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Updated: Mar 22 2022

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
    • 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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