72-year-old male with fever of unknown origin
DESCRIPTION
72-year-old male with fever of unknown origin. Lananh Nguyen, M.D. Division of Neuropathology University of Pittsburgh Medical Center. Clinical history. Patient complains of fluctuating fevers for the last 3 weeks. He has diplopia due to CN IV palsy but no surgical or family history. - PowerPoint PPT PresentationTRANSCRIPT
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Lananh Nguyen, M.D.Division of Neuropathology
University of Pittsburgh Medical Center
72-year-old male with fever of unknown origin
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• Patient complains of fluctuating fevers for the last 3 weeks.• He has diplopia due to CN IV palsy but no surgical or family
history.
Clinical history
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What is the differential diagnosis for fever of unknown origin (FUO)?
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What is the differential diagnosis for fever of unknown origin (FUO)?
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• Patient has myalgias, joint pain, headache and jaw claudication.
• Scalp was tender to palpation.• Imaging was negative for mass lesions.• Serologies and cultures were negative.• Prophylactic antibiotics were initiated without significant
improvement.
Additional history and workup results
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• A temporal artery biopsy was ordered with a suspicion for an inflammatory disorder.
• The biopsy specimen was sent to pathology for evaluation.
What did the clinician do next and why?
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Gross description:5 x 0.2 cm tissue labeled “left temporal artery”
Serial sections of the entire vessel
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Why is it important to evaluate the entire length of the vessels?
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• Inflammatory lesions show patchy distribution hence, you need to evaluate for skip lesions.
• Skip lesions are just that, non contiguous lesions along the length of the vessels.
Why is it important to evaluate the entire length of the vessels?
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This is one section on the slide. Name the histologic layers indicated by ***. What is your interpretation?
******
***
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This is a normal section. This is an artery as evident by the thick muscular wall.
Media - muscular wallAdventitia
Intima
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This is another section on the slide. What vessel layer are we in and what do you see?
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There is inflammation in the vasa vasorum, branches of the temporal artery
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This is another section. What do you see?
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This section shows significant inflammation around and infiltrating the vessel wall.
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Higher magnification shows inflammatory cells in the adventitia and infiltrating the media.
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This is another section where severe inflammation can be appreciated even at low power.
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Higher magnification shows inflammatory cell infiltration with disruption of the internal elastic lamina, the structure which separates the intima from the media.
Internal elastic lamina, note the squiggly pink line
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Another section shows loss of vessel integrity. No granulomas or giant cells were identified.
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Final diagnosis• ARTERY, LEFT TEMPORAL, BIOPSY:• POSITIVE FOR ARTERITIS.
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What is the treatment?
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What is the treatment?
• Glucocorticoids, even prior to confirmatory biopsy. This is key to preventing anterior ischemic optic neuropathy (otherwise known as permanent visual loss). Preferably, biopsy should be perform soon after administration.
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Discussion – Take home points
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• Giant cell arteritis (GCA) is a large vessel vasculitis (aorta and primary branches with a predilection for extracranial arteries)
• Pathologically, giant cells may not be present and skip lesions can occur. Therefore, evaluate multiple levels and evaluate the vasa vasorum (branches of the temporal artery) for evidence of inflammatory cells.
• Mean age 70; female:male 2:1• SIGNS AND SYMPTOMS: new-onset headache, fever, visual symptoms (blindness,
amaurosis fugax, diplopia), TA/scalp tenderness, jaw claudication, weight loss• LABS: elevated ESR/CRP but also anemia, hypoalbuminemia, transaminitis. SIADH
Is associated with GCA• 40% with GCA have polymyalgia rheumatica (PMR) but only 10% with PMR have
GCA on temporal artery biopsy. It’s a spectrum.• American College of Rheumatology criteria for diagnosis: 3 out of the following 5
• Age > 50• New headache• Temporal artery abnormality• ESR > 50• Abnormal temporal artery biopsy