scholarly journals AB0371 PATIENTS WITH PROLONGED SYMPTOMS BEFORE GCA DIAGNOSIS DO NOT INCUR HIGHER RATES OF VISUAL LOSS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1212.1-1212
Author(s):  
V. Yang ◽  
C. Mcmaster ◽  
C. Owen ◽  
J. Leung ◽  
R. Buchanan ◽  
...  

Background:Giant cell arteritis (GCA), if left untreated, confers the threat of serious cranial ischaemic complications including permanent visual loss. Although achieving a prompt and accurate diagnosis remains challenging, early diagnosis is viewed as being paramount in preventing significant morbidity.1 This raises the question of whether GCA patients are at greater risk of developing visual sequelae if there is a longer window between symptom onset and presentation.Objectives:To compare the frequency of lasting visual loss in patients diagnosed with GCA undergoing temporal artery biopsy (TAB) within three months and after three months of symptom onset.Methods:Patients who underwent TAB from January 2011 to November 2020 were identified from the pathology database of an Australian rheumatology referral centre. The diagnosis of GCA was established for each patient based on either positive TAB or, in the setting of negative TAB, clinical diagnosis by a rheumatologist. Baseline demographics, symptoms and major confounders – including age, sex, history of polymyalgia rheumatica or inflammatory arthritis, headache, jaw pain, fatigue, temporal artery tenderness or diminished pulse, and number of 1990 American College of Rheumatology (ACR) classification criteria for GCA2 fulfilled – were manually extracted from electronic medical records, as was the duration between onset of GCA symptoms and TAB, and the presence of visual loss before and after TAB. Logistic regression log-likelihood tests were used to examine the two cohorts presenting before and after three months.Results:There were 167 patients who underwent TAB during the study period with accessible clinical information. Of these, 31 (19%) had a delayed presentation of greater than three months from symptom onset. There were no statistical differences in patient demographics between the two groups (Table 1). No patients with delayed presentation experienced lasting, objective visual loss. In contrast, there were three cases in the cohort of patients who presented more promptly; these included two patients who developed permanent unilateral blindness, and one who experienced unilateral vision loss with some improvement at three months of follow-up.Table 1.Patient characteristics by time from symptom onset to TAB.Presentation <3 monthsPresentation ≥3 monthsp-valueAge (years)73.45±10.0669.84±10.750.080Female92 (67.65%)20 (64.52%)0.738History of polymyalgia rheumatica23 (16.91%)4 (12.90%)0.586History of inflammatory arthritis6 (4.41%)2 (6.45%)0.633Headache110 (80.88%)23 (74.19%)0.406Jaw pain37 (27.21%)5 (16.13%)0.206Fatigue28 (20.59%)6 (19.35%)0.878Temporal artery tenderness or diminished pulse46 (33.82%)11 (35.48%)0.860ACR classification criteria2.83±0.992.58±0.890.199Conclusion:GCA patients with a lengthier course of symptoms before diagnosis did not experience any enduring visual loss. This may reflect a pattern of more aggressive disease leading to earlier presentation, but further study should explore whether longer symptom duration before diagnosis necessitates a higher degree of clinical concern.References:[1]Font C, Cid MC, Coll-Vinent B, López-Soto A, Grau JM. Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis. Br J Rheumatol. 1997 Feb;36(2):251-4. doi: 10.1093/rheumatology/36.2.251. PMID: 9133940.[2]Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990 Aug;33(8):1122-8. doi: 10.1002/art.1780330810. PMID: 2202311.Disclosure of Interests:None declared

2019 ◽  
Vol 80 (8) ◽  
pp. 448-455
Author(s):  
Mehdi Raza ◽  
Yasser El Maideny ◽  
Nadia Bokhari

Giant cell arteritis has been widely studied throughout the world. Involvement of cranial vessels can lead to visual loss and strokes. This review primarily focusses on the presentation, diagnosis and treatment. The last 10 years have brought dramatic improvements in the imaging and medical therapies for this condition. After the American College of Rheumatology suggested criteria for the diagnosis of giant cell arteritis, many studies have been performed to find alternatives to a temporal artery biopsy. There is growing evidence that a biopsy may not be needed when one can make a convincing clinical and radiological diagnosis. Although glucocorticoids are the mainstay of treatment and their role has not changed, various biological and non-biological therapies are being used to reduce relapses and prolong remission of symptoms.


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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1549.2-1549
Author(s):  
D. Ludwig ◽  
M. Naja ◽  
S. Voo ◽  
V. Morris

Background:Giant cell arteritis (GCA) may affect both cranial and extra-cranial vessels; where the latter occurs, it can be termed large-vessel GCA (LV-GCA). Large vessel involvement is common: histological evidence has been seen in 80% of autopsies of patients with known GCA, and imaging studies suggest large vessel involvement in over 80%1. LV-GCA is important to diagnose due to the risks of vascular complications such as occlusion and ischaemic stroke. The clinical diagnosis can be challenging, and the American College of Rheumatology (ACR) GCA classification criteria often underperform in cases of LV-GCA1. F-fluorodeoxyglucose positron emission tomography (FDG-PET) has been found to be useful in the detection of extra-cranial involvement to support the diagnosis of LV-GCA.2Objectives:To appreciate the variability in presentation of cases of LV-GCA, and to further characterise a subgroup of patients with vertebral arteritis.To explore the use of FDG-PET imaging in GCA patients in addition to or in place of traditional diagnostic tools (temporal artery ultrasound / biopsy).Methods:Through evaluation of the new GCA fast-track pathway implemented at UCLH, a subgroup of patients diagnosed with vertebral arteritis was identified. The history and presentation of these patients were analysed.Results:Three patients were diagnosed with vertebral arteritis. All three were male, Caucasian and aged over 70. All were investigated for GCA due to a history of severe headache (frontal in one, occipital in one, bi-temporal in one) with associated red flag symptoms. Two had a history of jaw claudication and visual disturbances (unilateral visual loss in one, transient diplopia in the other). Both of these patients had positive temporal artery biopsies. The third patient had no ischaemic symptoms but a strong history of prominent polymyalgic features and a positive temporal artery ultrasound. Inflammatory markers were raised in two, and normal in one, of the patients. Only one had systemic symptoms (weight loss). All three proceeded to FDG-PET scans which showed vertebral arteritis and were commenced on immunosuppressive treatment.Conclusion:The cases discussed illustrate the heterogeneity of the presentation of LV-GCA, and the diagnostic challenge this poses. FDG-PET imaging is useful in confirming extra-cranial involvement and therefore guiding treatment.References:[1]Large-vessel giant cell arteritis: diagnosis, monitoring and management.Matthew J Koster, Eric L Matteson, Kenneth J Warrington.2018, Rheumatology, Vol. 57, pp. 32-42.[2]EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice.Dejaco C, Ramiro S, Duftner C, et al.2018, Annals of the Rheumatic Diseases, Vol. 77, pp. 636-643.Disclosure of Interests: :None declared


2016 ◽  
Vol 43 (7) ◽  
pp. 1393-1399 ◽  
Author(s):  
Eric Liozon ◽  
François Dalmay ◽  
Fabrice Lalloue ◽  
Guillaume Gondran ◽  
Holy Bezanahary ◽  
...  

Objective.To determine the risk factors for permanent visual loss (PVL) in patients with biopsy-proven giant cell arteritis (GCA) and the usefulness of the factors in clinical practice.Methods.From 1976 through 2015, the clinical charts and laboratory results of 339 patients with biopsy-proven GCA were recorded prospectively at the time of diagnosis. We used multivariable logistic regression analysis to determine which of 24 pretreatment characteristics were associated with PVL.Results.Visual ischemic manifestations occurred in 108 patients, including PVL in 53 (16%), bilaterally in 15 patients (28%). The independent predictors associated with an increased risk of PVL were age (OR 1.06, 95% CI 1.01–1.12, p = 0.01), a history of transient visual ischemic symptoms (OR 2.62, 95% CI 1.29–5.29, p < 0.01), and jaw claudication (OR 2.11, 95% CI 1.09–4.10, p = 0.03). The presence of fever (OR 0.30, 95% CI 0.14–0.64, p < 0.01) and rheumatic symptoms (OR 0.23, 95% CI 0.10–0.57, p = 0.001) were associated with a markedly reduced risk of developing visual loss (3.7% if features were both present). No laboratory variables were independently associated with PVL.Conclusion.The visual ischemic risk of untreated GCA can be readily estimated upon simple clinical findings, but not laboratory variables. However, we did not identify a subgroup of patients carrying no risk of developing visual loss. Glucocorticoid treatment remains, therefore, urgent for any patient with a high clinical suspicion index.


2008 ◽  
Vol 18 (2) ◽  
pp. 91-101 ◽  
Author(s):  
H A Bird ◽  
Helen Mac Iver

Polymyalgia rheumatica and giant cell arteritis are closely related conditions, considered by many to represent opposite poles of a single disease spectrum. They can occur together or separately.Polymyalgia rheumatica is characterized by pain and morning stiffness in the shoulder girdle and sometimes the pelvic girdle. The symptoms are felt to be related to synovitis of proximal joints and extra-articular synovial structures. Giant cell arteritis displays a frank vasculitis affecting the regions supplied by the temporal artery to give visual loss and scalp tenderness but is increasingly recognized to also affect the aorta and its extra-cranial branches. For this reason the term ‘giant cell arteritis’, which is descriptive of the pathology, is used instead of the alternative term ‘temporal arteritis’, which gives a misleading impression of localization but which was the term used in previous reviews for this journal, the most recent in 2003.


Author(s):  
Marília A. Dagostin ◽  
Rosa M.R. Pereira

Giant cell arteritis (GCA) is the most common vasculitis in adults, with the incidence increasing with the advancing age. The aorta and its branches, especially the carotid extracranial branches, are the classic targets of inflammation in GCA. Visual loss, upper limb ischemia, and stroke are complications described. Suspicion of GCA is a medical emergency, and patients need to be quickly diagnosed/treated to prevent irreversible damage. Headache is the most common symptom, and a new-onset headache in older adults should always raise the suspicion of GCA. Patients may also present with scalp tenderness or tongue/jaw pain. GCA is often found to be the cause of an obscure-origin fever in older patients. A positive temporal artery biopsy is considered the gold standard for the diagnosis, but imaging techniques enable the assessment of cranial and extracranial arteries and the aorta. Ultrasound of temporal arteries is recommended and noncompressible “halo” sign is the typical finding. PET, MRI, or CT may be useful for the detection of the disease in the aorta and other vessels. The treatment must be started promptly with prednisone 1 mg/kg/day. When visual symptoms/unilateral visual loss is present, methylprednisolone pulse is recommended. Methotrexate, leflunomide and tocilizumab may be effective and well-tolerated glucocorticoid-sparing agents in GCA. Cardiovascular diseases are the leading causes of death in patients.


2019 ◽  
Vol 46 (5) ◽  
pp. 501-508 ◽  
Author(s):  
Matthew J. Koster ◽  
Karthik Yeruva ◽  
Cynthia S. Crowson ◽  
Francesco Muratore ◽  
Cristian Labarca ◽  
...  

Objective.To determine the effect of methotrexate (MTX) on relapse risk and glucocorticoid (GC) use in a large single-institution cohort of patients with giant cell arteritis (GCA).Methods.Patients diagnosed with GCA from 1998 to 2013 with confirmed evidence of temporal artery biopsy and/or radiographic evidence of large vessel vasculitis were identified. Each patient with GCA treated with adjunct MTX (case) was matched to a similar patient with GCA treated only with GC (control). GC requirements and relapse events before and after MTX initiation (or corresponding index date) were compared using rate ratios (RR).Results.Eighty-three cases and 83 controls were identified and compared. No significant differences in age, demographics, laboratory variables, baseline disease characteristics, or mean initial prednisone doses were observed. Median [interquartile range (IQR)] time from GCA diagnosis to MTX initiation in cases was 39 (13–80) weeks and the median (IQR) starting dose was 13.5 (10–15) mg/week. RR comparing relapse rates before and after MTX initiation/index date were significantly reduced in both cases (RR 0.32, 95% CI 0.24–0.41) and controls (RR 0.60, 95% CI 0.43–0.86). The decrease in relapse rate was significantly greater in patients taking MTX than in those taking GC alone (p = 0.004). Rates of GC discontinuation did not differ between groups.Conclusion.In this large single-institution cohort, the addition of MTX to GC decreased the rate of subsequent relapse by nearly 2-fold compared to patients taking GC alone. MTX may be considered as adjunct therapy in patients with GCA to decrease the risk of further relapse events.


2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Nadia Ahmad ◽  
Elizabeth Price ◽  
Areli Cuevas-Ocampo ◽  
Khin Yein ◽  
Azeem Ahmed

Abstract Introduction This intriguing case describes a patient in who initial giant cell arteritis (GCA)/temporal arteritis (TA) presentation was preceded by bilateral acute anterior uveitis. He presented several months later after being treated for GCA with new neurological symptoms not typical of ischaemic cerebrovascular accident (CVA) on brain imaging. After ruling out a variety of differentials including an infection, he was treated for cerebral vasculitis secondary to temporal arteritis confirmed on brain biopsy which remains gold standard for diagnosis. Case description A 73-year-old patient with a background history of hypertension and mild asthma presented with three week history of ocular pain, headache and photosensitivity after a fall. CT head and lumbar puncture (LP) were unremarkable. He was diagnosed with bilateral acute anterior uveitis by ophthalmologists and treated with topical cyclopentolate and dexamethasone . In view of headaches, scalp tenderness, jaw claudication and raised inflammatory markers he was treated with 60mg of prednisolone for presumed giant cell arteritis (GCA) and temporal artery biopsy (TAB) was organised. He showed marked symptomatic improvement on steroids. Inflammatory markers normalised (erythrocyte sedimentation rate (ESR) 77 → 5 and C-reactive protein (CRP) 130 → <1). Temporal artery biopsy was negative, but took more than four weeks after starting steroids and was only 9mm in length. Serum screening was unremarkable for complements C3,4, antinuclear antibodies (ANA), anti neutrophil cytoplasmic antibodies (ANCA), bacterial or viral antibodies. Ten months later he was admitted with a two-week history of gradually worsening bilateral lower limb weakness on the background of chronic lower back pain. Magnetic resonance imaging (MRI) head showed parasagittal abnormalities which were thought to be atypical for ischemic infarction. Intracranial angiogram did not reveal any pathology. LP demonstrated elevated white cells (18 × 106/L – normal <5 × 106/L) and protein 0.61g/L (normal < 0.15-0.45g/L) with negative oligoclonal bands. The serology for neuronal autoantibodies and quantiferon was negative. ESR was elevated (50). Echocardiogram showed no vegetations. He was managed for acute cerebral vasculitis with methylprednisolone and pulsed cyclophosphamide (CYC). He also underwent a repeat TAB which was normal. In view of clinical deterioration he underwent repeat MRI head and spine which showed persistent active inflammation. Brain biopsy was organised which confirmed granulomatous inflammation with multinucleated giant cells. Unfortunately he continued to deteriorate, suffered from multiple infections and sadly passed away at his home with his family. Discussion Giant cell arteritis is a systemic vasculitis characterized by granulomatous inflammation of aorta and its main vessels. Visual complications are mostly due to vasculitis of posterior ciliary arteries. Uveitis as a presenting feature of GCA is uncommon. We should be aware that, although unusual, uveitis in elderly patients can be a presenting feature of GCA. Cardiovascular risk is increased in these patients. Several case series of myocardial infarction and stroke have been reported. About 30% of patients present with neurological manifestations, the most common are neuropathies (14%), including mono- and polyneuropathies of the limbs; stroke has been extensively described (5-20%), particularly vertebrobasilar ischemia. Cerebral vasculitis may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation of systemic vasculitis. In GCA, the involvement of CNS arteries is very rare (<2%). Our patient’s imaging revealed bilateral parafalcine frontal lobe changes in anterior cerebral artery territory. However, infarction in this territorial area is quite rare unless there is space occupying lesion or anatomical anomalies of vasculature. In our patient the MRI appearances were not convincing for ischaemic infarction. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on a combination of clinical, laboratory and imaging findings. In systemic vasculitis an acute inflammatory response with raised ESR and CRP may be present. CSF studies reveal mild lymphomonocytic pleocytosis or protein elevation in more than 90%. Magnetic resonance imaging, with or without contrast, is the investigation of choice to detect and monitor cerebral involvement. The treatment recommendations are derived from protocols for systemic vasculitides. A combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. Methotrexate, azathioprine and mycophenolate mofetil can be used for maintenance therapy similar to ANCA associated vasculitis. Key learning points Our case highlighted the rare presenting feature of GCA in the form of bilateral uveitis. Our patient was at high risk for developing ischaemic cerebral vascular event in view of large vessel vasculitis, his age and co-morbid hypertension but radiological imaging wasn’t typical for this and raised the suspicion of active cerebral vasculitis.  One should suspect multifocal brain disease like vasculitis when neurological deficit can’t be explained easily by territorial distribution of cerebral circulation. Cerebral vasculitis can be suspected on brain imaging and confirmed with biopsy. It is important to make this diagnosis as the treatment is immunosuppression different from that of a typical stroke and can be rewarding. Our patient was managed with immunosuppressive therapy but continued to deteriorate that prompted the need for brain biopsy which remains the gold standard for diagnosing cerebral vasculitis. Conflicts of interest The authors have declared no conflicts of interest.


Author(s):  
Kaveh Abri Aghdam ◽  
Mostafa Soltan Sanjari ◽  
Navid Manafi ◽  
Shabnam Khorramdel ◽  
Sayyed Amirpooya Alemzadeh ◽  
...  

Purpose: To assess the use of temporal artery biopsy (TAB) in diagnosing giant cell arteritis (GCA) and to evaluate patients’ clinical and laboratory characteristics. Methods: We conducted a retrospective chart review of patients with suspected GCA who underwent TAB and had complete workup in a tertiary center in Iran between 2008 and 2017. The 2016 American College of Rheumatology (ACR) revised criteria for early diagnosis of GCA were used for each patient for inclusion in this study. Results: The mean age of the 114 patients in this study was 65.54 ± 10.17 years. The mean overall score according to the 2016 ACR revised criteria was 4.17 ± 1.39, with 5.82 ± 1.28 for positive biopsies and 3.88 ± 1.19 for negative biopsies (p <0.001). Seventeen patients (14.9%) had a positive biopsy. Although the mean post-fixation specimen length in the biopsy-positive group (18.35 ± 6.9 mm) was longer than that in the biopsy-negative group (15.62 ± 8.4 mm), the difference was not statistically significant (P = 0.21). There was no statistically significant difference between the groups in terms of sex, serum hemoglobin, platelet count, and erythrocyte sedimentation rate. There were statistically significant differences between the biopsy-negative and biopsy-positive groups with respect to patients’ age and C-reactive protein level (P < 001 and P = 0.012, respectively). Conclusion: The majority of TABs were negative. Reducing the number of redundant biopsies is necessary to decrease workload and use of medical services. We suggest that the diagnosis of GCA should be dependent on clinical suspicion.


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