Review: What Is the Current Evidence for Disease Subsets in Giant Cell Arteritis?

Geest, Kornelis S M van der; Sandovici, Maria; Sleen, Yannick van; Sanders, Jan-Stephan; Bos, Nicolaas A.; Abdulahad, Wayel H.; Stegeman, Coen A.; Heeringa, Peter; Rutgers, Abraham; Kallenberg, Cees G. M.; Boots, Annemieke M. H.; Brouwer, Elisabeth

doi:10.1002/art.40520

Cited by 60 publications

(47 citation statements)

References 99 publications

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“…Patients with overlapping disease also had a high prevalence of laboratory abnormalities and constitutional symptoms. Similar to prior studies , patients with LV involvement, including those with overlapping disease, were less likely than patients without LV involvement to have visual changes.…”

Section: Discussionsupporting

confidence: 83%

“…With the widespread adoption of vascular imaging into clinical practice, clinical variability among patients with GCA is increasingly being recognized. Previous studies have shown that patients with GCA can differ in their systemic inflammatory response, extent of arterial involvement, and treatment response , and suggest that there are distinct subgroups of GCA with potentially divergent disease etiology. Identifying subgroups may lead to stratified clinical decision‐making and enable research into differences in disease pathology.…”

Section: Discussionmentioning

confidence: 99%

“…Traditionally, GCA was thought to be confined to the cranial arteries; however, many patients also have evidence of large vessel (LV) involvement . GCA patients with LV involvement often present with different clinical features than are found in patients with cranial GCA, but the extent to which patients have overlapping versus distinct cranial and extracranial disease is not well characterized .…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic Assessment Strategies and Disease Subsets in Giant Cell Arteritis: Data From an International Observational Cohort

Gribbons

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Craven

et al. 2020

Arthritis & Rheumatology

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Objective Diagnostic assessment in giant cell arteritis (GCA) is rapidly changing as vascular imaging becomes more available. This study was undertaken to determine if clinical GCA subsets have distinct profiles or reflect differential diagnostic assessments. Methods Patients were recruited from an international cohort and divided into 4 subsets based on a temporal artery (TA) abnormality (positive TA biopsy [TAB] or halo sign on TA ultrasound [TA‐US]) and/or evidence of large vessel (LV) involvement on imaging: 1) both TA abnormality and LV involvement (TA+/LV+ GCA); 2) TA abnormality without LV involvement (TA+/LV− GCA); 3) LV involvement without TA abnormality (TA−/LV+ GCA); and 4) clinically diagnosed GCA without LV involvement or TA abnormality (TA−/LV− GCA). Results Nine hundred forty‐one patients with GCA were recruited from 72 international study sites. Most patients received multiple forms of diagnostic assessment, including TAB (n = 705 [75%]), TA‐US (n = 328 [35%]), and LV imaging (n = 534 [57%]). Assessment using TAB, TA‐US, and LV imaging confirmed the diagnosis of GCA in 66%, 79%, and 40% of cases, respectively. GCA subsets had distinct profiles independent of diagnostic assessment strategies. TA+/LV− were the most common subset (51%), with a high burden of cranial ischemia. Those in the TA−/LV− subset (26%) had a high prevalence of cranial ischemia and musculoskeletal symptoms. Patients in the TA−/LV+ subset (12%) had prevalent upper extremity vascular abnormalities and a low prevalence of vision loss, and those in the TA+/LV+ subset (11%) were older and had a high prevalence of cranial ischemia, constitutional symptoms, and elevated acute‐phase reactant levels. Conclusion Vascular imaging is increasingly incorporated into the diagnostic assessment of GCA and identifies clinical subsets of patients based on involvement of temporal and extracranial arteries.

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Section: Discussionsupporting

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Diagnostic Assessment Strategies and Disease Subsets in Giant Cell Arteritis: Data From an International Observational Cohort

Gribbons

Ponte

Craven

et al. 2020

Arthritis & Rheumatology

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“…Ocular ischaemia is a feared complication of GCA 1. Laboratory testing often reveals systemic inflammation, that is, high C-reactive protein (CRP) levels, anaemia and thrombocytosis 2…”

Section: Introductionmentioning

confidence: 99%

Novel ultrasonographic Halo Score for giant cell arteritis: assessment of diagnostic accuracy and association with ocular ischaemia

et al. 2020

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ObjectivesUltrasound of temporal and axillary arteries may reveal vessel wall inflammation in patients with giant cell arteritis (GCA). We developed a ultrasound scoring system to quantify the extent of vascular inflammation and investigated its diagnostic accuracy and association with clinical factors in GCA.MethodsThis is a prospective study including 89 patients suspected of having GCA, of whom 58 had a confirmed clinical diagnosis of GCA after 6 months follow-up. All patients underwent bilateral ultrasound examination of the three temporal artery (TA) segments and axillary arteries, prior to TA biopsy. The extent of vascular inflammation was quantified by (1) counting the number of TA segments and axillary arteries with a halo and (2) calculating a composite Halo Score that also incorporated the thickness of each halo.ResultsHalo counts and Halo Scores showed moderate diagnostic accuracy for a clinical diagnosis of GCA. They correlated positively with systemic inflammation. When compared with the halo count, the Halo Score correlated better with C-reactive protein (CRP) levels and allowed to firmly establish the diagnosis of GCA in more patients. Higher halo counts and Halo Scores were associated with a higher risk of ocular ischaemia. They allowed to identify subgroups of patients with low risk (≤5%) and high risk of ocular ischaemia (>30%).ConclusionsUltrasound halo scoring allows to quantify the extent of vascular inflammation in GCA. Extensive vascular inflammation on ultrasound may provide strong diagnostic confirmation and associates with ocular ischaemia in GCA.

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“…temporal headache, scalp tenderness and jaw claudication) before considering a GCA diagnosis, although these "classic" symptoms are by no means universal in GCA [7]. A recent clinical review by van der Geest et al classified GCA as a clinically and immunologically heterogeneous autoimmune disease, dividing it into three subsets: (i) systemic inflammation, (ii) vasculitis of large systemic arteries and (iii) polymyalgia rheumatica [8]. Related to the specific outcome of visual loss, studies have suggested that the risk of visual loss is higher in GCA patients with symptoms such as jaw claudication and in patients who do not have the typical temporal headache [2].…”

Section: Introductionmentioning

confidence: 99%

Characteristics of patients with giant cell arteritis who experience visual symptoms

et al. 2019

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Permanent vision loss is one of the most serious complications of giant cell arteritis (GCA) and therefore prompt diagnosis is paramount. However, diagnosis of GCA remains challenging due to its frequently non-specific presentation. Our aim was to identify differences in the characteristics of GCA patients with, and without, current visual symptoms. A cross-sectional survey was mailed to patients with a GCA Read code entered in their GP electronic medical record. Responders were categorised as those currently reporting a visual symptom or not. We compared general and GCA-specific characteristics in these two groups. The association of diagnostic delay with subsequent experience of visual symptoms was examined using unadjusted and adjusted linear regression analysis. 318 GCA patients responded to the survey (59.6%). Responders were predominantly female (69.8%), with a mean age of 73.7 years (SD 8.2). 28% reported current visual symptoms. There was no statistically significant difference in the general characteristics between those with and without visual symptoms. Of GCAspecific characteristics, pre-GCA diagnosis of diplopia (p = 0.018), temporary (p ≤ 0.001) or permanent visual problems (p = 0.001) and hoarseness (p = 0.004) were more common among those reporting current visual symptoms. There was no association between the extent of diagnostic delay and reporting of current visual symptoms. Though we found few characteristics to distinguish between GCA patients with or without current visual symptoms, diagnostic delay was not associated with current visual symptoms. Our findings highlighted the continued difficulty for clinicians to identify GCA patients at the highest risk of visual complications.

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Review: What Is the Current Evidence for Disease Subsets in Giant Cell Arteritis?

Cited by 60 publications

References 99 publications

Diagnostic Assessment Strategies and Disease Subsets in Giant Cell Arteritis: Data From an International Observational Cohort

Diagnostic Assessment Strategies and Disease Subsets in Giant Cell Arteritis: Data From an International Observational Cohort

Novel ultrasonographic Halo Score for giant cell arteritis: assessment of diagnostic accuracy and association with ocular ischaemia

Characteristics of patients with giant cell arteritis who experience visual symptoms

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