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 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 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 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.

scholarly journals Giant Cell Arteritis in Ophthalmology Practice: a Case Report

2019 ◽  
Vol 16 (1) ◽  
pp. 109-114
Author(s):  
V. A. Chernukha ◽  
D. S. Atarschikov ◽  
N. V. Khamnagdaeva ◽  
I. V. Pozharov
Keyword(s):  
Case Report ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss ◽  
Systemic Vasculitis ◽  
Ischemic Optic Neuropathy ◽  
Reactive Protein ◽  
Retinal Artery ◽  
The Right

Introduction. Giant cell (temporal) arteritis refers to a group of chronic and acute systemic vasculitis mainly affecting the extracranial and intracranial arteries of large and medium caliber. Loss of vision due to anterior ischemic optic neuropathy (AION) or occlusion of the central retinal artery is one of the most severe and most common complications of giant cell arteritis. This case report describes a patient with giant cell arteritis, the outcome of it was a total vision loss in the right eye. The patient repeatedly visited the ophthalmologists in the outpatient clinics with complaints of intermittent episodes of vision loss.Purpose. To present methods of diagnosis and treatment, through which the doctor at the initial reception will be able to suspect the disease and start treatment timely.Conclusions. The anamnestic criteria for the GCA diagnosis in the practice of ophthalmologist are: female, age over 50 years, headaches with paresthesia, intermittent lameness of the mandible, short-term episodes of vision loss. The necessary laboratory methods of research include: clinical blood test with determination of erythrocyte sedimentation rate, determination of C-reactive protein level.

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 Vision Loss in Patients with Giant Cell Arteritis Treated with Tocilizumab

2020 ◽  
Author(s):  
Jennifer Amsler ◽  
Iveta Kysela ◽  
Christoph Tappeiner ◽  
Luca Seitz ◽  
Lisa Christ ◽  
...  
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Structural Changes ◽  
Vision Loss ◽  
Treated Patient ◽  
Laboratory Data ◽  
Reactive Protein ◽  
American College Of Rheumatology ◽  
Low Incidence ◽  
Initiation Of Therapy

Abstract Objectives: Giant cell arteritis (GCA) may lead to vision loss. To what extent tocilizumab (TCZ) is able to prevent vision loss is unknown. The aim was to analyze the occurrence of vision loss in a large GCA cohort treated with TCZ.Methods: In this observational monocentric study, GCA patients treated with TCZ between the years 2010 and 2018 were studied. Demographic, clinical and laboratory data were analyzed. Results: A total of 186 patients were included (62% female); 109 (59%) fulfilled the American College of Rheumatology (ACR) criteria, in 123 (66%) patients, large vessel vasculitis was diagnosed in magnetic resonance-angiography (MRA). Cumulative duration of TCZ treatment was 224 years, median treatment duration was 11.1 (IQR 5.6-17.9) months. Glucocorticoids (GC) were tapered over a median of 5.8 (IQR 3.0-8.5) months. At baseline, visual symptoms were present in 70 (38%) and vision loss in 21 (11%) patients. Patients with vision loss at baseline were older (p=0.032), had a lower C-reactive protein (p=0.002), more often cranial symptoms (p<0.001) or jaw claudication (p=0.031) and showed a negative association with MRA of the aorta (p=0.006). Two patients (1.1%) developed vision loss, both at initiation of TCZ treatment.Conclusion: Our data show a very low incidence of vision loss in TCZ-treated patient. The two cases of AION occurred at initiation of therapy, they support the hypothesis that advanced, and established structural changes of arteries are key factors for this accident. Whether shorter duration of concomitant GC treatment is risky regarding vision loss needs to be studied.

Vision Loss From Giant Cell Arteritis in Patients With Other Ocular Diagnoses

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Prem Nichani ◽  
Valérie Biousse ◽  
Nancy J. Newman ◽  
Jonathan A. Micieli
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Vision Loss

Giant cell arteritis of the legs. Clinical isolation of severe disease with gangrene and amputations

1986 ◽  
Vol 81 (4) ◽  
pp. 727-733 ◽  
Author(s):  
George M. Greene ◽  
Douglas Lain ◽  
Richard M. Sherwin ◽  
Janet E. Wilson ◽  
Bruce M. McManus
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Severe Disease

Vertebral arteries: A target for FDGPET imaging in giant cell arteritis?

2011 ◽  
Vol 50 (01) ◽  
pp. 28-32 ◽  
Author(s):  
J. Weinerth ◽  
C. Hrdina ◽  
K. Pfadenhauer
Keyword(s):  
Ischemic Stroke ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Axillary Artery ◽  
Posterior Circulation ◽  
Large Arteries ◽  
Fdg Uptake ◽  
Vertebral Arteries ◽  
Good Target ◽  
High Doses

Summary Aim: To demonstrate the capacity of FDG-PET to show active giant cell arteritis (GCA) of the extracerebral vertebral artery (VA) and to compare it with clinical, ultrasonographic (US) and biopsy findings. Patients, methods: Observational study of 46 consecutive patients with the diagnosis of active GCA and abnormal high FDG uptake in the aorta and other large arteries suggestive for GCA. Results: 15 of the 46 GCA patients had abnormal high FDG uptake within the extending from the V0 to V3 segment in 13 and confined to single segments in 2 patients. In 2 patients high FDG uptake in one VA was the onIy PET abnormality. In 13 patients high FDG uptake was also found in other large arteries (carotid n = 10, subclavian/axillary artery n = 12, thoracic aorta n = 12). Abnormal PET was detectable in 5 patients despite glucocorticoid (GC) treatment. Nuchal and occipital pain and ischemic stroke or TIA in the posterior circulation (n = 3) were found in 10 patients with high VA FDG uptake. US detected halos of the V0–2 segments in 8/46 patients (5/15 VA PET positive and 3/31 VA PET negative patients). Biopsies were available in 10/15 VA PET positive patients with evidence of active GCA in 7 cases. Conclusion: In patients with severe GCA and a high TVS the extracranial VA are a good target for PET imaging in active GCA with abnormal findings in 33% of patients with a positive PET. VA abnormalities can be an early and isolated finding in active GCA. PET is superior to US for the detection of active VA-GCA. A strong correlation between VA abnormalities and associated clinical abnormalities existed in 2/3 of patients. PET abnormalities of the VA could be detected in some cases after GC treatment has been started even at high doses.

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