Increased incidence of giant cell arteritis and associated stroke during the COVID-19 pandemic in Spain: A nation-wide population study.

INTRODUCTION: SARS-CoV-2 infection and COVID-19 vaccines might have increased the incidence of giant-cell arteritis (GCA) and the risk of associated stroke in Spain. METHODS: Retrospective nation-wide observational analysis of all adults hospitalized with GCA in Spain during 5 years (Jan-2016 and Dec-2021). The incidence and proportion of admissions with or because of GCA and GCA-associated stroke were compared between pre-pandemic (2016-2019) and pandemic (2020 and 2021) years. Sensitivity analyses were conducted for the different COVID-19 waves and vaccine timing schedules. RESULTS: A total of 17,268 hospital admissions in patients diagnosed with GCA were identified. During 2020 there were 79.3 and 8.1 per 100,000 admissions of GCA and GCA-associated stroke, respectively. During 2021 these figures were 80.8 and 7.7 per 100,00 admissions, respectively. As comparison, yearly admissions due to GCA and GCA-associated stroke were 72.4 and 5.7 per 100,00, respectively, during the pre-pandemic period (p < 0.05). Coincident with the third wave of COVID-19 (and first vaccine dosing), the rate of GCA-associated stroke admissions increased significantly (from 6.7 to 12%; p < 0.001). Likewise, there was an increase in GCA-associated stroke (6.6% vs 4.1%, p = 0.016) coincident with the third dose vaccination (booster) in patients older than 70 at the end of 2021. In multivariate analysis, only patients admitted during the third COVID-19 wave (and first vaccine dosing) (OR = 1.89, 95% CI 1.22-2.93), and during the third vaccination dosing in patients older than 70 (booster) (OR = 1.66, CI 1.11-2.49), presented a higher GCA-associated stroke risk than the same months of previous years after adjustment by age, sex, classical cardiovascular risk factors and COVID-19 diagnosis. CONCLUSIONS: The COVID-19 pandemic led to an increased incidence of GCA during 2020 and 2021. Moreover, the risk of associated stroke significantly risen accompanying times of COVID-19 vaccine dosing, hypothetically linked to an increased thrombotic risk of mRNA-SARS-CoV-2 vaccines. Hence, forthcoming vaccine policies and indications must weigh the risk of severe COVID-19 with the risk of flare or stroke in patients with GCA.

Outcomes during and after long-term tocilizumab treatment in patients with giant cell arteritis.

OBJECTIVE: To assess outcomes in giant cell arteritis (GCA) patients during and after long-term tocilizumab (TCZ) treatment. METHODS: Retrospective analysis of GCA patients treated with TCZ at a single centre (2010-2022). Time to relapse and annualised relapse rate during and after TCZ treatment, prednisone use, and safety were assessed. Relapse was defined as reappearance of any GCA clinical manifestation that required treatment intensification, regardless of C reactive protein levels and erythrocyte sedimentation rate. RESULTS: Sixty-five GCA patients were followed for a mean (SD) of 3.1 (1.6) years. The mean duration of the initial TCZ course was 1.9 (1.1) years. The Kaplan-Meier (KM)-estimated relapse rate at 18 months on TCZ was 15.5%. The first TCZ course was discontinued due to satisfactory remission achievement in 45 (69.2%) patients and adverse events in 6 (9.2%) patients. KM-estimated relapse rate at 18 months after TCZ discontinuation was 47.3%. Compared with patients stopping TCZ at or before 12 months of treatment, the multivariable adjusted HR (95% CI) for relapse in patients on TCZ beyond 12 months was 0.01 (0.00 to 0.28; p=0.005). Thirteen patients received >1 TCZ course. Multivariable adjusted annualised relapse rates (95% CI) in all periods on and off TCZ aggregated were 0.1 (0.1 to 0.2) and 0.4 (0.3 to 0.7), respectively (p=0.0004). Prednisone was discontinued in 76.9% of patients. During the study, 13 serious adverse events occurred in 11 (16.9%) patients. CONCLUSION: Long-term TCZ treatment was associated with remission maintenance in most patients with GCA. The estimated relapse rate by 18 months after TCZ discontinuation was 47.3%.

Giant Cell Arteritis: A Case-Based Narrative Review of the Literature.

PURPOSE OF REVIEW: Giant cell arteritis (GCA) is a chronic, inflammatory condition, primarily affecting the medium and larger arteries. The purpose of this narrative review is to describe GCA in the context of headache and facial pain, based on a case and the available current literature. Understanding the etiology, pathophysiology, the associated conditions, and the differential diagnoses is important in managing GCA. RECENT FINDINGS: In a patient presenting with unilateral facial/head pain with disturbances of vision, GCA should be considered in the differential diagnosis. There is an association of GCA with several comorbid conditions, and infections including coronavirus-19 (COVID-19) infection. Management of GCA primarily depends upon the identification of the affected artery and prompt treatment. Permanent visual loss and other serious complications are associated with GCA. GCA is characterized by robust inflammation of large- and medium-sized arteries and marked elevation of systemic mediators of inflammation. An interdisciplinary approach of management involving the pertinent specialties is strongly recommended.

Polymyalgia Rheumatica Following COVID-19 Vaccination.

We herein report a 71-year-old woman presented with a fever, arthralgia, general malaise and leg muscle stiffness following administration of the COVID-19 mRNA vaccine (Comirnaty, Pfizer-BioNTech). Laboratory findings showed an elevated C-reactive protein level and erythrocyte sedimentation rate. In addition, Gallium-67 scintigraphy demonstrated an increased uptake in multiple joints. Typing of human leukocyte antigen (HLA) revealed the presence of the DRB1*0404/*0803 allele. These findings met the diagnostic criteria for polymyalgia rheumatica (PMR), and when we started steroid treatment, her symptoms improved rapidly. This patient developed PMR after receiving a COVID-19 mRNA vaccine (Comirnaty, Pfizer-BioNTech). This case is considered to be valuable, as the HLA-DRB1 allele was also confirmed.

COVID-19 outcomes in giant cell arteritis and polymyalgia rheumatica versus rheumatoid arthritis: A national, multicenter, cohort study.

OBJECTIVES: To determine whether giant cell arteritis and polymyalgia rheumatica (GCA/PMR) represent independent risk factors for worse outcomes in COVID-19. METHODS: Observational, national, French, multicenter cohort (NCT04353609) comprising patients aged ≥18 years with confirmed diagnoses of either GCA, PMR or rheumatoid arthritis (RA) having presented COVID-19; those under rituximab were excluded. Primary endpoint was COVID-19 severity in GCA/PMR patients as compared to RA. We also aimed to describe the evolution of GCA/PMR patients following COVID-19. Multinomial logistic regression models were performed, with and without adjustment on pre-specified confounding factors (i.e., age, sex, body mass index, arterial hypertension, diabetes and cardiovascular disease). Unadjusted and adjusted multinomial odds-ratio (OR/aOR) and their 95% confidence intervals (CIs) were calculated as effect size using RA as reference group. RESULTS: Between April 15, 2020, and August 20, 2021, 674 patients [45 (6.6%) GCA, 47 (7.0%) PMR, 582 (86.4%) RA; 62.8 years, 73.2% female] were included. Compared to RA patients, those with GCA/PMR were older and more frequently presented hypertension, diabetes and cardiovascular disease. Severe COVID-19 and death occurred in 24 (26.1%) and 16 (17.8%) patients with GCA/PMR, respectively. Unadjusted analyses revealed higher odds of severe COVID-19 [OR = 3.32 (95% CI 1.89-5.83; p < 0.001)] and death [OR = 3.20 (95%CI 1.67-6.13; p < 0.001)] for GCA/PMR compared to RA. After model adjustment, these odds were attenuated. CONCLUSION: Patients with GCA/PMR were more likely to have severe COVID-19 and higher mortality compared to those with RA. This worse prognosis is mostly due to well known risk factors for the general population rather than vasculitis per se.

Giant cell arteritis with spontaneous remission.

The case of a 75-year-old woman diagnosed with polymyalgia rheumatica (PMR), treated with low doses of prednisone, and with clinical and analytical remission is reported. Two years later, she presented with a clinical picture of giant cell arteritis (GCA), including headache, diplopia, jaw pain, feeling of swelling in both temples, and elevation of acute phase reactants. Symptoms spontaneously subsided 2 weeks later, while analytical parameters improved without any treatment. A high-resolution colour Doppler ultrasound showed thickening of the intima-media complex with 'halo' sign in the right temporal artery. A biopsy of the right temporal artery was performed, although it was not successful, as no artery could be found, and the procedure became more complicated with an eyebrow ptosis due to a lesion in the frontal branch of the facial nerve. GCA diagnosis was based on the clinical, laboratory, and ultrasound findings. The patient was treated with prednisone and methotrexate, without clinical or analytical relapse. Comments are presented on the described cases of GCA with spontaneous remission, and the most appropriate treatments in these cases are discussed. Other peculiarities of the case, such as the progression to GCA more than 2 years after the onset of PMR, and the complications from the temporal artery biopsy are also mentioned.

Thinking Beyond Giant Cell Arteritis in COVID-19 Times.

BACKGROUND: The coronavirus disease 2019 has displayed multi-system manifestations since its first presentation. This article highlights an unusual presentation of COVID-19 that was reviewed by our instituition's otolaryngology and ophthalmology team. METHODS: We present 2 cases of COVID-19 which presented with unilateral otalgia and ipsilateral pulsatile headaches involving the temporal area. They were referred to the otolaryngology team for assessment of otalgia and subsequently referred to the ophthalmology team for possible giant cell arteritis (GCA). Both patients had no jaw claudication, scalp pain, or tenderness. RESULTS: Serology testing showed raised C-reactive protein (CRP) but normal platelets and erythrocyte sedimentation rate. Case 1 was tested for COVID-19 as part of a preoperative workup which returned positive. With a marked similarity in presentation, Case 2 was tested for COVID-19 which also returned positive. CONCLUSIONS: These 2 cases highlight another set of symptoms that COVID-19 patients may present with. In the context of a COVID-19 pandemic, if a patient presents symptoms similar to GCA but with isolated CRP, it should prompt consideration for COVID testing.

Arteritic Anterior Ischemic Optic Neuropathy in the Course of Giant Cell Arteritis After COVID-19.

BACKGROUND Giant cell arteritis (GCA) is an inflammation of large vessels that affects the lining of the arteries and leads to vessel swelling and the eventual reduction of blood flow. This can result in ischemia of the optic nerve, which is known as arteritic anterior ischemic optic neuropathy (AAION). The present case seems noteworthy because the patient developed GCA with the ocular manifestation of AAION shortly after having COVID-19. CASE REPORT A 69-year-old woman was admitted to the Clinic of Ophthalmology after having COVID-19. She reported vision loss in the left eye, which appeared 2.5 weeks after a positive SARS-CoV-2 test. While in the hospital, she was diagnosed with AAION and GCA. The patient was treated with enoxaparin sodium, prednisone, and methotrexate. Three months after the hospitalization, the visual acuity of the left eye was limited to light perception, and optic nerve atrophy was reported. CONCLUSIONS We would like to emphasize the role of SARS-CoV-2 infection as a possible risk factor for the onset of GCA and its ocular manifestations, such as AAION. However, further research is needed to determine the relationship between SARS-CoV-2 infection and GCA. Because some symptoms of the 2 diseases are similar, the diagnosing process might be long and challenging. The diagnosis of GCA should be made as soon as possible to avoid serious complications, such as bilateral vision loss.

Current advances in giant cell arteritis.

PURPOSE OF REVIEW: The aim of this study was to present the latest advances in giant cell arteritis (GCA) care, and recent national and international rheumatology societies guidance which influences clinical practice. RECENT FINDINGS: Cranial ultrasound reduces diagnostic delay and improves clinical outcomes. Immediate high dose glucocorticoids remain the standard treatment for GCA. Controlled trial evidence using Tocilizumab, an interleukin-6 receptor antagonist, shows good clinical efficacy with steroid-sparing effects. SUMMARY: Improved patient outcomes require formalizing pathways to diagnosis and closer liaison with rheumatology for long-term management with second-line therapies.

A Case of Post-COVID-19-Associated Paracentral Acute Middle Maculopathy and Giant Cell Arteritis-Like Vasculitis.

ABSTRACT: A 47-year-old man with a history of COVID-19 infection 2 months before presentation, presented with a scotoma of the paracentral visual field of the right eye. After thorough testing and evaluation, a diagnosis of paracentral acute middle maculopathy (PAMM) was established. Two months later, the patient developed temporal headache and jaw claudication. High-dose steroids were initiated, and workup for giant cell arteritis (GCA) was undertaken. The patient experienced resolution of the symptoms within 24 hours of steroid initiation. ESR, CRP, and temporal artery biopsy results were normal, although all were obtained more than 2 weeks after steroid initiation. To the best of our knowledge, our patient represents the first individual to date to potentially implicate COVID-19 in both small and large vessel vasculitis in the ophthalmic setting.

Giant Cell Arteritis and COVID-19: Similarities and Discriminators. A Systematic Literature Review.

OBJECTIVE: To identify shared and distinct features of giant cell arteritis (GCA) and coronavirus disease 2019(COVID-19) to reduce diagnostic errors that could cause delays in correct treatment. METHODS: Two systematic literature reviews determined the frequency of clinical features of GCA and COVID-19 in published reports. Frequencies in each disease were summarized using medians and ranges. RESULTS: Headache was common in GCA but was also observed in COVID-19 (GCA 66%, COVID-19 10%). Jaw claudication or visual loss (43% and 26% in GCA, respectively) generally were not reported in COVID-19. Both diseases featured fatigue (GCA 38%, COVID-19 43%) and elevated inflammatory markers (C-reactive protein [CRP] elevated in 100% of GCA, 66% of COVID-19), but platelet count was elevated in 47% of GCA but only 4% of COVID-19 cases. Cough and fever were commonly reported in COVID-19 and less frequently in GCA (cough, 63% for COVID-19 vs 12% for GCA; fever, 83% for COVID-19 vs 27% for GCA). Gastrointestinal upset was occasionally reported in COVID-19 (8%), rarely in GCA (4%). Lymphopenia was more common in COVID-19 than GCA (53% in COVID-19, 2% in GCA). Alteration of smell and taste have been described in GCA but their frequency is unclear. CONCLUSION: Overlapping features of GCA and COVID-19 include headache, fever, elevated CRP and cough. Jaw claudication, visual loss, platelet count and lymphocyte count may be more discriminatory. Physicians should be aware of the possibility of diagnostic confusion. We have designed a simple checklist to aid evidence-based evaluation of patients with suspected GCA.