Updated: 3/13/2018

Medium Vessel Vasculitides

Topic
Review Topic
0
0
Questions
4
0
0
Evidence
6
0
0
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, malaise, 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
    • 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
 

Please rate topic.

Average 5.0 of 3 Ratings

Thank you for rating! Please vote below and help us build the most advanced adaptive learning platform in medicine

The complexity of this topic is appropriate for?
How important is this topic for board examinations?
How important is this topic for clinical practice?
Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
Calculator

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(M1.CV.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? Review Topic

QID: 100576
1

Polyarteritis nodosa

100%

(4/4)

2

Microscopic polyangiitis

0%

(0/4)

3

Thromboangiitis obliterans

0%

(0/4)

4

Raynaud disease

0%

(0/4)

5

Systemic lupus erythematosis

0%

(0/4)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(M1.CV.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?
Review Topic

QID: 100575
1

Increased endothelial permeability

0%

(0/12)

2

Necrotizing inflammation involving renal arteries

0%

(0/12)

3

Segmental vasculitis of small and medium-sized arteries

75%

(9/12)

4

Eosinophil-rich granulomatous inflammation

25%

(3/12)

5

Concentric thickening of the arteriolar wall

0%

(0/12)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3
ARTICLES (6)
Topic COMMENTS (5)
Private Note