Giant cell arteritis complicated by tongue necrosis and bilateral cerebellar ischaemic stroke

2021 ◽  
Vol 14 (12) ◽  
pp. e244948
Author(s):  
Emily Charlotte Rose ◽  
Liam Stuart Carroll ◽  
Sue Evans ◽  
Alice Mason
Keyword(s):  
Ischaemic Stroke ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Visual Disturbance ◽  
Temporal Artery Biopsy ◽  
Stroke Risk Factors ◽  
Temporal Arteries ◽  
Oral Pain ◽  
Oral Candida

Giant cell arteritis (GCA) typically presents with headache, scalp tenderness or visual disturbance. Other symptoms include orofacial pain, constitutional symptoms and ischaemic stroke. An 81-year-old woman with a background of type-2 diabetes and hypertension presented with headache, oral pain and right visual loss. Examination showed hypertension, nodular temporal arteries, reduced visual acuity and suspected oral candida. Inflammatory markers were raised and she was diagnosed with GCA and commenced on corticosteroids. During treatment she developed tongue ulceration, then acute vertigo and incoordination with nystagmus and ataxia. Neuroimaging confirmed bilateral, cerebellar ischaemic strokes and temporal artery biopsy was consistent with GCA. With corticosteroids and secondary prevention of stroke measures she is now functionally independent. Oral pain is an uncommon symptom of GCA and delays in recognition may lead to catastrophic consequences. Clinicians should be aware of uncommon presentations and to optimise additional ischaemic stroke risk-factors.

A case of giant cell arteritis lacking typical symptoms presenting with recurrent cerebellar infarctions: A case report and case-based review

2021 ◽  
Author(s):  
Ryuichiro Hiyama ◽  
Hiroshi Oiwa ◽  
Yukari Kanou ◽  
Shiho Nishibe ◽  
Tomoyuki Kono ◽  
...  
Keyword(s):  
Physical Examination ◽  
Ischaemic Stroke ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Protein Levels ◽  
Temporal Arteries ◽  
First Case ◽  
Case Based

ABSTRACT Giant cell arteritis (GCA) occasionally presents with ischaemic stroke. Generally, symptoms related to GCA or elevated levels of inflammation markers would be a clue for the diagnosis of GCA. However, we encountered a rare case of GCA that presented with recurrent cerebellar infarctions without symptoms related to GCA (headache, fever, or jaw claudication). Furthermore, C-reactive protein levels, measured at the time of two of the stroke attacks, were within the normal range. On physical examination, the temporal arteries were prominent and weakly pulsatile. Temporal artery ultrasonography showed halo signs, and temporal artery biopsy revealed GCA. To our knowledge, this is the first case of GCA presenting with recurrent ischaemic stroke lacking GCA features but diagnosed before death. Considering this case-based review, we suggest that GCA may have been missed in elderly patients with ischaemic stroke, especially in those with posterior circulation infarction. Therefore, physical examination of the temporal arteries, temporal artery ultrasonography, and vessel wall magnetic resonance imaging may be useful in those patients.

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 Imaging Tests in the Early Diagnosis of Giant Cell Arteritis

2021 ◽  
Vol 10 (16) ◽  
pp. 3704
Author(s):  
Diana Prieto-Peña ◽  
Santos Castañeda ◽  
Isabel Martínez-Rodríguez ◽  
Belén Atienza-Mateo ◽  
Ricardo Blanco ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Early Recognition ◽  
Imaging Modality ◽  
Imaging Techniques ◽  
Vascular Complications ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries

Early recognition of giant cell arteritis (GCA) is crucial to avoid the development of ischemic vascular complications, such as blindness. The classic approach to making the diagnosis of GCA is based on a positive temporal artery biopsy, which is among the criteria proposed by the American College of Rheumatology (ACR) in 1990 to classify a patient as having GCA. However, imaging techniques, particularly ultrasound (US) of the temporal arteries, are increasingly being considered as an alternative for the diagnosis of GCA. Recent recommendations from the European League Against Rheumatism (EULAR) for the use of imaging techniques for large vessel vasculitis (LVV) included US as the first imaging option for the diagnosis of GCA. Furthermore, although the ACR classification criteria are useful in identifying patients with the classic cranial pattern of GCA, they are often inadequate in identifying GCA patients who have the extracranial phenotype of LVV. In this sense, the advent of other imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET)/CT, has made it possible to detect the presence of extracranial involvement of the LVV in patients with GCA presenting as refractory rheumatic polymyalgia without cranial ischemic manifestations. Imaging techniques have been the key elements in redefining the diagnostic work-up of GCA. US is currently considered the main imaging modality to improve the early diagnosis of GCA.

scholarly journals Rapid visual recovery following intravenous tocilizumab in glucocorticoid resistant refractory giant cell arteritis

2019 ◽  
Vol 12 (10) ◽  
pp. e229236 ◽  
Author(s):  
Carl Richard Svasti-Salee ◽  
Susan P Mollan ◽  
Ann W Morgan ◽  
Vanessa Quick
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Rescue Therapy ◽  
Temporal Artery ◽  
High Dose ◽  
Temporal Artery Biopsy ◽  
Sustained Remission ◽  
Temporal Arteries ◽  
Multiple Pulses

A 72-year-old man presented with a short history of headache, jaw claudication, double vision, amaurosis fugax and distended temporal arteries. A diagnosis of giant cell arteritis (GCA) was confirmed on temporal artery ultrasound and temporal artery biopsy. Despite treatment with high-dose oral glucocorticoid (GC) and multiple pulses of intravenous methylprednisolone, his vision deteriorated to hand movements in one eye. 8 mg/kg intravenous tocilizumab, a humanised, recombinant anti-IL-6 receptor antibody, was administered within 48 hours of vision loss and continued monthly, resulting in marked visual improvement within days, as well as sustained remission of GCA. This case suggests a possible role for tocilizumab as a rescue therapy to prevent or recover visual loss in patients with GCA resistant to GC treatment, termed refractory GCA. Further research is required to elucidate the role of intravenous administration of tocilizumab in this setting.

scholarly journals IgG,kappa monoclonal gammopathy of unknown significance with AL amyloidosis simulating giant cell arteritis

2017 ◽  
Vol 55 (3) ◽  
pp. 179-182
Author(s):  
Valer Mihai Pompilian ◽  
Ştefăniţă Tănăseanu ◽  
Camelia Badea ◽  
Sabina Zurac ◽  
Claudiu Socoliuc ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Monoclonal Gammopathy ◽  
Temporal Artery ◽  
Al Amyloidosis ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries ◽  
First Case ◽  
Unknown Significance ◽  
Tunnel Syndrome

Abstract Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA). We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension. Muscular strength was normal. She had high ESR and CRP; in this clinical context, GCA was suspected. A gamma spike on serum protein electrophoresis raised the suspicion of monoclonal gammopathy (MG). Immunoelectrophoresis revealed monoclonal bands for IgG and kappa chains. Massive deposits of amyloid and no inflammation were found on temporal artery biopsy. Multiple myeloma and lymphoma were ruled out. A diagnosis of AL amyloidosis complicating MGUS was formulated. She did well on therapy with bortezomib, cyclophosphamide and dexamethasone. Cases published in medical literature reveal amyloidosis mimicking GCA in the setting of established MGUS. As far as we know, this is the first case of MGUS with IgG and kappa chains in which a GCA-like picture induced by amyloidosis was present from the very onset.

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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