Updated: 7/10/2019

Giant Cell Arteritis

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Snapshot
  • A 65-year-old woman presents to the emergency department due to a severe headache and visual impairment in the right eye. Her symptoms are associated with pain with chewing and proximal muscle morning stiffness. On physical exam, she has decreased visual acuity of the right eye, scalp tenderness on the right, and an absent pulse in the right temporal area. Laboratory testing is significant for an elevated erythrocyte sedimentation rate. She is admitted and immediately started on systemic glucocorticoids. 
Introduction
  • Clinical definition
    • a chronic large- and medium-sized vessel vasculitis that typically involves the
      • temporal artery
      • cranial arteries
  • Epidemiology
    • incidence
      • most common systemic vasculitis affecting patients ≥ 50 years of age
    • demographic
      • woman > men
  • Etiology
    • presumed to be autoimmune
  • Pathogenesis
    • T-cells and monocytes are recruited to the vessel wall and result in an inflammatory response
  • Associated conditions
    • polymyalgia rheumatica 
  • Prognosis
    • relapses can occur in 20-50% of patients
Presentation
  • Symptoms
    • temporal headache
    • jaw claudication
    • amaurosis fugax
    • symptoms of polymyalgia rheumatica (PMR)
      • morning stiffness
  • Physical exam
    • scalp tenderness
    • palpation of the temporal area may demonstrate
      • absent pulse
      • knot-like swelling
    • vision loss
    • findings of PMR
      • distal extremity swelling
Imaging
  • Color-coded duplex ultrasound
    • indication
      • to assess the temporal arteries and extracranial vessels
Studies
  • Labs
    • ↑ erythematous sedimentation rate
    • ↑ C-reactive protein
  • Temporal artery biopsy
    • confirms the diagnosis
Differential
  • Migraine
    • distinguishing factors
      • may be accompanied by an aura, photophobia, and phonophobia
  • Takayasu arteritis
    • distinguishing factors
      • typically affects young and Asian women
      • also treated with steroids
Treatment
  • Management approach
    • high-dose systemic glucocorticoids should be promptly administered even before the diagnosis is established 
    • temporal artery biopsy confirms the diagnosis
  • Medical
    • corticosteroids  
      • indication
        • standard initial treatment for patients suspected of having temporal arteritis
Complications
  • Blindness 
 

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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, 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
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(M2.RH.4691) A 74-year-old woman with no significant past medical history presents with 1 week of fever, unremitting headache and hip and shoulder stiffness. She denies any vision changes. Physical examination is remarkable for right scalp tenderness and range of motion is limited due to pain and stiffness. Neurological testing is normal. Laboratory studies are significant for an erythrocyte sedimentation rate (ESR) at 75 mm/h (normal range 0-22 mm/h for women). Which of the following is the most appropriate next step in management? Review Topic

QID: 107607
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Obtain CT head without contrast

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Perform a lumbar puncture

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Perform a temporal artery biopsy

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Start oral prednisone

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Start IV methylprednisolone

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M2

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(M2.RH.4691) A 72-year-old man presents to his primary care provider complaining of fatigue, mild headache, and discomfort with chewing for roughly 1 week. Before this, he felt well overall, but now is he is quite bothered by these symptoms. His medical history is notable for hypertension and hyperlipidemia, both controlled. On examination, he is uncomfortable but nontoxic-appearing. There is mild tenderness to palpation over his right temporal artery, but otherwise the exam is not revealing. Prompt recognition and treatment can prevent which of the following feared complications: Review Topic

QID: 107656
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Renal failure

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Blindness

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Pericarditis

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Pulmonary fibrosis

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Cognitive impairment

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M2

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(M3.RH.62) A 66-year-old woman presents to the emergency department with a throbbing headache. She states that the pain is worse when eating and is localized over the right side of her head. Review of systems is only notable for some blurry vision in the right eye which is slightly worse currently. The patient's past medical history is notable only for chronic pain in her muscles and joints for which she has been taking ibuprofen. Her temperature is 99.1°F (37.3°C), blood pressure is 144/89 mmHg, pulse is 87/min, respirations are 14/min, and oxygen saturation is 98% on room air. Physical examination is significant for tenderness to palpation over the right temporal region. Which of the following is the best initial step in management? Review Topic

QID: 103085
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100% oxygen

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CT head

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Ibuprofen and acetaminophen

50%

(3/6)

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Methylprednisolone

33%

(2/6)

5

MRI head

17%

(1/6)

M2

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PREFERRED RESPONSE 4
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