vasculitis

see also:

• collagen vascular disorders

• 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.

• 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

• Kawasaki disease
  • children with fever > 5 days and mucocutaneous syndrome with risk of coronary artery inflammation
• polyarteritis nodosa (PAN) - rare, esp. 45-65yr olds; 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
  • hepatitis C associated polyarteritis nodosa
• cutaneous polyarteritis
• Primary vasculitis of the CNS, angiography-positive primary angiitis of the CNS (PACNS)

• Henoch-Schonlein purpura (HSP)
  • palpable purpura in dependent part of body, abdominal pain (which may be associated with GI hemorrhage or intussusception in children), arthritis, and evidence of glomerulonephritis (GN)
  • IgA immune deposition in small vessels
  • 90% of cases occur in children but can occur in adults and when it does is often more severe with greater renal damage (30% develop renal impairment, usually within 2 months of onset, but can be up to 6 months, however, steroids do not appear to reduce the risk)¹⁾ requiring more close monitoring +/- colchicine for severe skin lesions, +/- steroids for severe GIT involvement or nephrotic range proteinuria / rapid progressive GN.
  • ~60% of adults have elevated serum IgA levels
• hypersensitivity vasculitis
  • may appear identical to Henoch-Schonlein purpura (HSP)
  • isolated cutaneous leukocytoclastic vasculitis
    • eg. Erythema elevatum diutinum (EED) see emedicine article
  • vasculitis secondary to infection, malignancies, and drugs, including hypersensitivity vasculitis
• Goodpasture's syndrome (antiglomerular basement membrane (GBM) antibody disease)
  • pulmonary hemorrhage and rapidly progressive glomerulonephritis (GN)
• 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)
    • causes purpuric rash, glomerulonephritis (GN), hemoptysis, CNS involvement, and arthralgias
  • Churg-Strauss syndrome (rare)
    • associated with eosinophilia, asthma, hypertension, nephritis, rhinosinus growths, pericarditis and neuropathies
    • nonfixed pulmonary infiltrates
    • ANCA in only 40%