Manifestations Of Giant Cell Arteritis Research Articles

Oral and intestinal manifestations of giant cell arteritis

Background: Giant cell arteritis (GCA) is the most common primary vasculitis in individuals over 50 years of age. GCA typically affects large- and medium-sized arteries and is classically associated with cranial manifestations of ischaemia, such as headaches (notably in the temporal region), jaw claudication and visual symptoms that can lead to blindness. Extracranial symptoms are less frequently reported and are related to involvement of the thoracic and abdominal aorta and its main branches. Classic diagnostic tools (such as temporal artery ultrasonography and temporal artery biopsy) can be negative in extracranial GCA. Case description: We report a difficult diagnosis of GCA in a 75-year-old woman who developed painful tongue ulcers that responded to prednisone treatment. However, a comprehensive diagnostic work-up did not lead to a certain diagnosis of GCA and corticosteroids were stopped after one month. A few months later, the patient suffered from mechanical ileus secondary to ischaemic stenosis of the small bowel. Pathological examination of the small bowel resection, and a second FDG-PET/CT, led to the diagnosis of extracranial GCA. Conclusions: We present a case of CGA which sequentially affected cranial and extracranial arteries and illustrate pitfalls in the diagnosis of this polymorph condition.

Fourth Nerve Palsy as the Presenting Manifestation of Giant Cell Arteritis.

Fourth Nerve Palsy as the Presenting Manifestation of Giant Cell Arteritis.

Infective agents and polymyalgia rheumatica: key discussion points emerging from a narrative review of published literature.

The aetiology of polymyalgia rheumatica (PMR) is unknown. Recently, reports on cases of PMR following the coronavirus disease 2019 (COVID-19) have revived the role of infection as an aetiological or triggering factor. It is estimated that patients with PMR have manifestations of giant cell arteritis (GCA) in < 20% of cases. To date, little is known on the potential role of infectious agents in facilitating this association. Given this background, we performed a review of published literature. Our first aim was to review and discuss the relationship between PMR and infective agents. Secondly, we compared data of PMR-only patients with PMR and overlapping GCA to seek any commonalities or differences regarding the type of infectious agent in these two subgroups. We performed a non-systematic literature search on Embase and Medline (COVID interface) with the following search terms: "polymyalgia rheumatica" AND "infections" OR "infectious agents", both MESH headings and free-text (in each language they were written). Each paper's reference list was scanned for additional publications meeting this study's aim. When papers reported data partially presented in previous articles, we referred to the most recent published data. Abstracts submitted at conferences or from non-peer-reviewed sources were not included. Polymyalgia rheumatica following vaccinations was an additional exclusion criterion. Several infectious agents have been held responsible for PMR. However, no definite causal link has been identified so far. According to our review, the search for a specific infectious agent, however intriguing, appears to be stagnating. Genetic background and epigenetic regulation probably play a key role. However, topical studies are lacking. Polymyalgia rheumatica as an adverse event following immunization should be kept methodologically distinct from PMR following an acute infection, as the adjuvants in the vaccine can make a significant difference. Finally, some infectious agents are able to replicate in human arteries or have an endothelium tropism. Whilst these can theoretically trigger GCA, their role in isolated PMR seems minimal.

The Importance of Early Recognition of Rare Ischaemic Complications of GCA to Prevent Permanent Vision Loss.

Scalp and tongue necrosis are rare ischemic manifestations of giant cell arteritis (GCA). Early recognition of these conditions is crucial to preventing permanent visual loss (PVL). We present two cases of scalp and tongue necrosis, respectively, where a delay in diagnosis resulted in irreversible vision loss and severe complications. These cases highlight the importance of educating non-rheumatologists about these manifestations to ensure prompt steroid treatment, which can prevent vision loss and reduce morbidity in GCA patients.

Incidence and clinical manifestations of giant cell arteritis in Spain: results of the ARTESER register

ObjectiveThis study aimed to estimate the incidence of giant cell arteritis (GCA) in Spain and to analyse its clinical manifestations, and distribution by age group, sex, geographical area and season.MethodsWe...

Visual manifestations in giant cell arteritis: identification of risk factors from the ARTESER Registry.

To determine the prevalence and predictive factors of visual manifestations in a large registry of patients with GCA. ARTESER is a large Spanish multicentre registry supported by the Spanish Society of Rheumatology. It includes patients with GCA from across the entire country diagnosed between June 2013 and March 2019. The variables collected at diagnosis were demographics, clinical manifestations (including all visual manifestations), laboratory, temporal artery biopsy, and imaging findings (ultrasound, FDG-PET/CT, MRI angiography, CT angiography). Patients with and without visual involvement were compared in a bivariate analysis. Multivariate logistic regression was performed to determine potential predictive factors of visual manifestations. The study population comprised 1636 GCA patients, of whom 599 (36.6%) presented visual manifestations. Anterior ischemic optic neuropathy was the most frequent (n = 274 of 599; 45.7%) ocular complication. The independent predictors that increased the risk (OR; 95% confidence interval) of visual involvement were older age (1.027; 1.009-1.045) and jaw claudication (1.724; 1.325-2.243). The variables associated with a reduced risk were polymyalgia rheumatica (0.541; 0.414-0.708), fever (0.373; 0.264-0.527), longer symptom duration (0.946; 0.909-0.985), and higher erythrocyte sedimentation rate (ESR) (0.992; 0.988-0.997), common features of patients with large vessel-GCA. One-third of GCA patients present visual manifestations at diagnosis. Older age and jaw claudication are independent predictors of visual manifestations, whereas polymyalgia rheumatica, fever, longer symptom duration, and high ESR reduce the risk of visual involvement.

Bilateral optic perineuritis as an initial presentation of giant cell arteritis

Giant cell arteritis (GCA), also known as Horton’s disease, is a granulomatous vasculitis affecting large and medium-sized arteries, frequently leading to visual complications due to ophthalmic artery involvement. We present a case of a 72-year-old woman with bilateral optic perineuritis, marking the first manifestation of GCA. The patient experienced severe headaches and progressive vision loss, with brain MRI revealing thickening of the temporal artery walls and optic nerve sheath enhancement. Optic perineuritis, while rare, can be an initial sign of GCA, underscoring the importance of early recognition and treatment to prevent irreversible visual impairment.

Clinical manifestations and prognosis of giant cell arteritis: A retrospective cohort study

The aim of the study was to evaluate the clinical manifestations and survival of patients with giant cell arteritis (GCA).Methods. A retrospective study included 166 patients with newly diagnosed GCA. Clinical, laboratory and instrumental data, three sets of classification criteria were used to confirm the diagnosis: the American College of Rheumatology (ACR) 1990, the revised ACR criteria of 2016 and/or the new ACR and European Alliance of Associations for Rheumatology (EULAR) 2022 criteria. Some of the patients underwent instrumental investigations: temporal artery ultrasound Doppler (n=61), contrast-enhanced computed tomography (CT) (n=5), CT angiography (n=6), magnetic resonance imaging (n=4), magnetic resonance angiography (n=3) and 18F-FDG positron emission tomography/CT (n=47). Overall and recurrence-free survival were analyzed using survival tables, Kaplan – Meier method.Results. The most frequent first manifestations of GCA were headache (81.8%), weakness (64%), fever (63.8%) and symptoms of rheumatic polymyalgia (56.6%). Changes of temporal arteries in color duplex scanning were detected in 44 out of 61 patients. GCs therapy was performed in all patients who agreed to be treated (n=158), methotrexate was used in 49 out of 158 patients, leflunomide – in 9 patients. In 45 (28.5%) out of 158 patients a stable remission was achieved as a result of GCs monotherapy, in 120 (75.9%) patients long-term maintenance therapy with GCs was required to prevent exacerbations, including 71 (44.9%) patients – in combination with methotrexate or other immunosuppressive drugs. The follow-up period of patients with a history of relapses was 21.0 (8.0–54.0) months. Relapses developed in 73 (46.2%) patients. The overall one-year survival rate was 97.1% [95% confidence interval (CI): 94.3; 99.9], and the five-year survival rate of patients was 94.6% [95% CI: 90.2; 99.0]. The one-year relapse-free survival rate was 86.4% [95% CI: 80.5; 92.3], and the five-year relapse-free survival rate was 52.4% [95% CI: 42.0; 62.8]. 12 (7.2%) of 166 patients died. The cause of death was myocardial infarction in two patients, stroke in two patients, and breast cancer in one patient; in the remaining seven cases, the cause of death was not determined.Conclusion. Given the high frequency of disease exacerbation, patients with GCA require long-term follow-up, especially during the first year after diagnosis.

Intermittent Ophthalmoplegia as the Initial Manifestation of Giant Cell Arteritis

Giant cell arteritis (GCA) is the most common form of vasculitis in elderly with multiple cardinal clinical features, such as headache associated with acute visual loss, jaw claudication and high inflammatory markers. GCA is readily treatable with immunosuppressants. Yet, if treated late, GCA could result in significant morbidities, such as aortic aneurysm, dissection, rupture and permanent visual loss. Early initiation of diagnostic evaluations, such as ultrasound of the temporal and axillary arteries and temporal artery biopsy, is thus very important. We present a case of an 84-year-old man who was diagnosed with GCA who presented with solely painless-intermittent ophthalmoplegia. He was later confirmed to have GCA with temporal artery ultrasound and biopsy. This case serves to emphasise the importance of recognising uncommon presentations of GCA and early initiation of its diagnostic tests and treatment.

P111 Tongue necrosis as an unusual manifestation of Giant Cell Arteritis

P111 Tongue necrosis as an unusual manifestation of Giant Cell Arteritis

Clinical Images: Tongue ulceration in giant cell arteritis associated with asymptomatic retinal ischemia

The patient, a 69-year-old female, presented with new-onset headache and facial and sinus pain initially treated by primary care with azithromycin for presumed sinusitis. Her erythrocyte sedimentation rate (ESR) was 114 mm/hour, and her C-reactive protein (CRP) level was 173.5 mg/l. Following antibiotics, she developed “thickened” tongue with white plaque (A) and was treated with oral nystatin for thrush. However, she subsequently developed masticatory and lingual claudication, a distal left tongue ulcer (A and B), and left temporal discomfort. A left temporal artery biopsy (TAB) showed histopathological findings consistent with giant cell arteritis (GCA). A retinal examination and fluorescein angiogram showed evidence of left eye retinal ischemia with presence of cotton wool spots, though asymptomatic and without visual field deficits. High-dose oral prednisone (100 mg/day) was started on the day of TAB (B). Within 48 hours, the headache and jaw and tongue claudication resolved. Seven days later, marked improvement in the tongue ulcer (C) was noted, and inflammatory markers had improved (ESR: 40 mm/hour; CRP level: 14.2 mg/l). Given significant clinical response, a prednisone taper without adjunct tocilizumab was used. A follow-up evaluation 3 months later (prednisone dose 10 mg/day) showed full resolution of the tongue ulcer (D), and the patient remained asymptomatic with a normal ESR (5 mm/hour) and CRP level (5.4 mg/l). Lingual manifestations in GCA include claudication, pain, edema, pallor, ulceration, and necrosis. Tongue claudication has been reported in approximately 3% of patients with GCA (1); however, tongue ulceration and frank necrosis are rare. GCA is considered the most common noninfectious vascular etiology of tongue necrosis, and its presence should prompt consideration and investigation for GCA. Visual ischemic features have been reported in up to 38% of patients with GCA with tongue ulceration or necrosis (2); therefore, all patients with GCA with tongue claudication or ulceration/necrosis should undergo detailed ophthalmologic examination. Prompt recognition and treatment initiation is paramount, and in this case, glucocorticoids preserved vision and resolved cranial and lingual symptoms. Disclosure Form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Systemic Manifestations of Giant Cell Arteritis.

Systemic Manifestations of Giant Cell Arteritis.

Tongue necrosis and paracentral acute middle maculopathy as manifestations of giant cell arteritis

Giant cell arteritis (GCA) is the most common primary vasculitis in adults. In general, the diagnosis is straightforward when characteristic symptoms such as headaches, scalp tenderness, jaw claudication, vision problems, or other ischemic complications are present. Atypical presentations of GCA have become increasingly recognized but we report for the first time a case of GCA presenting as partial tongue necrosis and paracentral acute middle maculopathy. This case adds to the literature and emphasizes the importance of rapid recognition of these atypical changes and optical coherence tomography in the evaluation of GCA patients.

Ophthalmic Manifestations of Giant Cell Arteritis.

Ophthalmic Manifestations of Giant Cell Arteritis.

Ocular hypotonia as first manifestation of giant cell arteritis

Ocular hypotonia as first manifestation of giant cell arteritis

Surdité dans l’artérite à cellules géantes : à propos d’une observation

IntroductionHearing loss is a rare manifestation in giant cell arteritis. The different types of deafness are possible with a predominance of sensorineural deafness. Case reportWe report a 75-year-old woman who presented with typical manifestations of giant cell arteritis associated concomitantly with the occurrence of bilateral mixed hearing loss confirmed on the audiogram. Corticosteroids allowed a rapidly favorable clinical and biological outcome. The follow-up audiogram at 3 months was markedly improved and showed a decrease in sensorineural hearing loss and disappearance of conductive hearing loss. ConclusionAny rapid onset deafness in an inflammatory context in the elderly should lead to a search for giant cell arteritis. The diagnosis can be difficult in the absence of other typical manifestations, especially since the biopsy of the temporal artery most often comes back negative. Corticosteroids are usually effective.

Ischemic ocular manifestations of giant cell arteritis: A Canadian case series

BackgroundDiagnosing giant cell arteritis (GCA) can be challenging due to the variable clinical presentation and lack of systemic symptoms in approximately 20% of cases, which are termed occult GCA. GCA affects the ocular circulation in more than half of cases and can cause irreversible bilateral blindness if not rapidly recognized and treated. We compared clinical and laboratory findings of patients with occult and systemic temporal artery biopsy (TAB)-positive GCA with ocular manifestations. MethodsRetrospective chart review of patients with TAB-positive GCA with ocular manifestations seen at a tertiary neuro-ophthalmology practice between 2015 and 2020. Results:GCA was occult in 14.3% (6/42) and systemic in 85.7% (36/42) of patients. Occult GCA affected older patients (average age 84.6 ± 5.3 vs 75.9 ± 8.2). There was no significant difference in ESR or CRP levels between two cohorts. In patients with occult GCA, 83.3% (5/6) presented with AION, and 16.7% (1/6) with isolated cranial nerve six palsy (CN6P). In the systemic GCA cohort, 47.2% (17) presented with AION, 5.6% (2) with CRAO, 2.8% (1) with PION, 2.8% (1) with isolated CWS, 2.8% (1) had both CRAO and AION, 2.8% (1) isolated cranial nerve 4 palsy (CN4P), 2.8% (1) isolated CN6P, 22.2% (8) had history of amaurosis fugax but normal examination, and 11.1% (4) had visual symptoms but a normal exam. Three of the above patients with AION also had CN4P. Of patients diagnosed with AION, presenting visual acuity in the affected eye(s) was significantly worse in patients with occult GCA. Involvement of multiple ocular circulations was seen in six patients in the systemic group but none in the occult group. ConclusionOccult GCA occurs more commonly in older patients who most of the time present with AION. Levels of inflammatory markers were similar in the occult and systemic groups. Though ocular ischemic manifestations were similar in both cohorts, in systemic GCA involvement of multiple ocular circulations is likely more common GCA should always be considered in older patients who present with signs of retinal and/or optic nerve head ischemia (AION and/or CWS) and new onset of CNPs, especially in patients where more than one ocular arterial circulation is involved.

Paracentral Acute Middle Maculopathy as the Presenting Manifestation of Giant Cell Arteritis.

Paracentral Acute Middle Maculopathy as the Presenting Manifestation of Giant Cell Arteritis.

Neurologic manifestations of giant cell arteritis.

Giant cell arteritis, the most frequent form of vasculitis in persons over 50years of age, is a granulomatous chronic vasculitis involving large and medium-sized vessels, most commonly the temporal and other cranial arteries. This common, treatable condition is associated with various clinical symptoms, including neurological ones, affecting both the central and peripheral nervous systems. In this review, we discuss the cranial and extra cranial neurological complications of giant cell arteritis, to help avoid the many pitfalls in the diagnosis of giant cell arteritis.

Updates in cutaneous manifestations of systemic vasculitis.

The main purpose of this review is to present newly reported cutaneous manifestations of systemic vasculitis, updates in investigations to verify systemic involvement in cases with cutaneous vasculitis and new therapeutic guidelines. The spectrum of COVID-19-related vasculitis is also covered. Only a few reports highlighted new cutaneous presentations or associations with some systemic vasculitic entities. For example, the association of inflammatory disorders with Takayasu arteritis, the importance of considering Kawasaki disease in febrile children with erythema nodosum, the development of necrotic ulcers on fingers and toes in Behçet's disease and the possible presence of polyarteritis nodosa-like pathological features in vulvar ulcers of Behçet's disease. New attempts to classify cutaneous manifestations of giant cell arteritis (GCA) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the diagnostic investigations for cutaneous vasculitis cases to verify systemic involvement are discussed. Treatment of systemic vasculitis with cutaneous vasculitis should be tailored according to disease status. A plethora of reports in the past 2 years focused on the broad spectrum of COVID-19 vasculitic manifestations. Although newly reported cutaneous manifestations of systemic vasculitis are relatively uncommon, the plethora of reports in the past 2 years on COVID-19 vasculitis necessitates the expansion of the classification of vasculitis associated with probable cause to include severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) vasculitis.