Updated: 7/7/2018

Large Vessel Vasculitides

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Review Topic
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Snapshot
  • A 62-year-old woman presents to her primary care physician due to an unremitting headache. She says she has been having this headache for the last few days and is nonresponsive to ibuprofen. Her headache is left-sided and affects the temporal region of her head. She notes to having jaw pain when chewing food or brushing her teeth. On physical exam, there is tenderness to palpation of the left temporal artery. (Giant cell temporal arteritis)
Large Vessel Vasculitides
 
Large Vessel Vasculitides
Type
Pathology
Presentation Studies Treatment
Giant cell temporal arteritis
  • Intimal thickening
  • Medial granulomatous inflammation leading to elastic lamina fragmentation 
    • may be focally found, requiring at least a 1 cm segment of the temporal artery
  • Elderly (> 50 years of age) patient with a headache in the temporal region, often worse with chewing
  • Pain upon palpation of the temporal artery
  • ~50% will present with jaw claudication
  • Abnormalities in vision may occur (e.g., diplopia or blindness) when the ophthalmic artery is involved 
  • May be associated with polymyalgia rheumatica
  • ↑ erythrocyte sedimentation rate (ESR)
  • Temporal artery biopsy
  • High-dose corticosteroids  
    • must be administered immediately in order to prevent blindness
Takayasu arteritis
  • Transmural fibrous thickening of the aortic arch and major vessels 
  • Classic in Japanese women who are < 40 years of age
  • Weak pulse and decreased blood pressure in the upper extremities
  • Fatigue, malaise, weight loss, and arthralgias
  • ↑ erythrocyte sedimentation rate (ESR)
  • Angiography or MRA
  • High-dose corticosteroids
 
 

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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, toal, 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 (template) 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm^3
Female: 3.5-5.5 million mm^3
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/mm^3
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 μm^3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm^3
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/mm^3
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/m^2
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(M1.CV.9) A 69-year-old woman presents to her primary care physician complaining of a new-onset, left-sided, throbbing headache that has lasted for several days. She reports that the pain is worsened by eating. Which of the following is a complication of this condition if not treated promptly? Review Topic

QID: 101673
1

Skin nodules

0%

(0/9)

2

Intestinal ischemia

0%

(0/9)

3

Monocular blindness

100%

(9/9)

4

Myocardial infarction

0%

(0/9)

5

Ischemic stroke

0%

(0/9)

M1

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PREFERRED RESPONSE 3

(M1.CV.63) A 68-year-old Caucasian male complains of severe headache and pain while chewing. Upon examination, he is found to have a left visual field deficit. Laboratory results show elevated erythrocyte sedimentation rate. Which of the following drugs would be the best choice for treatment of this patient?
Review Topic

QID: 100579
1

Propranolol

9%

(1/11)

2

Prednisone

91%

(10/11)

3

Sumatriptan

0%

(0/11)

4

Pilocarpine

0%

(0/11)

5

Clopidogrel

0%

(0/11)

M1

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PREFERRED RESPONSE 2

(M1.CV.69) A 30-year-old Japanese female presents with flu-like symptoms and weak pulses in her upper extremities. An angiogram reveals granulomatous inflammation of the aortic arch. Which of the following disease processes is most similar to this patient's disease? Review Topic

QID: 100585
1

Temporal arteritis

58%

(7/12)

2

Polyarteritis nodosa

0%

(0/12)

3

Kawasaki disease

33%

(4/12)

4

Buerger's disease

0%

(0/12)

5

Infectious vasculitis

8%

(1/12)

M1

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PREFERRED RESPONSE 1
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