table 25-7).
  • Vasculitis should be suspected whenever a patient with systemic symptoms has organ dysfunction. Symptoms are associated with the size of the affected vessel (Table 25-8).
  • We will review some of the most common vasculitis in the following sections.
Table 25-7: Vasculitis Classification
Vessel SizeTypes of VesselTypes of Vasculitis
Large vesselAorta and main branches
  • Takayasu arteritis
  • Giant cell arteritis
Medium vesselMain visceral arteries and veins and their initial branches
  • Polyarteritis nodosa
  • Kawasaki disease
Small vesselArterioles, venules, and capillariesANCA-associated vasculitis:
  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
  • Microscopic polyangiitis
Immune complex mediated:
  • Cryoglobulinemic vasculitis
  • Henoch-Schönlein purpura (HSP)
  • Hypocomplementemic urticarial vasculitis
Variable vesselCan involve any vessel size
  • Behcet disease
  • Cogan syndrome
Table 25-8: Vasculitis Features by Vessel Size
Organ SystemSmall VesselMedium VesselLarge Vessel
DermatologicPalpable purpuraLivedo reticularisCyanosis
RenalHematuria with RBC casts, proteinuriaHematuria, flank painHypertension
GastrointestinalGI bleedingBowel perforationBowel infarction
NeurologicPolyneuropathyPolyneuropathy, strokesStrokes

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