Updated: 8/7/2019

Medium Vessel Vasculitides

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Snapshot
  • A patient presents with a fever, diffuse myalgias, abdominal pain, a peripheral neuropathy, as well as hypertension and a recent weight loss of 10 pounds.  The patient has a past medical history of hepatitis B and C.  Angiography demonstrates a "string of pearls appearance" in the renal artery, as well as in several other organ systems with the exception of the pulmonary arteries.
Polyarteritis Nodosa
  • Introduction
    • immune complex-mediated transmural vasculitis with fibrinoid necrosis
      • involves renal, coronary, and mesenteric arteries
      • does not involve the pulmonary arteries
      • lesions of different ages (the rationale being that circulating immune complexes deposit at different times)
  • Symptoms
    • flu-like symptoms
      • fever, malaise, and myalgia
    • GI symptoms
      • weight loss, abdominal pain, melena
    • hypertension
    • neurologic dysfunction
    • cutaneous eruptions
  • Findings
    • hepatitis B (HBsAg) seropositivity in 30% of patients 
    • multiple aneurysms and constrictions on arteriogram
    • segmental ischemic necrosis with lesions of different ages 
    • increased inflammatory markers like ESR and CRP
  • Treatment
    • corticosteroids
    • cyclophosphamide
Snapshot
  • A 7-year-old male presents with conjunctival injection and a rash that has persisted for quite some time.  On physical exam, the patient has a bright red tongue, adenopathy, and hepatosplenomegaly as well as a fever that has persisted for the past 7 days.
Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)
  • Introduction 
    • acute, self-limiting necrotizing vasculitis 
    • coronary arteries often affected (thrombosis and aneurysm)
    • seen in infants and children
      • leading cause of death from acquired heart disease in children
    • association with Asian ethnicity
  • Presentation
    • fever
    • conjunctivitis
    • cervical lymphadenitis
    • desquamative skin rash
      • with changes in lips/oral mucosa ("strawberry tongue")
    • myocardial infarction (if coronary arteries affected)
    • crash and burn
      • conjunctivitis
      • rash
      • adenopathy
      • strawberry tongue
      • hands (peeling skin)
      • fever
  • Treatment 
    • IV immunoglobulin
    • aspirin
    • vaccinations (do not want the patient to get a viral infection due to aspirin treatment)
Snapshot
  • A 69-year-old gentleman presents with painful discoloration and ulceration of his fingers. The patient's symptoms have been slowly worsening over the past several months.  This patient has smoked 3 packs of cigarettes per day for the past 40 years.
Thromboangiitis Obliterans (Buerger Disease)
  •  Introduction
    • vasculitis with digital vessel thrombosis
    • seen in heavy smokers, most commonly in men between ages 25 and 50 
    • thought to be caused by a hypersensitivity reaction to components of tobacco smoke
  • Presentation
    • intermittent claudication
    • painful ulceration due to the involvement of nerve and vessel
    • Raynaud's phenomenon
    • may lead to gangrene and autoamputation of digits
  • Treatment
    • smoking cessation
    • cilostazol
    • iloprost
    • calcium channel blockers (nifedipine)
Snapshot
  • A 49-year-old woman with abnormally smooth skin on her face and hands presents with a chief concern of episodes of intense pain in her hands.  She states that her hands at times will turn pale, then will flush red after a short period of time.  She describes the episodes as very painful and unpleasant.
Raynaud's Disease
  • Introduction
    • vasculitis affecting vessels of fingers and toes 
    • ↑↑ vasomotor reaction to cold/stress
    • seen in young women
  • Presentation
    • digital color changes (white to blue to red)
    • chronic cases may progress to ulceration and gangrene
  • Diagnosis
    • nail fold capillaroscopy Nailfold capillaroscopy - in Raynaud's
  • Treatment
    • calcium channel blockers (nifedipine, amlodipine) 
    • sildenafil (phosphodiesterase inhibitor)
Raynaud's Phenomenon
  • Introduction
    • vasculitis affecting vessels of fingers and toes
    • the phenomenon as result of an underlying disease   
      • e.g., CREST syndrome and systemic sclerosis
    • affects adults (no gender specificity)
  • Presentation
    • digital color changes
    • chronic cases may progress to ulceration and gangrene
  • Treatment
    • lifestyle and general
      • avoid triggers such as cold and stress
      • cessation of smoking
      • avoid medications that can precipitate attacks such as beta-blockers and ergotamines
    • medical
      • calcium channel blockers (nifedipine)
        • first-line
      • topical nitroglycerin
        • second-line

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