Metaplastic Ossification of the Temporal Artery with Osteoclast-Like Giant Cells: A Mimicker of Giant Cell (Temporal) Arteritis

2017 ◽  
Vol 27 (3) ◽  
pp. e99-e103 ◽  
Author(s):  
Miroslav Sekulic ◽  
Alexander M. Truskinovsky
Keyword(s):  
Giant Cell ◽  
Temporal Arteritis ◽  
Temporal Artery ◽  
Giant Cells ◽  
Temporal Artery Biopsy ◽  
Tunica Media ◽  
Luminal Narrowing ◽  
Stage Renal Disease ◽  
Metaplastic Ossification ◽  
Medial Calcification

Purpose To describe a patient presenting with suspected giant cell (temporal) arteritis (GCA) in whom subsequent temporal artery biopsy showed luminal narrowing by medial calcification, metaplastic ossification, and fibrointimal proliferation, consistent with calciphylaxis. Methods A 55-year-old man with end-stage renal disease presented with unilateral loss of vision and elevated erythrocyte sedimentation rate and was initially treated as though he had GCA; however, a subsequent temporal artery biopsy showed marked luminal narrowing by medial calcification, metaplastic ossification, and fibrointimal proliferation, consistent with calciphylaxis. In addition, the tunica media of the affected artery contained multinucleate giant cells, but these represented osteoclasts and foreign body giant cells reacting to calcium, rather than a part of GCA. Results This is a rare report of metaplastic ossification and the finding of non-GCA-related giant cells in the tunica media of the temporal artery, thus representing a clinical and histopathologic mimicker of GCA. Conclusions The clinical differential diagnosis of GCA includes other etiologies that can present similarly; however, temporal artery biopsy can discern the underlying pathology. Importantly, the identification of giant cells is not required for the diagnosis of GCA, and likewise, as our case shows, the finding of giant cells in the wall of a temporal artery does not always imply a diagnosis of GCA.

Introducing an ANP-led temporal artery biopsy service for patients with suspected giant cell arteritis

2021 ◽  
Vol 30 (9) ◽  
pp. 512-519
Author(s):  
John Cooper
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Nurse Practitioner ◽  
Temporal Arteritis ◽  
Temporal Artery ◽  
Medical Emergency ◽  
Temporal Artery Biopsy ◽  
Sight Loss

Giant cell arteritis (GCA) is an uncommon autoimmune inflammatory vasculopathy that can lead to the destruction and occlusion of various arteries that consequently can cause serious complications such as stroke or sight loss. It is seen as a medical emergency. The most commonly affected vessel in GCA is the temporal artery in the side of the head, hence the condition is sometimes also referred to as ‘temporal arteritis’. This article discusses the introduction of an advanced nurse practitioner-led temporal artery biopsy service.

scholarly journals Polymialgia Rheumatica, Giant Cell Arteritis or Both?

2018 ◽  
Vol 15 (6) ◽  
pp. 51-58
Author(s):  
Adina Cociorvei ◽  
Mădălina Ababei
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Arteritis ◽  
Morning Stiffness ◽  
Systemic Vasculitis ◽  
Diagnostic Algorithm ◽  
Signs And Symptoms ◽  
Temporal Artery ◽  
Clinical Suspicion ◽  
Temporal Artery Biopsy

AbstractGiant cell arteritis (GCA), or temporal arteritis, is the most common systemic vasculitis, and the greatest risk factor for developing GCA is aging. The disease almost never occurs before age 50, and its incidence rises steadily thereafter, peaking between ages 70 to 79, the risk of development being two times higher in women.Polymialgia rheumatica (PMR) is an inflammatory rheumatic condition characterized clinically by aching and morning stiffness at the shoulders, hip girdle, and neck. PMR is almost exclusively a disease of adults over the age of 50, with a prevalence that increases progressively with advancing age. The peak incidence of PMR occurs between ages 70 and 80, the same as in the case ofGCA. PMRis 2-3 times more common in women than in men.PMR is two to three times more common than GCA and occurs in about 50% of patients with GCA. The percentage of patients with PMR who experience GCA at some point varies widely in reported series ranging from 5 to 30 percent. PMR can precede, accompany or follow GCA. The diagnostic in the case of PMR is made first of all on clinical features, in the patients in whom another disease to explain the findings is not present. For GCA we must follow the diagnostic algorithm presented below (figure 1) and keep in mind that a negative result for temporal artery biopsy does not exclude the diagnostic if clinical suspicion of GCA is highWe present the case of a 81 year-old male with signs and symptoms from both conditions, PMR and GCA.

THU0291 IS GIANT CELL ARTERITIS REALLY GIANT CELL ARTERITIS? A HISTOLOGICAL REVIEW OF TEMPORAL ARTERY BIOPSIES FROM A UK DISTRICT GENERAL HOSPITAL

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 373.1-373
Author(s):  
S. K. Amar ◽  
D. Christidis ◽  
G. Kousparos ◽  
M. Lloyd
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Infiltrate ◽  
Temporal Artery ◽  
Giant Cells ◽  
The Body ◽  
British Society ◽  
Temporal Artery Biopsy ◽  
Intimal Thickening ◽  
Inflammatory Lesions

Background:Despite the advent of newer imaging techniques, temporal artery biopsy (TAB) retains a key role in the diagnosis of giant cell arteritis (GCA). The classical histological description of GCA is that of granulomatous lesions characterized by a transmural inflammatory infiltrate1. Giant cells are typically noted in the internal elastic lamina. Vascular remodeling and structural changes are also frequently described, with intimal hyperplasia or fragmentation, fibrosis and calcifications1.Objectives:To identify the type and location of the inflammatory lesions in TAB-positive cases of GCA.Methods:We conducted a retrospective analysis of all TABs undertaken at our unit between 2011- 2018 with clinical record review. TABs were performed by vascular, ophthalmology and ENT teams.Results:379 TABs were reviewed of which 68 (17.9%) were reported as positive and 10 (2.6%) were equivocal (presence of fragmentation and intimal thickening). Of the TAB-positive cases, 43 (63.2%) were greater than 1cm in keeping with the British Society for Rheumatology guidance and 65 (95.6%) were biopsies in patients on corticosteroids at the time of procedure. The following tables demonstrate the frequency of the type and location of the inflammatory lesions detected in TAB-positive cases of GCA.Type of inflammatory lesionFrequencyChronic inflammatory infiltrate (lymphocytes, macrophages, plasma cells)66Giant cells41Intimal thickening22Intimal fragmentation33Location of inflammatory infiltrateFrequencyFull thickness32Intima only7Intima and Media only3Media only7Media and Adventitia only8Adventitia only4Intima and Adventitia only3Not recorded4Conclusion:Only 60% of our TAB-positive biopsies had giant cells present. Although perhaps surprisingly low, this finding is similar to other studies1,2. It emphasises the need to review the body of a report as well the conclusion. Other non-giant cell features present in positive reported biopsies may suggest a less certain diagnosis and prompt clinical review. There was considerable variablity in the style of reporting. With no standardised scoring system in place, the variable spectrum of inflammation and differences in reporting, there is the potential for inconsistencies amongst pathologists in interpreting and recording TAB results. Consistent reporting templates and close collaboration between rheumatologists and pathologists is needed to help correlate clinical, laboratory and imaging findings.References:[1]Cavazza A, Muratore F, Boiardi L, Restuccia G, Pipitone N, Pazzola G, et al. Inflamed temporal artery: histologic findings in 354 biopsies, with clinical correlations. Am J Surg Pathol. 2014;38(10):1360-70.[2]Hernandez-Rodriguez J, Murgia G, Villar I, Campo E, Mackie SL, Chakrabarty A, et al. Description and Validation of Histological Patterns and Proposal of a Dynamic Model of Inflammatory Infiltration in Giant-cell Arteritis. Medicine (Baltimore). 2016;95(8):e2368.Disclosure of Interests:Soha Khaled Amar: None declared, Dimitrios Christidis: None declared, George Kousparos: None declared, MARK LLOYD Speakers bureau: £700 into department fund

scholarly journals 051 Overlapping syndrome of giant cell arteritis and ANCA-associated vasculitis complicated by severe axonal neuropathy: a case report

2019 ◽  
Vol 90 (e7) ◽  
pp. A17.1-A17
Author(s):  
Laura Perju-Dumbrava ◽  
Fariha Islam ◽  
Rohitha Makonahalli ◽  
Catriona McLean ◽  
Hedley Griffiths ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Axonal Neuropathy ◽  
Temporal Artery ◽  
Giant Cells ◽  
Temporal Artery Biopsy ◽  
Vasculitic Neuropathy ◽  
Prednisolone Dose ◽  
Anca Associated Vasculitis ◽  
Peripheral Nerve Involvement

CaseA 61-year- old woman presented with what was thought to be a refractory, temporal artery biopsy-proven giant cell arteritis (GCA). After 4 months of therapy she was unable to get the prednisolone dose below 40 mg/day, so weekly subcutaneous Tocilizumab was introduced but with minimal response after 8 weeks. She was then admitted to hospital with a severe rapidly progressive length dependent sensorimotor peripheral neuropathy. Nerve conduction studies showed predominantly an axonal neuropathy with multiple pseudo-conduction blocks.Sural nerve and gastrocnemius biopsies revealed a necrotising vasculitis with numerous giant cells and active axonal degeneration. She proved to be ANCA-PR3 positive, without other systemic manifestations. ANCA-associated vasculitic neuropathy was diagnosed and she was treated with two 1g Rituximab infusions, 2 weeks apart.On follow-up after a month, she had regained some strength but still required a wheelchair for mobility. Further Rituximab infusion after 6 months is planned; prednisolone had been successfully weaned to 10 mg/day.ConclusionPeripheral nerve involvement, which is relatively common in ANCA-associated vasculitis, has also been reported in 14% of GCA cases. But ANCA-PR3 positivity is rare in biopsy-proven GGA, with only a handful of well-documented cases.Heightened suspicion of an alternative diagnosis in the face of an unusual clinical course (lack of steroid response and appearance of small vessel vasculitic symptoms for what is accepted to be a large vessel vasculitis) is critical and our experience highlights the important fact that a diagnosis of one of these disorders does not preclude the subsequent diagnosis of the other.

Pathological features of temporal arteries in patients with giant cell arteritis presenting with permanent visual loss

2008 ◽  
Vol 68 (1) ◽  
pp. 84-88 ◽  
Author(s):  
D Chatelain ◽  
P Duhaut ◽  
J Schmidt ◽  
R Loire ◽  
S Bosshard ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Arterial Occlusion ◽  
Temporal Artery ◽  
Giant Cells ◽  
Elastic Membrane ◽  
Temporal Artery Biopsy ◽  
Pathological Features ◽  
Arterial Lumen

Background:Permanent visual loss (PVL) is the most feared complication of giant cell arteritis (GCA), and its risk factors are still unclear.Objectives:The aim of our study was to assess the pathological features predictive of PVL on temporal artery biopsy (TAB) specimens in patients with GCA.Methods:The slides of 391 TAB specimens from patients with GCA were reviewed by two pathologists without clinical information.Results:A total of 29 patients (26 females and 3 males, mean age 78.3 years) presented with unilateral PVL at the onset of the disease, and 362 patients (258 females, 104 males, mean age 74.7 years), did not. The pathological features strongly predictive for PVL were the presence (p = 0.003), number (p = 0.001) and aggregates of giant cells (p = 0.001), presence of plasmocytes (p = 0.002), thickened intima (p = 0.007), neoangiogenesis (p = 0.001) and degree of arterial occlusion (p = 0.006). Presence of neutrophils, eosinophils, parietal necrosis, calcification in the arterial wall and disruption of the internal elastic membrane were similar in both groups. Total obstruction of the arterial lumen by a thrombus, intensity of the inflammatory cells infiltration and inflammation of small vessels, nerves and veins surrounding the temporal artery were not associated with blindness. In multivariate analysis, only giant cells remained significantly associated with PVL.Conclusion:Giant cells are strongly associated with PVL, with a significant gradient between great risk and large number of giant cells. However, PVL was neither associated with the intensity of the inflammatory infiltrate, nor with the presence of arterial thrombosis.

scholarly journals P040 An unusual case of giant cell arteritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson
Keyword(s):  
Inflammatory Markers ◽  
Illicit Drugs ◽  
Unusual Case ◽  
Temporal Arteritis ◽  
White Cell Count ◽  
Healthcare Providers ◽  
Temporal Artery ◽  
Low Grade ◽  
Temporal Artery Biopsy ◽  
The Right

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.

scholarly journals OP0150 WHAT IS THE ROLE OF TEMPORAL ARTERY BIOPSY IN GIANT CELL ARTERITIS FAST-TRACK PATHWAYS WHEN TEMPORAL ARTERY ULTRASOUND IS NEGATIVE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Sensitivity And Specificity ◽  
Fast Track ◽  
Temporal Artery ◽  
Clinical Findings ◽  
Specimen Size ◽  
Temporal Artery Biopsy ◽  
Duration Of Treatment ◽  
Relevant Role

Background:A number of centres are now running fast track pathways for diagnosis and management of Giant cell arteritis with ultrasound as the first port of call for diagnosis1. Temporal artery biopsies (TABs) have become the second line of investigation, and it is unclear how useful TAB is in this setting.Objectives:This study looked at accuracy of Temporal artery biopsy (TAB) in patients with suspected Giant Cell arteritis (GCA) with negative/inconclusive ultrasound (U/S) and how duration of treatment on steroids prior to these investigations and arterial specimen size affected it.Methods:Prospective study of all patients with suspected GCA referred for TAB when U/S was negative or inconclusive, as part of the local fast-track pathway (Coventry). Database included clinical findings, serological work up, U/S and TAB results and treatment. Sensitivity and specificity of U/S and TAB was calculated and compared based on duration of treatment with steroids.Results:One hundred and nine patients were referred for TAB via Coventry fast-track-pathway. The sensitivity of U/S in this cohort of patients was 9.08% and specificity was 93.33%. After 3 days of steroid this was 0% and 100% respectively. For TAB when done within 10 days of starting steroids, this was 65% and 87.5% respectively. After 20 days of steroids this was 0 % and 100%. The sensitivity and specificity was 20% and 85% when arterial specimen size was 11-15mm and 47% and 100% when specimen size was 16 mm or more. Sensitivity and specificity of U/S of 644 suspected GCA patients was 48% and 98%.Conclusion:Our study demonstrates that TAB plays a relevant role in GCA fast-track-pathways, when U/S is negative/inconclusive. TAB was more sensitive than U/S in this cohort of patients, but overall sensitivity of U/S was higher when calculated for all patients suspected with GCA. Both remain useful tests if performed early. TAB specimen size should ideally be 16mm or more and done within 10 days of starting steroids.References:[1]Jonathan Pinnell, Carl Tiivas, Kaushik Chaudhuri, Purnima Mehta, Shirish Dubey, O38 The diagnostic performance of ultrasound Doppler in a fast-track pathway for giant cell arteritis,Rheumatology, Volume 58, Issue Supplement_3, April 2019, kez105.036,https://doi.org/10.1093/rheumatology/kez105.036Disclosure of Interests:None declared

Evaluating the Usefulness and Timing of the Temporal Artery Biopsy for the Diagnosis of Giant Cell Arteritis

2011 ◽  
Vol 121 (S5) ◽  
pp. S264-S264
Author(s):  
Stephen V. Tornabene ◽  
Raymond Hilsinger ◽  
Raul M. Cruz
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Temporal Artery Biopsy
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