Myeloproliferative invasion of arterial walls: Premortem diagnosis by temporal artery biopsy

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.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6534 ◽

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

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

The Laryngoscope ◽

10.1002/lary.22215 ◽

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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AB0551 The impact of temporal artery biopsy on diagnosis of giant cell arteritis in clinical practice

10.1136/annrheumdis-2017-eular.4749 ◽

2017 ◽

Author(s):

E Kaltsonoudis ◽

D Kirochristos ◽

A Bai-Papoudou ◽

P Voulgari ◽

AA Drosos

Keyword(s):

Clinical Practice ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Temporal Artery ◽

Temporal Artery Biopsy ◽

The Impact

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AB1174 TRACKING THE EFFECTS ON A CLINICAL SERVICE OF INTRODUCING ULTRASOUND FOR DIAGNOSIS OF GIANT CELL ARTERITIS: DESIGN OF A SERVICE EVALUATION USING LEAN METHODOLOGY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.646 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1877.2-1878

Author(s):

S. Mackie ◽

A. Barr ◽

A. Cracknell ◽

S. Farrell ◽

J. Parvin ◽

...

Keyword(s):

Lead Time ◽

Axillary Artery ◽

Care Pathway ◽

Temporal Artery ◽

Service Evaluation ◽

Significant Shift ◽

Temporal Artery Biopsy ◽

Prednisolone Dose ◽

Patient Feedback ◽

The Impact

Background:In our large, multi-site hospital, patients with suspected GCA are started promptly on high-dose prednisolone but until 2019, patients waited for temporal artery biopsy (TAB) until the GCA diagnosis could be confirmed (“GCA”) or refuted (“not-GCA”). Reports of the impact of introducing temporal and axillary artery ultrasound (TAUS) have mainly come from smaller hospitals. Agreement between TAUS and TAB has been reported by others with a Cohen’s kappa of 0.35 [1] and 0.40 [2]. We used Lean methodology to identify metrics across 5 key domains: delivery, quality, service, morale and cost.Objectives:To design metrics for a service evaluation to measure impact of introducing TAUS, and to test their feasibility of measurement within routine care.Methods:Our primary driver was time from presenting to our service to diagnostic confirmation (lead time). Pathway mapping, value stream mapping and a driver diagram identified key ideas for improvement.We chose to measure: Delivery (mean lead time for each month), Quality (proportion of patients with GCA and positive TAB/TAUS; total (cumulative) prednisolone dose in patients with not-GCA, Service (patient feedback), Morale (staff feedback) and Cost (number of patients; cost of tests per patient; overall costs). We plotted these by month on run charts and defined a significant shift as 6 consecutive monthly values below baseline median. Cohen’s kappa was calculated using GraphPad QuickCalcs.Results:Routine TAUS for suspected GCA was introduced from January 2019, alongside a multidisciplinary team monthly meeting. TAUS was done a median of 2.5 days from referral. Agreement between TAB and TAUS results was good (Table 1). The run chart showed a significant shift in our Delivery (median lead time fell from 28.7 days to 21 days after introduction of ultrasound) and both Quality metrics (proportion of GCA with positive TAB/TAUS increased from 29% to 69%; total prednisolone dose for not-GCA fell from 1.335g to 0.846g).Table 1.Concordance between temporal and axillary artery ultrasound (TAUS) and temporal artery biopsy (TAB) in scans performed through 2019. Cohen’s weighted kappa 0.59 (including equivocal results as separate category).TAUS positiveTAUS negativeTAUS equivocalTAB positive1411TAB negative5275TAB equivocal030Within Costs, average per-patient costs of TAB/TAUS declined from £1004/patient to £792/patient, but total referrals for TAB/TAUS increased from 6/month to 10/month, increasing overall costs. Staff and patient feedback (Service, Morale) revealed that further improvements to the care pathway were needed to manage the additional complexity.Conclusion:Lean methodology identified multiple metrics for evaluating the impact of TAUS on our service. Introducing TAUS improved Delivery and Quality, but measuring Costs, Morale and Service helped identify unintended consequences. Concordance between TAUS and TAB was good. We plan to continue to improve and monitor the care pathway based on our multi-stakeholder feedback.References:[1]Luqmani et al., HTA 2016[2]Mukhtyar et al., Clin Rheum 2019Disclosure of Interests:Sarah Mackie Grant/research support from: Roche (attendance of EULAR 2019; co-applicant on research grant), Consultant of: Sanofi, Roche/Chugai (monies paid to my institution not to me), Andrew Barr: None declared, Alison Cracknell: None declared, Shannon Farrell: None declared, Jimmy Parvin: None declared, Ajay Patil: None declared, Ian Simmons: None declared, Kate Smith Grant/research support from: Medical education grants from Sanofi and Biogen, Speakers bureau: Novartis, Andrea Sweeting: None declared, Max Troxler: None declared, Tara Webster: None declared, Richard Wakefield Speakers bureau: Novartis, Janssen, GE

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

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.055 ◽

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.

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