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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(M1.CV.13.60) A 48-year-old male presents to his primary physician with the chief complaints of fever, abdominal pain, weight loss, muscle weakness, and numbness in his lower extremities. UA is normal. A biopsy of the sural nerve reveals transmural inflammation and fibrinoid necrosis of small and medium arteries. Chart review reveals a remote history of cigarette smoking as a teenager and Hepatitis B seropositivity. What is the most likely diagnosis? Tested Concept

QID: 100576
1

Polyarteritis nodosa

68%

(25/37)

2

Microscopic polyangiitis

5%

(2/37)

3

Thromboangiitis obliterans

14%

(5/37)

4

Raynaud disease

5%

(2/37)

5

Systemic lupus erythematosis

3%

(1/37)

M 2 D

Select Answer to see Preferred Response

(M1.CV.12.59) An 31-year-old Israeli male with a history of heavy smoking presents to your office with painful ulcerations on his hands and feet. Upon examination, he is found to have hypersensitivity to intradermally injected tobacco extract. Which of the following processes is most likely responsible for his condition?
Tested Concept

QID: 100575
1

Increased endothelial permeability

4%

(1/23)

2

Necrotizing inflammation involving renal arteries

4%

(1/23)

3

Segmental vasculitis of small and medium-sized arteries

65%

(15/23)

4

Eosinophil-rich granulomatous inflammation

26%

(6/23)

5

Concentric thickening of the arteriolar wall

0%

(0/23)

M 2 E

Select Answer to see Preferred Response

Evidence (6)
VIDEOS (1)
Topic COMMENTS (8)
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