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vasculitis

vasculitis

introduction

  • vasculitis is inflammation of the blood vessels (usually the term is applied to arterial system rather than venous which tend to fall under the thrombophlebitis term)
  • systemic vasculitis may be acute, subacute or chronic, and may cause widespread organ damage from either local bleeding, thrombosis or ischaemia, and generally only causes inflammation in certain sizes of vessels depending upon the pathology.

large vessel vasculitis

  • giant cell temporal arteritis
  • Takayasu's arteritis - transmural inflammation with giant cells involving the aorta and its major branches resulting in characteristic clinical features caused by stenotic large vessels and subsequently decreased blood supply to the organ systems such as claudication

medium vessel vasculitis

    • children with fever > 5 days and mucocutaneous syndrome with risk of coronary artery inflammation
  • polyarteritis nodosa (PAN) - nodular skin lesions, neuropathy, and hypertension
    • childhood systemic polyarteritis nodosa:
      • small and mid-size artery necrotizing vasculitis on biopsy with at least 2 of:
        • skin involvement (eg, livedo reticularis, tender subcutaneous nodules, other vasculitic lesions)
        • myalgia
        • systemic hypertension
        • mononeuropathy or polyneuropathy
        • abnormal urinalysis and/or impaired renal function (GFR < 50% normal for age)
        • testicular pain or tenderness
        • signs or symptoms suggesting vasculitis of any other major organ system (GI, cardiac, pulmonary, CNS)
    • childhood cutaneous polyarteritis nodosa:
      • presence of subcutaneous nodular, painful, nonpurpuric lesions with or without livedo reticularis and absence of systemic involvement
      • associated with streptococcus / streptococcal infections infection in 40%
  • hepatitis B associated polyarteritis nodosa
  • cutaneous polyarteritis
  • Primary vasculitis of the CNS, angiography-positive primary angiitis of the CNS (PACNS)

small vessel vasculitis

    • IgA immune deposition in small vessels in children
    • palpable purpura in dependent part of body, abdominal pain (which may be associated with GI hemorrhage or intussusception), arthritis, and evidence of glomerulonephritis (GN)
  • hypersensitivity vasculitis
    • may appear identical to Henoch-Schonlein purpura (HSP)
    • isolated cutaneous leukocytoclastic vasculitis
    • vasculitis secondary to infection, malignancies, and drugs, including hypersensitivity vasculitis
  • Goodpasture's syndrome (antiglomerular basement membrane (GBM) antibody disease)
  • hypocomplementic urticarial vasculitis
  • primary vasculitis of the CNS, angiography-negative, small vessel PACNS
  • Behçet's disease (mainly venous, rare)
    • recurrent oral aphthous ulcers, genital ulcers, and uveitis +/- optic neuropathy
    • may cause erythema nodosum and lesions similar to pyoderma gangrenosum.
    • can also involve visceral organs such as the gastrointestinal tract (esp. ileocecal valve), pulmonary, musculoskeletal (eg. arthralgia), and neurological systems (eg. aseptic meningitis, dural sinus thrombosis, encephalopathy)
    • can be fatal due to ruptured vascular aneurysms (eg. pulmonary artery aneurysm) or severe neurological complications
  • vasculitis associated with connective tissue diseases (eg. systemic lupus erythematosus (SLE))
  • Cogan syndrome (rare)
    • recurrent keratitis, vertigo and deafness, often with fever, fatigue and LOW
    • atypical form is called Logan syndrome
  • ANCA-associated vasculitis:
    • granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis)
      • most commonly involves the sinopulmonary system
    • microscopic polyangiitis (MPA)
    • Churg-Strauss syndrome (rare)
      • associated with eosinophilia, asthma, hypertension, nephritis, rhinosinus growths, pericarditis and neuropathies
      • nonfixed pulmonary infiltrates
      • ANCA in only 40%
vasculitis.txt · Last modified: 2013/02/03 00:53 (external edit)