Stroke and Internal Carotid Dissection as the First Manifestation of Giant Cell Arteritis: A Case Report

Introduction: Giant Cell Arteritis (GCA) is a systemic vasculitis that involves medium-sized and larger arteries.GCA rarely can affect the brain-arteries, resulting in ischemic strokes and transient ischemic attacks, whereby the most affected region is vertebrobasilar. Also, it's very unusual that cocurate with large artery dissections. We describe a patient with anterior brain territory stroke and right internal carotid dissection as the first manifestation of GCA.Case report: A 51-year-old man was presented with sudden onset of right-side blurred vision, frozen movements, and ptosis in the right eye and left side paresis. There was a history of right-sided frontotemporal headache, diabetes mellitus, and dyslipidemia. The Laboratory tests show erythrocyte sedimentation rate (ESR)=90, C-reactive protein (CRP)=15mg/L. CT scan and brain MRI indicated an acute ischemic infarction in the right frontal region. CT-Angiography has shown internal carotid artery dissection. Due to the presentation and lab tests, we started Methylprednisolone 1 gr for treating GCA, and temporal artery biopsy was positive for GCA in pathology findings. After ten days, inflammatory markers were reduced (ESR:40). Besides improving headaches, there was no significant change in eye deficits and reduction of left limb's force.Conclusion: Due to the noisy symptoms in patients with Stroke and carotid dissection, the diagnosis of GCA may be neglected as an underlying cause. The association of high CRP levels and the rate of ESR with Stroke, although nonspecific, should draw some attention to vasculitis, topped by GCA.

Giant cell arteritis complicated by tongue necrosis and bilateral cerebellar ischaemic stroke

Giant cell arteritis (GCA) typically presents with headache, scalp tenderness or visual disturbance. Other symptoms include orofacial pain, constitutional symptoms and ischaemic stroke. An 81-year-old woman with a background of type-2 diabetes and hypertension presented with headache, oral pain and right visual loss. Examination showed hypertension, nodular temporal arteries, reduced visual acuity and suspected oral candida. Inflammatory markers were raised and she was diagnosed with GCA and commenced on corticosteroids. During treatment she developed tongue ulceration, then acute vertigo and incoordination with nystagmus and ataxia. Neuroimaging confirmed bilateral, cerebellar ischaemic strokes and temporal artery biopsy was consistent with GCA. With corticosteroids and secondary prevention of stroke measures she is now functionally independent. Oral pain is an uncommon symptom of GCA and delays in recognition may lead to catastrophic consequences. Clinicians should be aware of uncommon presentations and to optimise additional ischaemic stroke risk-factors.

159 Acute coronary syndrome in patient with giant cell arteritis: a fascinating case

Aims The diagnosis of acute coronary syndrome with persistent ST segment elevation, although apparently simple to detect by electrocardiographic abnormalities, can sometimes be insidious due to a difficult differential diagnosis with aortic dissection. Performing a coronary angiography in case of aortic dissection can be potentially life-threatening so, when suspected, this diagnosis needs to be rule out. Furthermore, coronary vasculitis is a rare but devastating complication of giant cell arteritis. Methods and results We describe a case of 62-year-old man hospitalized for acute coronary syndrome with persistent ST segment elevation involving the inferior wall. Ha was a heavy smoker with familiar history of cardiovascular disease and recent suspicion of Horton arteritis with aortic involvement under investigation, no previous cardiological events. Presenting symptoms are chest pain, sweating, bradycardia, and hypotension. In the emergency department, due to the history of suspected arteritis, chest CT scan was performed in order to rule out the hypothesis of aortic dissection before proceeding to coronarography. No signs of aortic rupture but the presence of thickening of the aorta from the arch to the carrefour was found. The coronary angiography showed critical stenosis in the medial tract of the circumflex artery and thrombotic subocclusion in the middle segment of the right coronary (culprit lesion—Figure 1) that was treated with PCI using drug-eluting stent. The echocardiogram showed mild left ventricular disfunction (EF 53%) due to posterolateral hypokinesia, no other pathological findings. Blood tests revealed anaemia, elevated platelets, and an increase of inflammation markers. Peak Hs-troponin was 18 000 ng/L (n.v. < 0.02 ng/ml). After 2 days we performed elective revascularization of the circumflex artery using a drug-eluting stent. Considering the medical history, we performed temporal artery biopsy and a total body PET scan that showed accumulation of radioactive tracer from the aortic arch to the sub-renal abdominal aorta, with a thickened wall. After that empirical corticosteroid therapy was started and some days later the result of the temporal artery biopsy confirmed the diagnosis of Horton arteritis. The patient was discharged to a rehabilitation structure in good general condition with cardioactive and corticosteroid therapy after 9 days of hospitalization. Conclusions Horton arteritis usually involves the external carotid artery and its branches, but sometimes inflammation of the aorta occurs in a subgroup of patients and rarely can involve the coronary arteries. An acute coronary syndrome in patients with Horton arteritis can be very insidious because aortic dissection can be the hidden cause, so chest CT scan must be performed before coronary angiography to avoid acute complication associated with an elevated mortality. Furthermore, being the vasculitic inflammation the trigger of the coronary involvement immunosuppressive therapy could be helpful to prevent subsequent acute coronary events.

A case of giant cell arteritis lacking typical symptoms presenting with recurrent cerebellar infarctions: A case report and case-based review

ABSTRACT Giant cell arteritis (GCA) occasionally presents with ischaemic stroke. Generally, symptoms related to GCA or elevated levels of inflammation markers would be a clue for the diagnosis of GCA. However, we encountered a rare case of GCA that presented with recurrent cerebellar infarctions without symptoms related to GCA (headache, fever, or jaw claudication). Furthermore, C-reactive protein levels, measured at the time of two of the stroke attacks, were within the normal range. On physical examination, the temporal arteries were prominent and weakly pulsatile. Temporal artery ultrasonography showed halo signs, and temporal artery biopsy revealed GCA. To our knowledge, this is the first case of GCA presenting with recurrent ischaemic stroke lacking GCA features but diagnosed before death. Considering this case-based review, we suggest that GCA may have been missed in elderly patients with ischaemic stroke, especially in those with posterior circulation infarction. Therefore, physical examination of the temporal arteries, temporal artery ultrasonography, and vessel wall magnetic resonance imaging may be useful in those patients.

Clinical presentation and treatment response in patients with polymyalgia rheumatica and giant cell arteritis during a 40-week follow-up

Objectives To study the clinical features of polymyalgia rheumatica and/or giant cell arteritis (PMR/GCA) and clinical predictors of treatment response during a 40-week follow-up period. Method Clinical data on 77 patients with newly diagnosed PMR/GCA who were treated by oral glucocorticoids were gathered at baseline and during 40-week follow-up period. A unilateral temporal artery biopsy (TAB) and 18 F-FDG PET/CT were undertaken at diagnosis. In total, each patient was seen at 5 occasions i.e. baseline, weeks 4, 16, 28, and 40. Treatment response was assessed considering clinical evaluations and results of inflammatory markers. Results Of 77 patients (49(63.6%) female, mean age : 71.8 ± 8.0), 64(83.1%) patients had pure PMR, 10(13.0%) concomitant PMR and GCA, and 3(3.9%) pure GCA. The patients reported clinical symptoms except scalp pain and duration of morning stiffness improved significantly at week 4 and remained lower at week 40 compared with the relative frequencies at baseline. Besides, all components of physical examination showed significant improvement and remained lower at week 40 compared with the baseline. 68.7%, 62.9%, 44.1% and 33.3% of the patients had a complete response at weeks 4, 16, 28, and 40, respectively. Several clinical features including female gender, younger age, fewer relapse, and lower level of baseline ESR were significantly associated with a better treatment response. Treatment response during follow-up period was independent of TAB results and FDG uptakes on 18 F-FDG PET/CT at diagnosis. Conclusion Obtaining valid disease specific outcome measures for evaluating treatment efficacy in PMR and GCA, that can be applied universally is clearly an unmet clinical need. Trial registration ClinicalTrials.gov, https://clinicaltrials.gov, NCT02985424

Short-Facelift Approach in Temporal Artery Biopsy: Is It Safe?

Giant cell arteritis (GCA) is a quite common panarteritis of the elderly that affects medium- and large-size arteries. Despite the increasing role of imaging with advancing technology, the gold standard for the diagnosis of GCA is still the temporal artery biopsy. A described complication of superficial temporal artery biopsy (STAB), for which incidence is not clear, is the accidental damage of the frontal branch of the facial nerve. In this paper, we described the short-scar facelift surgical approach for STAB on 23 consecutive patients who underwent unilateral superficial temporal artery biopsy for GCA suspicion. We collected data in terms of postoperative complications, biopsy specimen length, biopsy result and cosmetic appearance of the scar. In our experience, this surgical approach combines the advantage of avoiding incisions within the dangerous anatomical area, minimizing the risk of facial nerve damage, with an acceptable complication rate and a good final aesthetic result which avoids visible scarring.

Presentation and Real-World Management of Giant Cell Arteritis (Artemis Study)

Background: Few studies of daily practice for patients with giant cell arteritis (GCA) are available. This French study aimed to describe the characteristics and management of GCA in a real-life setting.Methods: Cross-sectional, non-interventional, multicenter study of patients ≥50 years old who consulted hospital-based specialists for GCA and were under treatment. Patient characteristics and journey, diagnostic methods and treatments were collected. Descriptive analyses were performed.Results: In total, 306 patients (67% females, mean age 74 ± 8 years old) were recruited by 69 physicians (internists: 85%, rheumatologists: 15%); 13% of patients had newly diagnosed GCA (diagnosis-to-visit interval <6 weeks). Overall median disease duration was 13 months (interquartile range 5–26). Most patients were referred by general practitioners (56%), then ophthalmologists (10%) and neurologists (7%). Most common comorbidities were hypertension (46%), psychiatric disorders (10%), dyslipidemia (12%), diabetes (9%), and osteoporosis (6%). Initial GCA presentations included cranial symptoms (89%), constitutional symptoms (74%), polymyalgia rheumatica (48%), and/or other extra-cranial manifestations (35%). Overall, 85, 31, 26, and 30% of patients underwent temporal artery biopsy, high-resolution temporal artery Doppler ultrasonography, 18FDG-PET, and aortic angio-CT, respectively. All patients received glucocorticoids, which were ongoing for 89%; 29% also received adjunct medication(s) (methotrexate: 19%, tocilizumab: 15%). A total of 40% had relapse(s); the median time to the first relapse was 10 months. Also, 37% had comorbidity(ies) related to or aggravated by glucocorticoids therapy.Conclusion: This large observational study provides insight into current medical practices for GCA. More than one third of patients had comorbidities related to glucocorticoid therapy for a median disease duration of 13 months. Methotrexate and tocilizumab were the most common adjunct medications.

Color doppler ultrasound and the giant cell arteritis probability score for the diagnosis of giant cell arteritis: a Canadian single center experience

Objectives To compare accuracy of colour doppler ultrasonography (CDUS) and temporal artery biopsy (TAB) to establish the final diagnosis of GCA and to determine how the giant cell arteritis probability score (GCAPS) performs as a risk stratification tool. Methods Descriptive statistics were performed on a retrospective cohort of patients referred to our vasculitis referral center between July 1st, 2017 and October 1st, 2020 for suspected GCA. CDUS, TAB, center-specific TAB (vasculitis center vs.s referring hospitals) and GCAPS were compared against the final diagnosis of GCA as determined by a GCA expert; CDUS was also compared with TAB results. Results Data from 198 patients were included: 60 patients with GCA and 138 patients without GCA. Sixty-two patients had a TAB. Using the final diagnosis by a GCA expert as a reference, sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative predictive value (NPV) were 93.3%, 98.5%, 96.6%, 97.1% for CDUS; and 69.2%, 100%, 100%, 81.8% for TAB. The false negative rate was 6.7% for CDUS and 30.8% for TAB. False negative TAB mostly occurred when performed in referring hospitals (57.1%) as opposed to our vasculitis center (21.1%). With a cut-off at 9.5 points, Se for GCAPS was 98.3% while Sp was 74.3%. Conclusion CDUS of the temporal and axillary arteries showed a high sensitivity and specificity and helped to diagnose GCA in patients with negative TAB. We validated that GCAPS is a useful clinical tool with a score < 9.5 making the diagnosis of GCA improbable.