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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
Will imaging change the diagnosis and management of giant cell arteritis?
Andra Chiriac1, Camelia Badea2,3, Cristian Băicuș2,3
1Colentina Clinical Hospital, Department of Neurology, Bucharest, Romania.
Insights
Giant cell arteritis diagnosis can be challenging. Ultrasonography, particularly the halo sign, aids in diagnosing cases where temporal artery biopsy is negative, improving patient management.
Area of Science:
- Rheumatology
- Internal Medicine
- Diagnostic Imaging
Background:
- Giant cell arteritis (GCA) is a prevalent systemic vasculitis in the elderly, affecting large arteries.
- Diagnosis typically relies on temporal artery biopsy (TAB) and American College of Rheumatology (ACR) criteria.
- Challenges arise in cases with atypical presentations or negative TAB results.
Observation:
- A patient presented with new-onset headache and systemic inflammation, not meeting classical GCA diagnostic criteria.
- Temporal artery biopsy (TAB) was negative, posing a diagnostic dilemma.
- Bilateral halo sign observed on color duplex ultrasonography (CDUS).
Findings:
- The halo sign on CDUS demonstrated a high positive likelihood-ratio (LR+ = 43), significantly increasing post-test probability to 88% in this case.
- Clinical evolution, including headache remission and rebound phenomena upon steroid tapering, supported the GCA diagnosis.
- Ultrasonography findings, combined with clinical response, proved crucial when traditional criteria were insufficient.
Implications:
- Color duplex ultrasonography (CDUS) offers a valuable, non-invasive tool for diagnosing GCA, especially in equivocal cases.
- The halo sign is a highly specific indicator for GCA, aiding clinical decision-making.
- Clinical evolution and treatment response should be considered essential reference standards in future GCA diagnostic studies.
Abstract:
Giant cell arteritis is a common systemic vasculitis affecting the elderly, with maximum prevalence in the 7th decade of age, targeting aortic derived medium and large vessels of the neck and head. Diagnosis is established on a biopsy specimen of the temporal artery wall, through pathological confirmation of panarteritis, typically characterized by mononuclear cell infiltrate, with the 1990 ACR criteria often used in clinical practice. We present the case of a patient with a new onset headache and systemic inflammation, who did not fulfil the classical diagnostic criteria, nor did the temporal artery biopsy (TAB) provide a positive result. However, the ultrasonographical features, clinical evolution and response to corticosteroid therapy confirmed the diagnosis. This patient had bilateral presence of the halo sign on color duplex ultrasonography (CDUS), cited as a highly specific feature, when compared to the ACR criteria as a standard reference. We employed its positive likelihood-ratio (LR+) of 43 as previously estimated, while considering a low pre-test probability for a positive diagnosis (15%), to calculate a post-test probability of 88%, leading to our decision to treat him as having giant cell arteritis. Remission of the headache and rebound phenomena when tapered off steroid therapy substantially contributed to the positive diagnosis, underlining the importance of future studies needing to use clinical evolution as a reference standard.
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