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.

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

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 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

The sensitivity of temporal artery biopsy in the detection of giant cell arteritis is independent of tissue length

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S28-S29
Author(s):  
H J Hurley ◽  
P Q Deb
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Average Length ◽  
Statistical Significance ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries ◽  
Biopsy Specimens ◽  
Two Samples ◽  
Left And Right

Abstract Introduction/Objective Giant cell arteritis (GCA) is the most common vasculitis of the elderly, and the most common primary systemic vasculitis overall, with an annual incidence of 200/million. The long term sequelae, namely vision loss and stroke, are permanent and devastating. While GCA is often treated empirically based on clinical presentation, panarteritis on temporal artery biopsy is required for diagnosis. However, these biopsies have the tendency to be falsely negative due to skip lesions, a common feature of GCA. Therefore, we set out to determine whether longer biopsy specimens were more sensitive in the detection of GCA. Methods/Case Report A census of temporal artery biopsies performed with the indication of clinical symptoms of GCA was taken at our institution. The patient age, sex, biopsy laterality, biopsy length, and pathological diagnosis were recorded for each cataloged sample. Statistical significance of difference in biopsy length was tested using an unpaired t-test in R 4.1.0. Results (if a Case Study enter NA) A total of 114 temporal artery specimens were biopsied from 94 different patients with the indication of GCA and assigned a definitive positive or negative diagnosis. Of the 94 patients, 54 were female and 40 were male. Of the total pathological specimens, 11 were positive and 103 were negative. The overall average length of biopsy specimens was 2.13 cm with a standard deviation of 0.65 cm. The average positive biopsy was 2.26 cm long, and the average negative was 2.12 cm, an insignificant difference (0.14 cm, t = 0.7, p = 0.43). In 25 patients, biopsies were taken from both the left and right temporal arteries. Of those patients, 2 were positive for GCA and the remaining 23 were negative. Interestingly, the biopsy result in every case was identical between the left and right samples; we found no instances of pathological evidence of GCA in only one of the two samples from the same patient. Conclusion According to data taken at our institution, there is no indication to lengthen the biopsy requirements from the existing 1.5 cm. However, we have demonstrated evidence that it may be unnecessary to biopsy both temporal arteries in a single patient. Larger studies would be required to confirm our findings.

scholarly journals Full-field optical coherence tomography for the diagnosis of giant cell arteritis

2020 ◽  
Author(s):  
Thomas Maldiney ◽  
Hélène Greigert ◽  
Laurent Martin ◽  
Emilie Benoit ◽  
Catherine Creuzot-Garcher ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Machine Intelligence ◽  
Vasa Vasorum ◽  
Temporal Artery Biopsy ◽  
Optical Coherence ◽  
Gabor Filtering ◽  
Full Field

AbstractHistopathological examination of temporal artery biopsy (TAB) remains the gold standard for the diagnosis of giant cell arteritis (GCA) but is associated with essential limitations that emphasize the need for an upgraded pathological process. This study pioneered the use of full-field optical coherence tomography (FF-OCT) for rapid and automated on-site pathological diagnosis of GCA. Sixteen TABs (12 negative and 4 positive for GCA) were selected according to major histopathological criteria of GCA following hematoxylin-eosin-saffron-staining for subsequent acquisition with FF-OCT to compare structural modifications of the artery cell wall and thickness of each tunica. Gabor filtering of FF-OCT images was then used to compute TAB orientation maps and validate a potential automated analysis of TAB sections. FF-OCT allowed both qualitative and quantitative visualization of the main structures of the temporal artery wall, from the internal elastic lamina to the vasa vasorum and red blood cells, unveiling a significant correlation with conventional histology. FF-OCT imaging of GCA TABs revealed destruction of the media with distinct remodeling of the whole arterial wall into a denser reticular fibrous neo-intima, which is distinctive of GCA pathogenesis and accessible through automated Gabor filtering. Rapid on-site FF-OCT TAB acquisition makes it possible to identify some characteristic pathological lesions of GCA within a few minutes, paving the way for potential machine intelligence-based or even non-invasive diagnosis of GCA.

scholarly journals The impact of Temporal Artery Biopsy for the diagnosis of Giant Cell Arteritis in clinical practice in a tertiary University Hospital

2019 ◽  
Author(s):  
E Kaltsonoudis ◽  
E Pelechas ◽  
A Papoudou-Bai ◽  
E.T. Markatseli ◽  
M Elisaf ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Laboratory Data ◽  
Unknown Origin ◽  
Temporal Artery ◽  
University Hospital ◽  
Temporal Artery Biopsy ◽  
Acute Phase Reactants ◽  
Anemia Of Chronic Disease ◽  
The Impact

ABSTRACTBackgroundTemporal artery biopsy (TAB) is useful in assisting with giant cell arteritis (GCA) diagnosis but lacks sensitivity. The aim of our study was to assess the diagnostic impact of TAB histology in patients with suspected GCA on hospital admission.MethodsA prospectively maintained database was queried for all TABs performed between 1-1-2000 until 31-12-2017 at the University Hospital of Ioannina. Thus, inclusion criteria were made on the grounds of every patient that underwent a TAB during the above-mentioned period, regardless of demographic, clinical and laboratory data.ResultsTwo hundred forty-five TABs were included (149 females and 96 males), with a mean age of 64.5 (±3.5) years. The mean symptoms duration until admission to the hospital was 8.6 (±1.3) weeks and all had elevated acute phase reactants on admission. The reasons of admission were fever of unknown origin (FUO) in 114 (46.5%) patients, symptoms of polymyalgia rheumatica (PMR) in 84 (34.3%), new headache in 33 (13.5%), anemia of chronic disease (ACD) in 8 (3.32%) and eye disturbances in 6 (2.5%) patients. Positive results were found in 49 (20%) TABs. More specifically, in 14% of patients with FUO, 21% in those with PMR, while in patients with a new headache the percentage was 27%. Finally, 5 out of 6 (83.3%) of patients with ocular symptoms and only one (12.5%) of those suffering from ACD. Visual manifestations and FUO are correlated with a positive TAB.ConclusionIt seems that TAB is useful in assisting with GCA diagnosis, but lacks sensitivity.

Temporal artery biopsy for giant cell arteritis: an audit of 471 cases – what have we learnt?

2015 ◽  
Vol 53 (10) ◽  
pp. e44
Author(s):  
J. Sloane ◽  
N. Rice ◽  
E. Kergozou ◽  
P. Chanyarungrojn ◽  
S. Walsh
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Temporal Artery Biopsy
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