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12/20/22, 4:20 PM Full-Mod1-ICC1 | Module 1 - Interactive Clinical Case (I)

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Module 1 - Interactive Clinical Case (I)


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Case Description
A 73-year-old male presents with a headache.
He has an atherosclerotic heart disease and is a former heavy smoker.

What further information is needed for the diagnostic


workup?
Pause and reflect.

******

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EULAR model answer and explanation

The location, quality and severity of the headache?

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12/20/22, 4:20 PM Full-Mod1-ICC1 | Module 1 - Interactive Clinical Case (I)

For how long has he been experiencing this symptom? Has he


experienced these symptoms before? Is the pain stable, intermittent or
getting worse?
Is the headache accompanied by neurological symptoms like dizziness,
visual disturbances, paraesthesia or paralysis?
Are there systemic symptoms like fever, weight loss, muscle pain, skin
rash or fatigue?
Is there additional pain and/or stiffness in the neck, shoulders and hip-
girdle area?
Has the patient jaw claudication or scalp tenderness?

Case continued
The patient reports that he had a sudden onset of headache, as he woke up
one morning. It is located “all over” the head, but more intense over the right
temporal region. The headache is continuously present.

There were no accompanying symptoms from the neck, shoulders or hip-


girdle. He does not report fever, weight loss, visual disturbances,
neurological symptoms, jaw or chest pain.

The blood pressure was 134/86 mmHg.

Blood test results: ESR 3 mm/h and C reactive protein was 3 mg/L.

Which of the following diagnosis should you consider as


differential diagnosis?
Three correct answers.

Cerebrovascular incident

Migraine

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Giant cell arteritis

Sinusitis

Brain tumour

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EULAR model answers and explanation


You are required to click through all answers to continue with the next
section.

 Cerebrovascular incident

 Migraine

 Giant cell arteritis

 Sinusitis

 Brain tumour

Should be considered. A new unexplained headache can be the


presenting symptom of a brain tumour. The headache is often worse in
the morning. In this case the headache is continuously present with no
variations of the intensity throughout the day.

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Case continued
The patient was admitted to a MRI scan of the head that showed no signs of
bleeding, cerebral ischaemia or brain tumour. Chronic age-related changes
commonly seen in his age were described.

He was further examined by an otolaryngologist, that revealed no signs of


sinusitis or infection. After four weeks from the onset of headache he
experienced jaw claudication, and pain in the area around the left ear. He
also experienced scalp tenderness.

He did not suffer from visual disturbances. New blood test showed an
increase in C-reactive protein to 55 mg/L, ESR was still within the normal
range but had increased from 3 mm/h to 15 mm/h.

What diagnosis do you now suspect?


One correct answer only.

Cerebrovascular incident

Migraine

Giant cell arteritis

Sinusitis

Brain tumour

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EULAR model answers and explanation

Giant cell arteritis (GCA)

Giant cell arteritis is a common disease of the geriatric age group in western
Europe especially. Headache is the most common symptom of GCA (72%)
which is often located at temple and occipital region. Jaw claudication is a
clinical symptom that has a high specificity of GCA, but a low sensitivity.

The superficial temporal artery (STA), occipital, posterior auricular or facial


arteries demonstrate thickening, nodularity, tenderness or erythema.

Laboratory findings included elevated C reactive protein. The ESR was still
within the normal range, however, the ESR may be normal in 22.5 % of biopsy
proven GCA cases.

How would you examine the patient further to confirm


your diagnosis?
Pause and reflect.

******

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EULAR model answer and explanation

In patients with suspected GCA, an early imaging test is recommended.


Ultrasound of the temporal (± axillary arteries) is recommended. In good
hands, the demonstration of a dark halo around the artery has revealed a
sensitivity of 77 % and a specificity of 96%. The demonstration of bilateral
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dark halos seems to correlate well with development of ischemic


complications in GCA, and has in experienced hands, a good correlation with
biopsy proven GCA.

In biopsy it is important to obtain a sufficient segment of STA (commonly


more than 2 cm). The histopathological changes in the temporal arteries
include luminal stenosis, intimal proliferation and disruption of internal
elastic lamina by a mononuclear cell infiltrate. The involvement is typically
patchy (‘skip lesions’).

Case continued
You perform a duplex sonography of the temporal arteries where you are
able to demonstrate a dark halo around the temporal arteries on both sides,
the halo is more protruding around the left temporal artery.

A biopsy from the left temporal artery is obtained, the size of the segment
from the artery is 13 mm.

The segment is examined by a pathologist who describes the findings as a


muscular artery with no apparent signs of inflammation.

In this case you have a 73-year old male with typical symptoms of giant cell
arteritis. Duplex sonography was positive with a characteristic halo around
the temporal arteries, while the harvested but rather short biopsy was
considered negative.

Would you try to get a second biopsy from the right


temporal artery in order to try to confirm the diagnosis of
GCA?
Pause and reflect.

******

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EULAR model answer and explanation

According to the EULAR recommendations for the use of imaging in large


vessel vasculitis, in patients with a clinical high suspicion of GCA and a
positive imaging test, the diagnosis of GCA may be made without an
additional test

Since up to 60% of patients with GCA may demonstrate involvement of aorta


and its branches, imaging studies (CT angiography, MRA or 18F-FDG-PET-CT)
should be considered. In addition, patients with aortitis have higher risk for
aneurysms and require further follow up

Patients of suspected GCA, who have typical symptoms of GCA, but are
biopsy negative, are described to have less constitutional symptoms, less
arterial wall abnormality and have lower chances of ischemic complications,
as compared to biopsy positive cases.

Figure 1 summarizes the approach to patients with suspected GCA.

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