scholarly journals Delayed Diagnosis of Giant Cell Arteritis in the Setting of Isolated Lingual Necrosis

2019 ◽  
Vol 12 ◽  
pp. 117954761985769
Author(s):  
Logan Christopher DeBord ◽  
Ilene Chiu ◽  
Nelson Eddie Liou

Background: Lingual necrosis is a rare complication of giant cell arteritis (GCA). Methods: A 77-year-old woman presented for treatment of a painful and discolored tongue, odynophagia, and dehydration refractory to antimicrobials over 2 weeks. An extensive, well-demarcated necrotic area was visualized on the anterior tongue upon admission. Leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate were present. Computed tomography angiogram of the head and neck revealed an undulated-beaded appearance of the distal internal carotid arteries and vertebral arteries bilaterally. Results: High-dose intravenous steroids were initiated for suspected vasculitis. Temporal artery biopsy confirmed the diagnosis of GCA. The patient’s condition improved and the anterior tongue was well healed at 1 month follow-up. Conclusions: An atypical presentation of GCA (eg, isolated lingual necrosis) risks a delay in diagnosis and increased morbidity. Any patient above the age of 50 years presenting with tongue necrosis, in the absence of known cause, should undergo expedited workup for GCA.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 383-384
Author(s):  
T. Kise ◽  
E. Takamasu ◽  
Y. Miyoshi ◽  
N. Yokogawa ◽  
K. Shimada

Background:Temporal artery biopsy (TAB) is the gold standard for diagnosing giant cell arteritis (GCA). However, previous studies have reported that the discordance rate of TAB is 3-45%,i.e., in unliteral TAB, GCA may be overlooked in one in five patients, approximately. Evidence as to whether bilateral TAB should be performed initially or one-sided TAB is sufficient for diagnosing GCA is lacking.Objectives:To investigate the predictors of patients with GCA in whom one-sided TAB is sufficient.Methods:The present study was a cross-sectional, single center study conducted from April 1, 2011 to July 31, 2019 at Tokyo Metropolitan Tama Medical Center. Of all consecutive GCA cases for which bilateral TAB was performed, bilaterally positive cases and unilaterally positive cases were extracted as bilateral positive group (BPG) and unilateral positive group (UPG), respectively. GCA was defined in accordance with the classification criteria of the 1990 American College of Rheumatology, and GCA was diagnosed if no other etiology was found within six months after beginning of high-dose glucocorticoid treatment. Demographic, clinical and laboratory data were obtained from the medical records, and the BPG and the UPG were compared statistically in each variable. Statistical significance was defined asp< 0.05.Results:During study, 264 biopsies were performed for 145 cases, who suspected GCA and underwent TAB. The pathological positivity rate was 26.1% (68 / 264 biopsies). Of these, 53 cases had final diagnosis of GCA, in which 43 cases were biopsy proven GCA. Thirty-seven biopsy proven GCA with bilateral TAB were enrolled; 64.9% women; mean (SD) age 75 (8.9) years; median [IQR] TAB length 17.5 [13.0,20.0] mm; headache 54.1%; jaw claudication 45.9%; scalp tenderness 16.2%; temporal artery (TA) tenderness 32.4%; TA engorgement 32.4%; TA pulse abnormality 5.4%; visual symptoms 2.7%; a fever of 38.5°C or higher 40.5%; shoulder girdle pain 48.6%; imaging of aortitis or arteritis 40.5%; median [IQR] white blood cell 9,100 [7200, 12050] /μl; median [IQR] platelet cell 37.5 [27.0, 46.3] ×104/μl; median [IQR] C-reactive protein (CRP) 10.1 [3.9, 16.5] mg/dL; erythrocyte sedimentation rate [IQR] 105 [66, 129] mm/h. Thirty-one in 37 cases were positive bilaterally while 6 in 37 cases were positive unilaterally; and the discordance rate was 16.2%. The median sample length after formalin fixation was 19.0 mm for the BPG and 14.5 mm for the UPG (p= 0.171). The parameters above were compared between UPG and BPG. Of these, only the serum CRP value (mg/dL) differed statistically between groups, and the median value of the two groups was 10.6 and 6.5, respectively (median test:p= 0.031). To predict BPG, in whom unilateral TAB is sufficient for diagnosing GCA, the cut-off value of serum CRP with a specificity of 100% and a sensitivity of 61.3% was set at 9.3 mg/dL (ROC analysis: AUC 0.726).Conclusion:When the serum CRP level is 10 mg/dL or higher in GCA suspected patients, an unilateral TAB alone was sufficient for an accurate diagnosis.References:[1]Hellmich, B, et al.Ann Rheum Dis2020;79(1):19-30.[2]Breuer, GS, et al.J Rheumatol. 2009;36(4):794-796.[3]Czyz CN, et al.Vascular2019;27(4):347-351.[4]Durling B, et al.Can J Ophthalmol2014;49(2):157-161.Figure.Comparison of median CRP levels between unilaterally positive group and bilaterally positive group.Disclosure of Interests:None declared


VASA ◽  
2005 ◽  
Vol 34 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Simon ◽  
Fritz ◽  
Amann-Vesti ◽  
Schenk Romer ◽  
Fischer ◽  
...  

A 77-year-old-man with giant cell arteritis who developed bitemporal scalp ulcerations is described. Since 1946 when Cooke et al. reported the first case of scalp necrosis there were approximately 55 cases published. Scalp ulceration is a rare complication of giant cell arteritis and occurs mainly in elderly persons, particularly women. About half of all patients were presented to dermatologists. Most of the patients (70%) had other serious complications of giant cell arteritis: blindness, gangrene of the tongue and nasal septum necrosis. Seventy percent of the cases were confirmed by a temporal artery biopsy. The necrosis were of varying extent and uni- or bilateral. Although, in most cases necrosis has been located bilaterally as in the presented case. Scalp healing was complete nearly in all patients by conservative treatment within a year. Scalp ulceration is a potentially reversible complication of giant cell arteritis which indicates extensive vessel involvement and adequate coricosteroid therapy is required and essential.


2021 ◽  
pp. 10.1212/CPJ.0000000000001083
Author(s):  
Valentina Poretto ◽  
Silvio Piffer ◽  
Valeria Bignamini ◽  
Enzo Tranquillini ◽  
Davide Donner ◽  
...  

A 74-year-old woman presented with acute worsening of six-months long history of vertigo and postural instability, with MRI evidence of cerebellar and brainstem acute infarcts. Extensive neurovascular assessment revealed a severe vascular damage with multiple stenoses and occlusions along vertebrobasilar axis (figure 1). Duplex ultrasonography showed hypoechoic halo sign along facial artery, while PET-CT highlighted increased [18F]-FDG uptake along vertebral and other larger arteries, thus allowing a diagnosis of giant cell arteritis (figure 2).1,2 Despite prompt treatment with high-dose steroids and tocilizumab, which probably made uninformative a subsequent temporal artery biopsy (figure 2), patient died of reported disability after strokes.


Diagnostics ◽  
2021 ◽  
Vol 11 (5) ◽  
pp. 879
Author(s):  
Natasja Justesen ◽  
Michael Hansen ◽  
Mads Jensen ◽  
Oliver Klefter ◽  
Jane Brittain ◽  
...  

: Giant cell arteritis (GCA) is the most common form of large vessel vasculitis. GCA is a medical and ophthalmological emergency, and rapid diagnosis and treatment with high-dose corticosteroids is critical in order to reduce the risk of stroke and sudden irreversible loss of vision. GCA can be difficult to diagnose due to insidious and unspecific symptoms—especially if typical superficial extracranial arteries are not affected. In these cases, verification of clinical diagnosis using temporal artery biopsy is not possible. This example illustrates the diagnostic value of hybrid imaging with 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography/computed tomography (2-[18F]FDG PET/CT), and the limitations of the temporal artery biopsy in bilateral vertebral GCA, causing transient ischemic attack in the visual cortex. In addition it indicates that inflammation in the artery wall can be visualized on 2-[18F]FDG PET/CT despite long term and ongoing high dose glucocorticoid treatment.


2019 ◽  
Vol 12 (10) ◽  
pp. e229236 ◽  
Author(s):  
Carl Richard Svasti-Salee ◽  
Susan P Mollan ◽  
Ann W Morgan ◽  
Vanessa Quick

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.


2019 ◽  
Vol 12 (11) ◽  
pp. e230795 ◽  
Author(s):  
Louise McDonald ◽  
Gavin Baker ◽  
Olga Kerr

An 81-year-old woman presented with an enlarging, tender ulcer on her scalp over an 8-week period, attributing it to a prior graze with garden shears. C-reactive protein and erythrocyte sedimentation rate were elevated at 87.7 mg/L and 112 mm/hour, respectively. Incisional biopsies demonstrated ulceration and full thickness necrosis with no evidence of malignancy. Vasculitis was suggested as a likely cause of such extensive necrosis and subsequent temporal artery biopsy findings were consistent with giant cell arteritis. The patient was initially treated with high-dose oral prednisolone and achieved complete healing of the scalp necrosis within 12 months, with a gradual down-titration of steroid therapy thereafter. Scalp necrosis is a rare, potentially life-threatening complication of giant cell arteritis. This case highlights the importance of considering scalp necrosis as a manifestation of giant cell arteritis when assessing scalp ulceration. Prompt diagnosis and treatment can prevent significant morbidity and potential mortality.


2012 ◽  
Vol 78 (12) ◽  
pp. 1362-1368 ◽  
Author(s):  
Irene Thomassen ◽  
Avalon N. Den Brok ◽  
Constantijn Jam Konings ◽  
Simon W. Nienhuijs ◽  
Marcel C. G. Van De Poll

Temporal artery biopsy (TAB) is the diagnostic gold standard for giant cell arteritis (GCA). GCA is treated by high-dose corticosteroids. In cases of high clinical suspicion, steroids may be administrated despite negative TAB, making TAB clinically irrelevant. We assessed the role of TAB in clinical decision-making in patients with suspected GCA and to identify factors associated with clinically irrelevant TAB. Charts of patients who underwent TAB from 2005 to 2010 were reviewed for clinical parameters potentially associated with GCA and clinically irrelevant TAB. We studied 143 patients with 99 negative (69%), 34 positive (24%), and 10 undefined (7%) TABs. Eventually 26 patients (18% of the entire cohort and 26% of the patients with a negative TAB) received steroid treatment for GCA despite negative TAB. The start of steroid treatment before TAB was associated with clinically irrelevant TABs. If clinical suspicion of GCA is high, a TAB can be considered clinically irrelevant.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1562.3-1562
Author(s):  
A. Sidhu ◽  
A. Bharadwaj ◽  
A. Nandagudi

Background:Giant cell arteritis (GCA) is an emergency. The initial treatment with high dose glucocorticoids (GC) is often started on clinical suspicion without waiting for Temporal artery biopsy (TAB) results, which can take days to be available. TAUS is a simple, non-invasive test which is readily available. However, like any other ultrasound, it is also operator dependent. A positive halo sign is the most specific abnormality seen on TAUS in GCA patients. The percentage of false positive TAUS in GCA diagnosis is low (1), but it can result in over diagnosis and unnecessary exposure to high dose GC in elderly population.Objectives:We looked at the reliability of TAUS in ruling out GCA after it was introduced within our rheumatology department one year ago.Methods:We adopted the quality improvement methodology for assessment. Retrospective data of suspected GCA patients was collected over the last two years. TAUS was introduced regularly to the investigative plan after eleven months. Two Rheumatology consultants were trained in TAUS. Results were compared before and after the introduction of ultrasound as a diagnostic tool. In collecting the data, our main focus for documentation was based on clinical symptoms, TAUS and TAB results. We aimed to increase the awareness of appropriate GCA referrals among the primary and secondary care with the support of teaching sessions.Results:From January 2018 to November 2019, 101 patients were referred to rheumatology with suspected GCA. Median age of our cohort was 72 years with male to female ratio of 1:3. 35 patients were referred in the first 11 months out of which, 10 (28.6%) were diagnosed with GCA. TAUS and TAB was done in 20% and 49% of patients respectively. 66 patients were referred in the next 12 months after TAUS was introduced. Out of 66, 14 patients (21.2%) were diagnosed as GCA. TAUS and TAB were done in 82% and 38% of the patients respectively. As listed in table 1, only 1 patient was found to have positive TAB after a negative TAUS (false negative). All of patients with positive TAUS were treated as GCA on the basis of clinical grounds, irrespective of TAB results. Despite the regular use of TAUS as a diagnostic tool in the second phase, there is a higher percentage of patients (78.8%) in which GCA was ruled out.TAUS introductionBefore regular TAUS(Jan 2018 – Nov 2018)After regular TAUS(Dec 2018 – Nov 2019)Patients referred3566GCA10 (28.6%)14 (21.2%)Not GCA25 (71.4%)52 (78.8%)TAUS done in20%82%TAB done in49%38%TAUS -ve and TAB +ve01TAUS +ve and TAB -ve/not done28Conclusion:After the routine introduction of TAUS, the percentage of patients diagnosed with GCA has declined and clinicians have been able to exclude suspected GCA diagnosis in a larger proportion of patients referred. This is noteworthy as our Rheumatologists are still in the learning phases of determining the significance of utility of TAUS. There is only a small decline in TAB frequency, which is expected to go down further in the coming years. We also noticed that the number of patients referred has almost doubled. This might be due to better education and awareness at the primary and secondary care level which was done as part of the project.References:[1]Fernández E, Monjo I, Bonilla G, et alOP0210 FALSE POSITIVES OF ULTRASOUND IN GIANT CELL ARTERITIS. SOME DISEASES CAN ALSO HAVE HALO SIGNAnnals of the Rheumatic Diseases2019;78:181Disclosure of Interests:None declared


2017 ◽  
pp. bcr-2017-219228 ◽  
Author(s):  
Roman Zuckerman ◽  
Mayur Patel ◽  
Deborah R Alpert

We describe an 80-year-old woman who presented with headaches, bilateral jaw claudication and visual symptoms. She was diagnosed with giant cell arteritis, which was confirmed by temporal artery biopsy. She was treated with high-dose prednisone followed by a slow taper, with complete resolution of symptoms. Approximately 4 years later, she developed progressively worsening renal function associated with haematuria. Serological workup revealed a high-titre perinuclear antinuclear cytoplasmic antibody and antibodies to myeloperoxidase. Renal biopsy demonstrated a pauci-immune focal crescentic glomerulonephritis. Extensive review of systems, physical exam and diagnostic studies demonstrated no evidence of extra-renal disease, and she was diagnosed with renal-limited microscopic polyangiitis. High-dose prednisone was resumed, but she refused treatment with either cyclophosphamide or rituximab due to concern for toxicity. Her prednisone dose was tapered and renal function stabilised. Our case highlights the need to recognise the successive occurrence of two distinct vasculitides in a single patient and monitor accordingly.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 95.3-95
Author(s):  
A. Sachdev ◽  
S. Dubey ◽  
C. Tiivas ◽  
M. George ◽  
P. Mehta

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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