Jaw Claudication Research Articles

Ultrasound in GCA: Halo Sign Quantification and Visual Symptoms, Systemic Inflammation and Relapse Risk.

A sonographic scoring system, termed Halo count and Halo score, of temporal and axillary arteries (TAXA) in suspected giant cell arteritis (GCA) has been proposed for outcome prognostication. We conducted a retrospective review into the relationship of Halo count and Halo score and clinical-laboratory parameters amongst patients diagnosed with GCA via our rapid-access pathway to determine whether these measures should form part of our local routine clinical practice. This review of TAXA ultrasound (US) images in patients with diagnosed GCA did not identify any correlation between Halo count/score and ocular symptoms, jaw claudication, 6-month relapse risk or inflammatory markers. This suggests that further prospective evaluation of Halo count and -score is required before adopting these measures into routine US scanning of TAXA for suspected GCA.

Predictors of Permanent Vision Loss in Giant Cell Arteritis

ABSTRACT To report on the occurrence and characteristics of eye manifestations and determine the predictors of permanent vision loss (PVL) in patients with giant cell arteritis. Case-control study. Retrospective cohort study of 258 patients diagnosed with giant cell arteritis (GCA) over a 20- year period at a single institution. Eighty nine of 258 patients (34.5%) with GCA had vision symptoms and 56 (21.7%) developed PVL. Acute loss of vision with no preceding ocular symptoms occurred in 28.5%; for those with symptoms, median time to vision loss was 4.5 days. Bilateral clinical eye involvement was present in 38%, mostly anterior ischemic optic neuropathy (AION), rarely vascular occlusions. Vision loss was severe with acuity of count fingers or less or mean deficit −20 decibels or less in at least one eye in 80% of patients with PVL. Following initiation of GC treatment, 23% of patients continued to have further vision loss and only 5% showed improvement. We found predictors of PVL by multivariate analysis to be increased age (OR 1.05, p0.040), jaw claudication (OR 2.29, p = .040), diplopia (OR 2.74, p = .039), increased platelets (OR 2.98, p = .020) and lower CRP (OR 0.63, p = .004). We found there is a 27.3% decrease in the odds of having PVL when CRP value is doubled, keeping all other variables fixed. Permanent vision loss was 3.07 times more likely in the absence of polymyalgia rheumatica and 4.25 times more likely in patients without headaches. Ocular involvement in GCA is common, usually severe with little chance of recovery. Detailed analysis of visual acuity and visual field loss in our PVL cohort adds valuable data to the literature which lacks this information. Older patients with jaw claudication, diplopia, and increased platelets are at higher risk for permanent vision loss, especially when PMR and headache are absent and acute phase reactants lower.

Self-limited giant cell arteritis: The dilemma associated with its diagnosis and treatment.

Giant cell arteritis (GCA) can result in visual loss and other sequelae. An 81-year-old man presented with a one-week history of fever. He had bilateral temporal headache, jaw claudication, tenderness of the temporal arteries and a recent skin rash. A temporal artery biopsy showed typical GCA, but the symptoms were self-limiting. We continued close observation, without administering prednisolone treatment. Five months later, the symptoms did not recur, and prednisolone again was not administered. Our patient presented with an atypical course of GCA that created a clinical dilemma. The final diagnosis was self-limiting GCA.

New-onset of giant cell arteritis with ischemic optic neuropathy following the seventh-dose of COVID-19 mRNA vaccination: A case report and literature review.

Coronavirus disease (COVID-19) vaccines have demonstrated excellent efficacy in reducing the morbidity and severity of the disease. However, some patients have been reported to develop systemic rheumatic diseases, such as rheumatoid arthritis, myocarditis, Guillain-Barre syndrome, and giant cell arteritis (GCA) following COVID-19 vaccination. We present a case of GCA with ischemic optic neuropathy following COVID-19 mRNA vaccination. A 73-year-old woman developed headache, myalgia, scalp tenderness, and jaw claudication 4 days after her seventh dose of the vaccination; she also developed severe visual disturbances 1 month after the vaccination. The blood examination tests showed an increased serum C-reactive protein level and erythrocyte sedimentation rate. The echogram for the temporal artery showed a halo sign. Ophthalmic examination revealed ischemic optic neuropathy in both eyes. The patient was treated with a high-dose glucocorticoid and tocilizumab under the diagnosis of GCA with ischemic optic neuropathy, obtaining mild improvement of the symptoms. This report underscores the need for clinical vigilance and further data collection regarding GCA cases after COVID-19 vaccination.

Antiplatelet therapy to prevent ischemic events in giant cell arteritis: protocol for a systematic review and meta-analysis

BackgroundGiant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and myocardial infarction. Oral antiplatelet therapy has been hypothesized to reduce GCA-related ischemic events. However, previous studies have demonstrated conflicting results regarding the efficacy of antiplatelet agents in GCA. The objective of this systematic review is to assess the safety and efficacy of antiplatelet therapy for the prevention of these events in adults with giant cell arteritis.MethodsIn this systematic review, we will include randomized controlled trials (RTCs), quasi-randomized trials, non-randomized intervention studies, cohort studies, and case–control studies on patients with new-onset or relapsing GCA. The intervention of interest will be pre-existing use or initiation of an oral antiplatelet medication (aspirin, clopidogrel, prasugrel, or ticagrelor) at GCA onset or relapse. The comparator of interest will be the absence of antiplatelet therapy. Endpoints will be evaluated after 6 and 12 months of follow-up. The primary outcome will be GCA-related ischemic events, including permanent blindness, stroke, myocardial infarction, and ischemic event-related deaths. Adverse events such as major bleeding and death caused by a bleeding event will be assessed.DiscussionGCA-related ischemic events are catastrophic, sudden, often irreversible, and lead to significant morbidity. Antiplatelet agents are affordable, accessible, and could be effective for the prevention of these events. Nevertheless, the potential benefits of platelet aggregation inhibition must be weighed against their associated risk of bleeding. Assessing the efficacy and safety of antiplatelet therapy in GCA is therefore clinically important.Systematic review registrationOur systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO, registration number CRD42023441574.

Fulminant Giant Cell Arteritis with Tongue Autoamputation

Abstract Giant cell arteritis (GCA) is a systemic vasculitis predominantly affecting large and medium-sized arteries. While headache, scalp tenderness, jaw claudication, and visual disturbances are common presentations in patients over 50 years old, rare manifestations such as tongue necrosis can pose diagnostic challenges. We present an 86-year-old female who initially presented with visual disturbances, jaw claudication, and constitutional symptoms. She was diagnosed with GCA based on clinical features, elevated inflammatory markers, and ultrasound findings of thickened temporal arteries. Despite treatment with high-dose corticosteroids, she subsequently developed spontaneous amputation of the anterior one-third of her tongue due to bilateral lingual artery occlusion, a rare complication occurring in approximately 1% of GCA cases. Despite treatment, the patient’s condition rapidly deteriorated, and she succumbed to the illness within 3 weeks of the initial presentation. This case highlights an atypical and fulminant presentation of GCA with tongue autoamputation, emphasizing the need for early recognition and aggressive management to prevent devastating ischemic complications.

E023 Intimomedial mucoid degeneration. A rare vascular disease mimicking giant cell arteritis presentation

Abstract Background/Aims The accuracy of giant cell arteritis (GCA) diagnosis has greatly improved in recent years with increasing use of fast track clinics and vascular ultrasound. The “halo” sign is recognised as confirmation of a positive test and is considered virtually pathognomonic of GCA in the setting of a correlating clinical presentation. There are caveats to this and “false halos” have been reported associated with arterial thickening seen in other conditions including atherosclerosis and lymphoma. In this case a focal area of arterial wall thickening mimicking a halo on ultrasound was due to a rare vascular condition called intimomedial mucoid degeneration. Methods A 63 year old female presented via the direct assessment unit to the fast track GCA service. She reported an 8 week history of a tender right forehead lump and a 3 week history of associated new headache with marked tenderness at the site of the lump. The headache was severe and had become persistent. Mild shoulder stiffness was present. No jaw claudication. Blurring while wearing glasses noted but no definite visual change. CRP 2mg/L, ESR at usual baseline of 30mm/hr. Other indices normal. On examination: No shoulder restriction. No visual field deficit. Palpable pulsatile area of artery right side of forehead that was exquisitely tender with less marked tenderness of the artery adjacent to this. No symptoms on left side. GCA probability score (GCAPS) 10 indicating intermediate probability of GCA. Results Temporal artery ultrasound performed using GE Logiq e and 10-22mHz probe. Left side imaged entirely normally. Right side symptomatic area imaged as a focal asymmetric thickened area of frontal branch artery measuring 1.5mm maximally in compression. The frontal branch artery imaged normally proximal and distal to this as did the right common superficial and parietal branches. Features were unusual however she was commenced on prednisolone 40mg daily pending further investigations. Eye assessment detected no ischaemic concerns. She returned for reassessment after 4 days. There was some symptomatic improvement however the tender lump persisted. A second opinion on the ultrasound was sought from the interventional radiologist, who felt the focal area may be a lymph node, with thickened artery adjacent. Surgical exploration and temporal artery biopsy was subsequently performed. Conclusion Histology showed there was actually mucoid change with eccentric thickening of the artery with aneurysmal change in keeping with the rare diagnosis of intimomedial mucoid degeneration. This is a very rare condition which causes predisposition to arterial aneurysm formation, and typically presents with acute pain of the artery involved. This lady had developed an aneurysm which was excised at the time of the biopsy, relieving her symptoms. This case is a reminder to pursue additional investigations and tissue diagnosis in the setting of unusual clinical and imaging findings. Disclosure S. Black: None. M. Wray: None. H. Stewart: None. E. Durkan: None. V. Serafimov: None. P. Davey: None. L. Smith: None.

P025 Routine Giant-cell arteritis turned Heidenhain variant of sporadic Creutzfeldt-Jakob disease: a mysterious case report!

Abstract Background/Aims Giant-cell arteritis (GCA) is a large vessel vasculitis predominantly affecting those above 50 years of age. Creutzfeldt-Jacob disease (CJD) is not a well-recognised differential diagnosis of GCA presentation. Prion diseases are progressive, currently untreatable and ultimately fatal. Methods We report the case of a 76-year-old female who had an eventful hospital stay of 37 days. She has a history of asthma, hypertension, ulcerative colitis and right-sided GCA 5 years back (normal inflammatory markers & biopsy). Results The patient presents to Harlow ED with a right temporal headache with tenderness, jaw claudication symptoms, blurred vision, diplopia, dizziness and painful right eye movements. Her CRP was <1 and ESR was 5. The plan was to treat it as a recurrence of her GCA and to continue with the high dose Prednisolone started by the ED. CT Head was normal. Owing to poor response, IV Methyl Pred was given. On day six, patient was re-admitted with worsening headache and generalised decline. Ophthalmology review noted multiple visual field defects and treated as GCA. A neurology review noted the patient to be encephalopathic. Advice was for MRI head and CSF analysis - both NAD. On day 12 she had ongoing headache, diplopia, poor vision/memory, and reduced hearing in right ear, but alert and following instructions. On day 17, ENT suggestion was to treat as Meniere’s and start on Betahistine. On day 22, the patient had a TIA episode and discussed with Stroke team. Repeat CT Head was nil acute. By day 26, the patient was unresponsive intermittently and was treated as hypoactive delirium. On day 31 a seizure activity was noticed. Neurology review on day 33 noted rapid decline- the patient being mute, seizure/atypical encephalitis. MRI-Head with contrast showed the possibility of CJD. The very next day patient was reviewed by the team from National CJD Research and Surveillance team, Edinburgh. The formal diagnosis given was: probable Sporadic CJD (visual/Heidanhain variant), rapidly progressive cognitive decline with visual signs, myoclonus, and akinetic mutism, positive MRIs, bland CSF, no clear iatrogenic or variant risk factors, and no credible alternative diagnosis. The distressed family members were updated and the patient was put on EoL pathway. She died peacefully four days later (Day 37). Conclusion The importance of this case is the conflicting inputs from at least five specialities in a rapidly deteriorating patient. A background of previous right-sided GCA did prompt initial treatment in those lines. MRI Head DWI sequence did show the classic ‘cortical ribboning’ with right caudate and lentiform hyperintensity; which clinched the mysterious diagnosis of this rare disease which requires public health surveillance. CJD is to be suspected in any undiagnosed rapidly progressive mental deterioration or dementia, often with behavioural abnormalities and myoclonus. Disclosure S. Narikkoottungal: None. K. Gunasekera: None. K. Ahmed: None.

Giant Cell Arteritis.

Giant cell arteritis (GCA) is the most common primary vasculitis and is associated with potential bilateral blindness. Neither clinical nor laboratory evidence is simple and unequivocal for this disease, which usually requires rapid and reliable diagnosis and therapy. The ophthalmologist should consider GCA with the following ocular symptoms: visual loss or visual field defects, transient visual disturbances (amaurosis fugax), diplopia, eye pain, or new onset head or jaw claudication. An immediate ophthalmological examination with slit lamp, ophthalmoscopy, and visual field, as well as color duplex ultrasound of the temporal artery should be performed. If there is sufficient clinical suspicion of GCA, corticosteroid therapy should be initiated immediately, with prompt referral to a rheumatologist/internist and, if necessary, temporal artery biopsy should be arranged. Numerous developments in modern imaging with colour duplex ultrasonography, MRI, and PET-CT have the potential to compete with the classical, well-established biopsy of a temporal artery. Early determination of ESR and CRP may support RZA diagnosis. Therapeutically, steroid-sparing immunosuppression with IL-6 blockade or methotrexate can be considered. These developments have led to a revision of both the classification criteria and the diagnostic and therapeutic recommendations of the American College of Rheumatologists and the European League against Rheumatism, which are summarised here for ophthalmology.

Cost-effectiveness analysis of the diagnosis of temporal arteritis

Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids.Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, doppler ultrasound) and therapeutic strategies (corticosteroid suspension). Material and methodObservational, retrospective study has been carried out on patients with AT (2012–2021). Demographic data, comorbidities, signs and symptoms suggestive of AT were collected. AT was diagnosed with a score ≥ 3 according to American College of Rheumatoloy criteria (ACR-SCORE). The costs of diagnosis and treatment modification were analysed. ResultsSeventy-five patients have been included, median age 77 (46–87) years. Headache, temporal pain and jaw claudication were significant for the diagnosis of TA.Patients with a halo on Doppler ultrasound and a positive biopsy have significantly elevated ESR and CRP compared to patients who do not.The cost of the AT diagnosis was 414.7 euros/patient. If we use ACR-SCORE ≥ 3-echodoppler it is 167.2 є/patient (savings 59.6%) and ACR-SCORE ≥ 3-biopsy 339.75 є/patient (savings 18%). If the corticosteroid was removed and a biopsy was performed, 21.6 є/patient (94.7% savings), if the corticosteroid was removed and Doppler ultrasound was performed, 10.6 є/patient (97.4% savings). ConclusionsHeadache, temporary pain and jaw claudication are predictors of AT. Elevated ESR and CRP are predictors of positive biopsy and presence of halo on ultrasound.The uses of ACR-SCORE ≥ 3 with Doppler ultrasound or biopsy, and with corticosteroid suspension, are cost-effective.

Upper respiratory tract and orofacial manifestations of new-onset giant cell arteritis: results from a large, prospective inception cohort study.

Giant cell arteritis (GCA) often features upper respiratory tract (URT) and orofacial manifestations, which signal the involvement of external carotid artery branches. In this study, we aimed to describe the frequency of various URT/orofacial symptoms at GCA onset, as well as the main characteristics of patients presenting these symptoms. We included all patients who were newly diagnosed with GCA between 1976 and April 2022 at the Internal Medicine Department of a tertiary-care hospital. Ten URT or orofacial symptoms were prospectively examined systematically in each patient. We used multivariate analyses to identify the GCA characteristics, including URT/orofacial symptoms, associated with temporal artery biopsy (TAB) positivity. At least 1 URT/orofacial symptom was present in 68.6% of the 599 patients (3 or more symptoms in 30% of cases). Jaw claudication, maxillary pain, and pain during mouth opening were the most prevalent symptoms. Dry cough was recorded in 17% of cases. GCA patients with URT/orofacial symptoms had more clinical abnormalities of the temporal artery bed and ischaemic ophthalmological complications, but less large-vessel vasculitis according to imaging. The likelihood of a positive TAB was increased in patients with an abnormal temporal artery upon clinical examination (OR 4.16; CI 2.75-6.37, p < 0.001) or jaw claudication (OR 2.18; CI 1.35-3.65, p = 0.002), and decreased in those with hoarseness (OR 0.47; CI 0.26-0.87, p = 0.02) or earache (OR 0.54; CI 0.31-0.95, p = 0.03). Isolated URT/orofacial presentation (i.e., without headache or visual signs) accounted for 5.2% of the entire cohort. Oral-facial symptoms were present in two-thirds of GCS cases. Thus, they could serve as leading clinical clues for a GCA diagnosis, and are a risk factor for permanent visual loss. Several URT/orofacial symptoms such as jaw claudication, hoarseness, and earache influenced the likelihood of a positive TAB. Isolated URT/orofacial presentation of GCA is a rare but potentially challenging occurrence.

Predictive Factors for Biopsy-Negative Giant Cell Arteritis and Alternative Diagnoses in a Neuro-Ophthalmology Context

ABSTRACT Giant cell arteritis is a challenging diagnosis for patients given the high prevalence of negative temporal artery biopsies (TAB). Despite the lack of histopathological evidence of giant cell arteritis in the TAB, patients can still have TAB-negative giant cell arteritis. The purpose of this paper is to analyse the predictors for TAB-negative giant cell arteritis and the alternative diagnosis of biopsy-negative patients without a giant cell arteritis diagnosis. A retrospective electronic database review of all TABs performed at the Royal Victorian Eye and Ear Hospital from February 2015 to May 2020. Logistic regression analysis was performed to determine predictive factors for a diagnosis of TAB-negative giant cell arteritis. In all cases, a clinical diagnosis of TAB-negative giant cell arteritis was determined by a neuro-ophthalmologist. Alternative diagnoses for negative TABs were identified and explored. A total of 368 TABs were analysed with 287 (78%) negative for histopathological evidence of GCA. Twenty-seven (9.4%) patients were diagnosed and treated as TAB-negative giant cell arteritis. The clinical predictors of a TAB-negative giant cell arteritis diagnosis were the presence of jaw claudication (OR 2.77, 95% CI 1.10–6.98) and CRP (OR 1.02, 95% CI 1.00–1.03). Alternative diagnoses included non-specific headache, non-arteritic anterior ischaemic optic neuropathy, retinal vessel occlusions, and ocular nerve palsies. Predictive factors for a diagnosis of TAB-negative giant cell arteritis were jaw claudication and an elevated CRP. Several alternative diagnoses can be considered for patients with a negative TAB in a neuro-ophthalmology context.

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.

Análisis coste/efectivo del diagnóstico de la arteritis de la temporal

Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids.Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, Doppler ultrasound) and therapeutic strategies (corticosteroid suspension). Material and methodObservational, retrospective study has been carried out on patients with TA (2012–2021). Demographic data, comorbidities, signs and symptoms suggestive of TA were collected. TA was diagnosed with a score ≥3 according to American College of Rheumatoloy criteria (ACR-SCORE). The costs of diagnosis and treatment modification were analysed. ResultsSeventy-five patients have been included, median age 77 (46-87) years. Headache, temporal pain and jaw claudication were significant for the diagnosis of TA.Patients with a halo on Doppler ultrasound and a positive biopsy have significantly elevated ESR and CRP compared to patients who do not.The cost of the TA diagnosis was 414.7 euros/patient. If we use ACR-SCORE≥3-echodoppler it is 167.2 €/patient (savings 59.6%) and ACR-SCORE≥3-biopsy 339.75 €/patient (savings 18%). If the corticosteroid was removed and a biopsy was performed, 21.6 €/patient (94.7% savings), if the corticosteroid was removed and Doppler ultrasound was performed, 10.6 €/patient (97.4% savings). ConclusionsHeadache, temporary pain and jaw claudication are predictors of TA. Elevated ESR and CRP are predictors of positive biopsy and presence of halo on ultrasound.The uses of ACR-SCORE≥3 with Doppler ultrasound or biopsy, and with corticosteroid suspension, are cost-effective.

Giant cell arteritis associated with scalp, tongue or lip necrosis: A French multicenter case control study

BackgroundScalp, tongue and/or lip necrosis are rare complications of GCA. ObjectivesTo describe characteristics and outcome of patients with giant cell arteritis (GCA) -related scalp, tongue and/or lip necrosis. MethodsA retrospective nationwide multicenter study included 20 GCA patients with scalp, tongue, and/or lip necrosis diagnosed between 1998 and 2021 and 80 GCA control patients matched for age, sex and management period. Logistic regression analyses were conducted to identify baseline characteristics associated with scalp, tongue and/or lip necrosis. ResultsCompared to controls, patients with scalp, tongue and/or lip necrosis showed significantly more cranial manifestations (headache, p=0.045; scalp tenderness, p=0.006; jaw claudication, p=0.02). No differences were observed between both groups regarding the occurrence of visual symptoms or large vessel involvement. At diagnosis, GCA patients with necrosis more likely received IV methylprednisolone infusions and higher doses of oral prednisone. There were no differences regarding vascular complications during follow up. Compared to controls, survival was decreased in GCA patients with necrosis (p=0.003). In a multivariable logistic regression model, scalp tenderness [odds ratio (OR) 4.81(95 % CI: 1.57, 14.79), p = 0.006] and cognitive disorder [OR 6.42 (95 % CI: 1.01, 40.60), p=0.048] were identified as factors associated to scalp, tongue, and/or lip necrosis. ConclusionOur results suggest that scalp, tongue, and/or lip necrosis is associated to higher mortality in GCA patients. Scalp tenderness and cognitive disorder were significant factors associated to this very rare complication of GCA.

Giant cell temporal arteritis: a clinicopathological study with emphasis on unnecessary biopsy.

Temporal artery (TA) biopsy is commonly used for the diagnosis of giant cell arteritis (GCA). However, a positive biopsy is no longer mandatory for diagnosis. This study aims to correlate the histopathological findings of TA biopsies in suspected cases of GCA to the clinical presentation in an ophthalmic tertiary eye care center to draw useful conclusions and advocate the possible implementation of guidelines for TA biopsy. Data was collected from patients' medical records including, demographics, clinical data, and histopathological findings and diagnosis. The 2022 American College of Rheumatology/ European Alliance of Associations for Rheumatology (ACR/EULAR) criteria have been used and partially adopted as a guide to compare the variables between TA biopsy-positive and negative groups as well as the TA biopsy-positive group and the group of patients with TA biopsy showing atherosclerosis. Out of the total 35 patients who underwent a TA biopsy during the period of 23 years, 22.9% of patients had histopathological findings consistent with GCA and 42.9% had TA atherosclerotic changes, while the remaining 34.3% had histologically unremarkable TA. The mean age of all patients was 66 ± 10.9 years. Slightly more than half were females (54.3%) and the remaining were males (45.7%). In the group with positive TA biopsies, the mean age was 71 ± 8.4 years with a higher female predominance (female-to-male ratio of 5:3). The mean diagnostic clinical score used in our study was higher (7.5 ± 2.33) in the GCA-positive group when compared to the other groups with statistical significance (mean of 4.85 ± 2.01 in patients with overall GCA-negative biopsies and 5.13 ± 2.10 in the group with atherosclerosis). Other three clinical variables that were found to be statistically significant in the GCA biopsy-positive group were scalp tenderness, jaw claudication, and optic nerve pallor. The mean age (71 ± 8.4 years) and the female predominance of GCA in our group of patients with positive TA biopsy (62.5%) was like other reports. In our study 22.9% of performed TA biopsies over the period of the study were positive confirming the diagnosis of GCA on histological exam, which was similar to another report and is considered to be relatively low. The incorporation of increased clinically focused assessments and algorithms, with the aid of the ACR/EULAR criteria, may decrease the frequency of TA biopsies that carries unnecessary cost and risk of procedure-related morbidity. We highly recommend applying the age of ≥ 50 years as an initial criterion for diagnosis, followed by the consideration of the statistically significant clinical features: scalp tenderness, jaw claudication, and optic nerve pallor.

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.

Ischémie subaiguë bilatérale des membres supérieurs révélant une Maladie de Horton, une présentation assez rare : à propos d'un cas et revue de la littérature

Study's goalOur goal is to enrich the medical literature by sharing our experience in managing a case of sub-acute upper limb ischemia that revealed Horton's disease. This is particularly relevant given the current lack of well-established guidelines. IntroductionAcute upper limb ischemia is rarely seen in Horton's disease. Case ReportWe present a case of a 63-year-old female patient with sub-acute ischemia in both upper limbs, accompanied by jaw claudication and absence of bilateral temporal pulses. The CT Angiography scan revealed bilateral occlusions and stenosis of the axillary and sub-clavier arteries, as well as involvement of the external carotid arteries and its branches. The Arteriography confirmed abnormalities in the medium-sized arteries of the supra-aortic trunks. The histopathology examination of the temporal artery biopsy was negative. The patient received emergency corticosteroid therapy followed by an immunosuppressant. Due to the improved clinical condition of the upper limbs, surgical intervention was not performed.

Jaw claudication and jaw stiffness in giant cell arteritis: secondary analysis of a qualitative research dataset.

Jaw symptoms can be a vital clue to the diagnosis of GCA. Guidelines recommend more intensive treatment if jaw claudication is present. We sought to explore how patients with GCA described their jaw symptoms. We carried out a secondary, qualitative analysis of interview data from 36 participants from the UK (n = 25) and Australia (n = 11), originally collected in order to develop a patient-reported outcome measure for GCA. In all cases, GCA had been confirmed by biopsy/imaging. Interview transcripts were organized within QSR NVivo 12 software and analysed using template analysis. Themes were refined through discussion among the research team, including a patient partner. Twenty of 36 participants reported jaw symptoms associated with GCA. The median age of these 20 participants was 76.5 years; 60% were female. Five themes were identified: physical sensations; impact on function; impact on diet; symptom response with CSs; and attribution to other causes. Physical sensations included ache, cramp, stiffness and 'lockjaw'. Functional impacts included difficulty in eating/chewing, cleaning teeth, speaking or opening the mouth. Dietary impacts included switching to softer food. Response to CSs was not always immediate. Jaw symptoms were initially mis-attributed by some participants to arthritis, age or viral illnesses; or by health-care professionals to a dental cavity, ear infection or teeth-grinding. Jaw symptoms in GCA are diverse and can lead to diagnostic confusion with primary temporomandibular joint disorder, potentially contributing to delay in GCA diagnosis. Further research is needed to determine the relationship of jaw stiffness to jaw claudication.

Diagnostic tardif d’une artérite à cellules géantes en rémission

Giant-cell arteritis (GCA) is a classical cause of chronical inflammation (CI) in the elderly, causing headaches, scalp hypersensitivity and jaw claudication. We describe a patient with a GCA revealed with a year-long biological inflammation and weight loss. Diagnosis was performed on a systematic temporal artery biopsy showing typical histological features. No treatment was intended as the patient had a spontaneous remission, maintained at one year of follow-up. This case highlights the benefit of a systematic temporal artery biopsy to explore CI and reminds us that GCA may undergo spontaneous remission.