scholarly journals Tongue Necrosis as an Initial Manifestation of Giant Cell Arteritis: Case Report and Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Jose R. Zaragoza ◽  
Natalia Vernon ◽  
Gisoo Ghaffari
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
The Elderly ◽  
External Carotid Artery ◽  
External Carotid ◽  
Low Grade ◽  
Initial Manifestation ◽  
Atypical Manifestations

Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries that mainly affects the external carotid artery. It is a diagnosis of the elderly that typically presents as low-grade fever, temporal tenderness, claudication of the jaw, and in some patients vision loss. In cases where GCA presents with atypical manifestations, the diagnosis may be more difficult, causing a delay in both diagnosis and treatment and ultimately leading to irreversible complications. In this paper, we present an atypical presentation of GCA with symptoms of neck swelling and lingual pain in an elderly female. These symptoms progressed to bilateral necrosis and eventual dislodgement of the tongue. Lingual necrosis is a severe potential complication in GCA. In patients presenting with lingual swelling, pain, and discoloration, GCA should be suspected and prompt therapy should be initiated to avoid irreversible complications.

scholarly journals Neck swelling and airway narrowing as an initial manifestation of giant cell arteritis

2021 ◽  
Vol 14 (3) ◽  
pp. e237743
Author(s):  
Zhen Sheng Lim ◽  
Colin Sharp
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
External Carotid Artery ◽  
External Carotid ◽  
Initial Symptom ◽  
Neck Swelling ◽  
Initial Manifestation ◽  
Airway Narrowing ◽  
Wide Range

Giant cell arteritis can result in a wide range of symptoms due to the extensive distribution of the external carotid artery. Face and neck swelling and trismus are under-recognised features of giant cell arteritis and can present as the initial symptom prior to the development of classical temporal tenderness and jaw claudication. The lack of awareness of the less common symptoms may result in a late diagnosis of giant cell arteritis, leading to irreversible vision loss. In this paper, we present a case of neck swelling and airway narrowing as the initial manifestation of giant cell arteritis.

scholarly journals Challenges in the Diagnosis of Giant Cell Arteritis Before Visual Loss

2020 ◽  
pp. 1-5
Author(s):  
Purnima Mehta ◽  
Faaiq Hassan ◽  
Muhammed Omar Qadir ◽  
Shirish Dubey ◽  
Sergio Pagliarini ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Systemic Vasculitis ◽  
The Elderly ◽  
Common Type ◽  
Atypical Presentation ◽  
Delay In Diagnosis ◽  
Atypical Presentations ◽  
High Index Of Suspicion

Background: Giant cell arteritis (GCA) is the most common type of systemic vasculitis affecting the elderly. Ophthalmic presentations of GCA in particular can be difficult to identify prior to permanent visual loss occurring. Methods: Here, we present 3 challenging cases as a retrospective series to highlight the variable presentations of GCA with ophthalmic involvement, but GCA was not suspected due to atypical presentation. Results: Unfortunately, all 3 cases went on to develop visual loss in the affected eye due to a delay in diagnosis or treatment. The authors wish to highlight the challenges posed to the referring clinicians, when patients had systemic/ocular co-morbidities, which delayed the suspicion of GCA Conclusion with a Practical Point: Our cases highlight the variable presentations of this condition as well as the devastating ophthalmic implications that GCA can have. A high index of suspicion must be maintained; particularly in elderly patients with atypical presentations.

scholarly journals Giant Cell Arteritis: From Neurologist’s Perspective

2021 ◽  
Author(s):  
Ravish Rajiv Keni ◽  
M. Sowmya ◽  
Sreekanta Swamy
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Multidisciplinary Care ◽  
The Elderly ◽  
External Carotid Artery ◽  
Laboratory Evaluation ◽  
High Dose ◽  
Granulomatous Vasculitis ◽  
Dose Corticosteroid

Giant cell arteritis (GCA) is a granulomatous vasculitis affecting large- and medium-sized arteries in the elderly and potentially causes visual loss. In an elderly patient presenting with acute pain in the distribution of the external carotid artery (e.g., headache, scalp tenderness); polymyalgia rhematica; or acute/transient visual loss or diplopia; a possibility of GCA should be considered in one of the differential diagnosis. Urgent laboratory evaluation (e.g., ESR, CRP, platelet count), followed immediately by empiric high-dose corticosteroid therapy is warranted in patients suspected of having GCA. Although ultrasound techniques are sensitive for the diagnosis of GCA, TAB remains the best confirmatory test. Patients with GCA often require long durations of steroid therapy and steroid-related complications are common. Multidisciplinary care and the use of steroid-sparing regimens are warranted in case of relapse.

scholarly journals The Immunopathology of Giant Cell Arteritis Across Disease Spectra

2021 ◽  
Vol 12 ◽  
Author(s):  
Michelle L. Robinette ◽  
Deepak A. Rao ◽  
Paul A. Monach
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Arthritis ◽  
Clinical Presentation ◽  
Systemic Vasculitis ◽  
The Elderly ◽  
Large Vessel ◽  
Circulating Cells ◽  
Conventional Histology ◽  
Active Disease

Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic imaging have revealed a greater degree of large vessel involvement than previously recognized, distinguishing classical cranial- from large vessel (LV)- GCA. GCA often co-occurs with the poorly understood inflammatory arthritis/bursitis condition polymyalgia rheumatica (PMR) and has overlapping features with other non-infectious granulomatous vasculitides that affect the aorta, namely Takayasu Arteritis (TAK) and the more recently described clinically isolated aortitis (CIA). Here, we review the literature focused on the immunopathology of GCA on the background of the three settings in which comparisons are informative: LV and cranial variants of GCA; PMR and GCA; the three granulomatous vasculitides (GCA, TAK, and CIA). We discuss overlapping and unique features between these conditions across clinical presentation, epidemiology, imaging, and conventional histology. We propose a model of GCA where abnormally activated circulating cells, especially monocytes and CD4+ T cells, enter arteries after an unknown stimulus and cooperate to destroy it and review the evidence for how this mechanistically occurs in active disease and improves with treatment.

scholarly journals Current advances in the treatment of giant cell arteritis: the role of biologics

2019 ◽  
Vol 11 ◽  
pp. 1759720X1982722 ◽  
Author(s):  
Candice Low ◽  
Richard Conway
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Severe Disease ◽  
Treatment Options ◽  
Biologic Agents ◽  
Necrosis Factor Alpha ◽  
Treatment Paradigm ◽  
High Doses

Giant cell arteritis (GCA) is the most common form of systemic vasculitis. It is a potentially severe disease with 25% of patients suffering vision loss or stroke. Our treatment paradigm is based on glucocorticoids. Glucocorticoids are required in high doses for prolonged periods and subsequently are associated with a significant amount of treatment-related morbidity. Alternative treatment options are urgently needed to minimize these glucocorticoid adverse events. Many other agents, such as methotrexate and tumour necrosis factor alpha inhibitors have been used in GCA, with limited or no evidence of benefit. Our emerging understanding of the pathogenic processes involved in GCA has led to an increased interest in the use of biologic agents to treat the disease. Two randomized controlled trials have recently reported dramatic effects of the use of the interleukin-6 targeted biologic tocilizumab in GCA, with significant increases in remission rates and decreases in glucocorticoid burden. While encouraging, longer-term and additional outcomes are awaited to clarify the exact positioning of tocilizumab in the treatment approach. Emerging data for other biologic agents, particularly abatacept and ustekinumab, are also encouraging but less well advanced. We are at the dawn of a new era in GCA treatment, but uncertainties and opportunities abound.

scholarly journals Hepatic Giant Cell Arteritis and Polymyalgia Rheumatica

1994 ◽  
Vol 8 (1) ◽  
pp. 36-38 ◽  
Author(s):  
Donald R Duerksen ◽  
Laurence D Jewell ◽  
Vincent G Bain
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Polymyalgia Rheumatica ◽  
The Elderly ◽  
Good Prognosis ◽  
Clinical Syndrome ◽  
Low Grade ◽  
Erythrocyte Sedimentation ◽  
Systemic Corticosteroids ◽  
Wedge Biopsy

Polymyalgia rheumatica (PMR) is a clinical syndrome of the elderly characterized by malaise, proximal muscle aching and stiffness, low grade fever, elevated erythrocyte sedimentation rare and the frequent association with temporal giant cell arteritis. The authors describe a case of PMR associated with hepatic giant cell arteritis. This lesion has been described in two other clinical reports. The distribution of the arteritis may be patchy; in this report, diagnosis was made with a wedge biopsy performed after an initial nonspecific percutaneous liver biopsy. The authors review the spectrum of liver involvement in PMR and giant cell arteritis. Hepatic abnormalities respond to systemic corticosteroids, and patients with hepatic arteritis have a good prognosis.

scholarly journals AB0485 INVESTIGATION OF PERMANENT ORGAN DAMAGE IN GIANT CELL ARTERITIS: DISEASE FLARES ARE ASSOCIATED WITH INCREASED DAMAGE SCORES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1540.2-1540
Author(s):  
B. Ince ◽  
S. Artan ◽  
Y. Yalçinkaya ◽  
B. Artim-Esen ◽  
A. Gül ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Damage Index ◽  
Organ Damage ◽  
Cardiovascular Damage ◽  
Vasculitis Damage Index

Background:Development of organ damage is a major concern in patients with systemic vasculitis. Treatment may also contribute to this important outcome. Scoring systems has been developed to evaluate organ damage in systemic vasculitis and specifically for large vessel vasculitis (1).Objectives:We aimed to investigate permanent organ damage and determining factors in our giant cell arteritis GCA cohort.Methods:Organ damage detected at the time of diagnosis and / or follow-up and irreversible for at least 3 months in GCA patients followed up between 1998-2018 were recorded by using Vasculitis Damage Index (VDI) and Vascular Vasculitis Damage Index (LVVID) fom patient records of our vasculitis clinic. In the statistical evaluation, chi-square, students t-test and logistic regression analysis were used.Results:Eighty-nine patients (64% women, mean age 67.9 ± 9.1) included in the study, the mean follow-up duration was 61.6 ± 58.6 months. All organ damage findings according to both VDI and LVVID are shown in table-1. In this cohort, cardiovascular damage items and diabetes mellitus were prevalent at baseline. At least one damage item was present in 53 (59,5%) according to VDI; 54 (%60,7) according to LVVID and agreement was high between two damage indices (kappa=0.97). Forty-seven of patients (52%) had a damage item presumably with contribution of GC treatment e.g. locomotor system findings, hypertension, diabetes and cataract; 12 (13,5%) had damage items related to disease (total or partial vision loss, ischemic optical neuropathy). Mean time to diagnosis after initial symptoms was longer in patients with permanent vision loss (10,2±4,3 vs. 5,2±1,2 months p=0.006). The presence of damage was associated with flares in univariate and multivariate analysis (29/54 vs. 2/35 p<0,001 OR=19 %95 GA 4,2– 87,9). All patients who had a flare during the first year (n = 15) developed signs of damage at follow-up. No association was found between the development of organ damage and the age of diagnosis, the time between first complaint and diagnosis, presence of cranial, ophthalmologic findings, PET-CT positivity, cumulative steroid dose, and DMARD use.Conclusion:In our study, permanent organ damage was analysed by using diffrerent indices. In this patient population baseline cardiovascular damage and diabetes mellitus were frequent as expected but information for osteoporosis was lacking. More than half of the patients had damage and significant part of the present items was considered due to corticosteroid treatment. The most common damage item developed was osteoporosis. There was a very good agreement between the two indices, despite few specific items in LVVID. The striking relationship of disease flare with damage and frequency of visual problems despite treatment indicate the necessity of new treatment strategies.References:[1]Kermani, T.A., et al.,Evaluation of damage in giant cell arteritis.Rheumatology (Oxford), 2018.57(2): p. 322-328.Disclosure of Interests: :None declared

scholarly journals Giant Cell Arteritis: Real-life Experience

2021 ◽  
pp. jrheum.210334
Author(s):  
Peter M. Villiger
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Life Experience ◽  
Real Life ◽  
The Elderly ◽  
Optimal Treatment ◽  
Frequent Form ◽  
European League Against Rheumatism ◽  
European League

Giant cell arteritis (GCA), the most frequent form of vasculitis in the elderly, still poses questions regarding optimal treatment. Because of the risk of permanent vision loss, the revised European League Against Rheumatism recommendation of 2018 stresses that glucocorticoid (GC) therapy should start as soon as a diagnosis of GCA is strongly suspected.1

scholarly journals Giant cell arteritis or tension-type headache?: A differential diagnostic dilemma

2014 ◽  
Vol 05 (04) ◽  
pp. 409-411 ◽  
Author(s):  
Abdul Qayyum Rana ◽  
Usman Saeed ◽  
Osama A. Khan ◽  
Abdul Rehman M. Qureshi ◽  
Dion Paul
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
The Elderly ◽  
Tension Type Headache ◽  
The Body ◽  
Diagnostic Dilemma ◽  
Disease States ◽  
Incorrect Diagnosis ◽  
Type Headache

ABSTRACTGiant cell arteritis (GCA) or Temporal arteritis (TA) is an autoimmune disease and the most common type of vasculitis in the elderly. It causes inflammation of the medium and large arteries in the upper part of the body. GCA is an under-recognized cause of head aches in the elderly, especially when it presents itself with atypical features, resulting in delayed or incorrect diagnosis. Since GCA is a treatable condition, an accurate diagnosis is crucial to prevent the most serious complication of CGA, permanent vision loss. The diagnosis can be further complicated as GCA may present with features of other painful neurological conditions. The present case is an 81-year-old woman diagnosed with GCA, who initially presented with features similar to tension-type headache. Due to overlapping features of these conditions, the diagnosis of GCA was delayed, resulting in irreversible vision loss. Although previous research highlights diagnostic dilemmas featuring GCA and other disease states, this case is exclusive in describing a unique dilemma where tension-type headache mimics GCA.

scholarly journals The Thromboembolic Risk in Giant Cell Arteritis: A Critical Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
A. Guida ◽  
A. Tufano ◽  
P. Perna ◽  
P. Moscato ◽  
M. T. De Donato ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Granulomatous Inflammation ◽  
Cardiovascular Prevention ◽  
Sudden Onset ◽  
New Drugs ◽  
Rheumatological Disease

Giant cell arteritis is a systemic vasculitis characterized by granulomatous inflammation of the aorta and its main vessels. Cardiovascular risk, both for arterial and venous thromboembolism, is increased in these patients, but the role of thromboprophylaxis is still debated. It should be suspected in elderly patients suffering from sudden onset severe headaches, jaw claudication, and visual disease. Early diagnosis is necessary because prognosis depends on the timeliness of treatment: this kind of arteritis can be complicated by vision loss and cerebrovascular strokes. Corticosteroids remain the cornerstone of the pharmacological treatment of GCA. Aspirin seems to be effective in cardiovascular prevention, while the use of anticoagulant therapy is controversial. Association with other rheumatological disease, particularly with polymyalgia rheumatica is well known, while possible association with antiphospholipid syndrome is not established. Large future trials may provide information about the optimal therapy. Other approaches with new drugs, such as TNF-alpha blockades, Il-6 and IL-1 blockade agents, need to be tested in larger trials.

Sign in / Sign up

Export Citation Format

Share Document