Search

Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65–0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06–1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis. [ABSTRACT FROM AUTHOR]

Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab. A lady in her 80s with past medical history of well-controlled hypertension, bladder cancer in remission, a twenty-pack year smoking history, cervical and lumbar spine stenosis, and recent immunizations presented with acute onset of right-sided pupil-sparing third nerve palsy. Labs were pertinent for an elevated ESR and CRP. Brain imaging was without acute abnormalities. A temporal artery biopsy established evidence of healed arteritis and a diagnosis of GCA was made. The patient was treated with pulse-dose steroids followed by an oral steroid taper and tocilizumab. At one month follow-up, there was partial resolution in her ophthalmoplegia. We underscore the importance of considering temporal arteritis as a potential cause of third nerve palsy in the elderly before attributing it solely to microvascular ischemia, particularly in patients with constitutional features. Additionally, in our comprehensive literature review, we aim to consolidate the existing data from similar presentations, shedding light on the clinical manifestation and disease trajectory. [ABSTRACT FROM AUTHOR]

Introduction: Giant cell arteritis (GCA) presents two major phenotypes – cranial (cGCA) and extracranial (exGCA). exGCA may be overlooked. The study aimed to compare the clinical characteristics between cGCA and exGCA. Methods: Electronic medical records of patients treated between January 2015 and July 2023 at the Department of Rheumatology were searched for the diagnosis of GCA. The clinical characteristics of patients with cGCA, exGCA, and overlapping GCA manifestations were compared. Results: Out of 32 patients with GCA, 20 had cGCA, 7 had exGCA, and 5 had overlap manifestations. The groups did not differ significantly in demographics, clinical signs/symptoms, or laboratory test results. Importantly, the combined group of patients with exGCA and overlap GCA had a statistically significant delay in initiating treatment (median 12 weeks) compared to patients with cGCA (median 4 weeks; p = 0.008). Conclusion: Our study confirmed the insidious nature of exGCA, which lacks distinctive clinical symptoms and consequently leads to delayed treatment. [ABSTRACT FROM AUTHOR]

Systemic vasculitides are among the less common disorders encountered in routine rheumatology practice. The low incidence and heterogeneous presentation at onset can potentially lead to delayed diagnosis. Not recognizing these in the early phase may prove detrimental, as some vasculitis may progress to a catastrophic course with major morbidity or mortality. The causes of diagnostic delay may vary among different types of vasculitis and may also be disease-, patient-, or physician-related. Disease-related factors include the myriad presentations with diverse and non-specific symptoms, mimicking other conditions like infections. In addition, some forms have prolonged prodromal phases before evident organ damage. Limited awareness among healthcare professionals, particularly outside rheumatology, and a lack of readily available diagnostic tools contribute to missed diagnoses. Delays in seeking care due to non-specific symptoms or lack of access to specialist care can worsen outcomes. The economic burden also increases with delayed diagnosis and damage accrual when the disease remains unrecognized or untreated for prolonged periods. Although the causes of vasculitis are numerous, including secondary causes, in this review, we focus on diagnostic delays in primary vasculitides and suggest potential steps to identify and treat these diseases early. These include educating both healthcare professionals and the public about the signs and symptoms of vasculitis; expanding the rheumatology workforce and facilitating timely referrals; implementing readily available and reliable tests for early detection; and streamlining care and diagnostic pathways. Such measures have the potential to improve the overall outcomes of the disease, with prolonged remission, minimal damage accrual, and improved quality of life. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA), more common in Northern European populations, has limited data in Arabcountries. Our study reports GCA's clinical manifestations in Jordan and reviews published research on GCA across Arab nations. In this retrospective analysis, GCA patients diagnosed from January 2007 to March 2019 at a Jordanian academic medical center were included through referrals for temporal artery biopsy (TAB). A comprehensive search in PubMed, Scopus, and the DOAJ (Directory of Open Access Journals) databases was conducted to identify all relevant English-language manuscripts from Arab countries on GCA without time limitations. Among 59 diagnosed GCA patients, 41 (69.5%) were clinically diagnosed with a negative TAB, and 19 (30.5%) had a positive result. Females comprised 74.6% (n = 44) with 1:3 male-female ratio. The mean age at diagnosis was 67.3 (± 9.5) years, with most presenting within two weeks (n = 40, 67.8%). Headache was reported by 54 patients (91.5%). Elevated ESR occurred in 51 patients (78%), with a mean of 81 ± 32.2 mm/hr. All received glucocorticoids for 13.1 ± 10 months. Azathioprine, Methotrexate, and Tocilizumab usage was 15.3% (n = 9), 8.5% (n = 5), and 3.4% (n = 2), respectively. Remission was observed in 57.6% (n=34), and 40.7% (n = 24) had a chronic clinical course on treatment. Males had higher biopsy-based diagnoses (p =.008), and biopsy-diagnosed patients were older (p =.043). The literature search yielded only 20 manuscripts originating in the Arab world. The predominant study types included case reports and retrospective analyses, with only one case series and onecase-control study. [ABSTRACT FROM AUTHOR]

The rates of relapses and therapy discontinuation in patients with giant cell arteritis (GCA) in the modern therapeutic era have not been defined. We aimed to evaluate the glucocorticoid (GC) discontinuation rate and the factors associated with relapses in a contemporary GCA cohort. Patient and treatment data were collected cross-sectionally at first evaluation and 2 years later (second evaluation), in a multicenter, prospective GCA cohort. Predictors of relapses were identified by logistic regression analyses. 243 patients with GCA were initially included (67% women, mean age at diagnosis: 72.1 years, median disease duration: 2 years) while 2 years later complete data for 160 patients were available and analyzed. All patients had received GCs at diagnosis (mean daily prednisolone dose: 40 mg) while during follow-up, 37% received non-biologic and 16% biologic agents, respectively. At second evaluation, 72% of patients were still on therapy (GCs: 58% and/or GC-sparing agents: 29%). Relapses occurred in 27% of patients during follow-up; by multivariable logistic regression analysis, large vessel involvement at diagnosis [odds ratio (OR) = 4.22], a cardiovascular event during follow-up (OR = 4.60) and a higher initial GC daily dose (OR = 1.04), were associated with these relapses. In this large, real-life, contemporary GCA cohort, the rates of GC discontinuation and relapses were 40% and 27%, respectively. Large vessel involvement, a higher GC dose at diagnosis and new cardiovascular events during follow-up were associated with relapses. [ABSTRACT FROM AUTHOR]

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) is a chronic vasculitis of large- and medium-sized vessels. The most frequent symptoms are temporal headaches, scalp tenderness, jaw claudication and polymyalgia rheumatica in 35% of patients. Atypical presentation with dry cough is very rare and could be isolated making the diagnosis difficult. Initial imaging including PET-CT could be helpful. Literature review yielded 13 case reports with available data and one case series which focused on cough and which were all be included in this study. Most of the cases included males (n = 8), with mostly isolated cough or associated to fever and weight loss. Angio-CT of aortic wall was mostly normal, whereas FDG PET-CT showed in all available cases abnormal arterial thoracic uptake. Temporal artery biopsy was almost suggestive of GCA in all available cases. Cough was steroid responsive usually within few days in all cases without any need of combined therapy. Giant cell arteritis is the most common large-vessel vasculitis over the age of 50 in western countries. Isolated dry cough is extremely rare and encountered in less than 5% of cases. [ABSTRACT FROM AUTHOR]

The objective is to investigate whether initial therapy with intravenous methylprednisolone pulses (ivMTP) or oral glucocorticoid (OG) influences the relapse rate in giant cell arteritis (GCA) patients. This is a retrospective observational study of patients with GCA from 2004 to 2021. Demographics, clinical and laboratory variables, cumulative glucocorticoid dose and relapse rate at 6-month follow-up defined according to EULAR recommendations were recorded. Univariate and multivariate logistic regression models were used to determine possible risk factors for relapse. A total of 74 GCA patients were included for analysis (54 (73%) female, mean (SD) age 77.2 (7.4) years). Overall, 47 (63.5%) patients received ivMTP at disease onset and 27 (36.5%) OG. Mean (SD) cumulative prednisone dose (mg) at 6-month follow-up was 3790.7 (1832.7) for patients with ivMTP vs 4298.1 (2930.6) for the OG group, p = 0.37. A total of 15 (20.3%) relapses occurred at 6-month follow-up. Relapse rates did not differ according to the initial therapy (19.1 vs 22.2%, respectively, p = 0.75). In the multivariate analysis, fever at disease onset (OR 4.837; CI 1.1–21.6) and dyslipidemia (OR 5.651; CI 1.1–28.4) were independent predictors for relapse. Initial therapy with ivMTP or OG does not influence the relapse rate of GCA patients. Fever at disease onset and dyslipidemia are independent predictors of disease relapse. [ABSTRACT FROM AUTHOR]

Introduction: Biopsy-proven giant cell arteritis (GCA) occasionally presents without acute-phase reaction. In this setting, GCA may be initially overlooked and glucocorticoid treatment unduly delayed, potentially increasing ischemic risk. Patients and methods: From an inception cohort of patients with newly diagnosed, biopsy-verified GCA, we retrieved all cases without elevation of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level before starting glucocorticoid treatment. We compared the baseline features and outcomes of these patients and two additional patients recruited after GCA diagnosis with those of 42 randomly selected patients with high baseline ESR and CRP. Results: Of 396 patients, 14 (3.5%) had lower baseline values of both ESR and CRP. Lower baseline ESR and CRP were associated with fewer American College of Rheumatology criteria met (p < 0.001, 95% CI − 1.1; − 0.9), and less jaw claudication (p = 0.06, 95% CI 0.8; 44.9), but similar rates of permanent blindness (p = 1.0). Patients with lower ESR and CRP also showed obvious differences regarding mean blood cell counts and mean hemoglobin level, but also less anti-cardiolipin antibody positivity (p = 0.04, 95% CI 0.8; ∞) and hepatic cholestasis (p = 0.03, 95% CI 1.0; 422). Patients with lower ESR and CRP had fewer GCA relapses (p = 0.03, 95% CI − 1.1; − 0.1), fewer glucocorticoid-induced complications (p = 0.01, 95% CI − 2.0; − 0.1), and successfully stopped glucocorticoids sooner than the other patients (18.3 months vs 34 months in average, p = 0.02, 95% CI − 27;− 0.9). Conclusion: Biopsy-proven GCA presenting with lower ESR and CRP is not an exceptional occurrence. It is clinically less typical but carries similar ischemic risk to other forms of the disease. Conversely, the late GCA prognosis of these patients is excellent. [ABSTRACT FROM AUTHOR]

Objective: To investigate whether methotrexate (MTX) use is associated with bone mineral density (BMD) in patients with polymyalgia rheumatica (PMR) and various forms of vasculitis. Methods: Rh-GIOP is a cohort study designed to evaluate bone health in patients with inflammatory rheumatic diseases. This cross-sectional analysis assessed the baseline visits of all patients with PMR or any kind of vasculitis. Following univariable analysis, multivariable linear regression analysis was performed. The lowest T-score of either the lumbar spine or the femur was chosen as the dependent variable to examine the relationship between MTX use and BMD. These analyses were adjusted for a variety of potential confounders, including age, sex, and glucocorticoid (GC) intake. Results: Of 198 patients with PMR or vasculitis, 10 patients were excluded for very high GC dose (n = 6) or short disease duration (n = 4). The remaining 188 patients had the following diseases: PMR 37.2%, giant cell arteritis 25.0%, granulomatosis with polyangiitis 16.5%, followed by rarer diseases. The mean age was 68.0 ± 11.1 years, mean disease duration was 5.58 ± 6.39 years, and 19.7% had osteoporosis by dual x-ray absorptiometry (T-score ≤ −2.5). 23.4% were taking MTX at baseline with a mean dose of 13.2 mg/week (median: 15 mg/week). 38.6% of those used a subcutaneous preparation. MTX users had similar BMD compared to non-users (minimum T-scores −1.70 (± 0.86) versus −1.75 (± 0.91), respectively; p = 0.75). There was no statistically significant dose–response relationship: neither current nor cumulative dose were associated with BMD in unadjusted or adjusted models (current dose: slope −0.02; −0.14 to 0.09; p = 0.69; cumulative dose: slope −0.12; −0.28 to 0.05; p = 0.15). Conclusion: In the Rh-GIOP cohort, MTX is used in about a quarter of patients with PMR or vasculitis. It is not associated with BMD levels. [ABSTRACT FROM AUTHOR]

Glucocorticoid remains the mainstay for treatment of large vessel vasculitis (LVV) including giant cell arteritis (GCA); however, the disease affects the elderly for whom the adverse effects of glucocorticoid are problematic. Recently, some reports have suggested that intravenous tocilizumab (TCZ) monotherapy is effective for this disease. To date, it remains unknown whether subcutaneous TCZ monotherapy is also effective. Here, we present a first case of GCA successfully treated with subcutaneous TCZ monotherapy. A 75-year-old woman presented with shoulder and hip pain. She was diagnosed with polymyalgia rheumatica (PMR) and treated with low-dose prednisolone (15 mg daily); however, she discontinued glucocorticoid therapy at her discretion due to the psychiatric adverse effect (cognitive dysfunction). Seven months later, her shoulder and hip pain relapsed. Furthermore, 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) revealed uptake in the descending thoracic aorta, indicating a complication of LVV. She refused to take glucocorticoid for fear of psychiatric adverse effects and chose subcutaneous TCZ monotherapy (162 mg weekly) for treating this life-threatening urgent condition. Nine months later, her shoulder and hip pain resolved and FDG-PET/CT demonstrated no uptake in the descending thoracic aorta, indicating a successful treatment with subcutaneous TCZ monotherapy for the disease. No adverse events and disease relapse were found during observation period. Our case and the literature review suggest that not only intravenous injection but also subcutaneous injection of TCZ monotherapy can serve as an alternative treatment for patients with GCA who have comorbidities or refuse to take glucocorticoid. [ABSTRACT FROM AUTHOR]

Aortic dilatation (AD) occurs in up to 30% of patients with giant cell arteritis (GCA). Reliable biomarkers for AD development, however, are still absent. The aim of this exploratory study was to evaluate whether immunological parameters are associated with the occurrence of AD in GCA. Cross-sectional study on 20 GCA patients with AD, 20 GCA patients without AD, and 20 non-GCA controls without AD measuring leukocytes, neutrophils, lymphocytes, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum amyloid A (SAA), interferon (IFN)-α, IFN-γ, IFN-γ-induced protein 10 (IP-10), interleukin (IL) 5, IL-8, IL-10, IL-17A, IL-18, IL-1 receptor antagonist, tumor necrosis factor (TNF)-α, platelet-derived growth factor (PDGF), L-selectin, P-selectin, and soluble intercellular adhesion molecule 1 (sICAM-1). AD was measured by aortic contrast-enhanced computed tomography and defined by enlargement of the aorta above population-based aortic diameters adjusted by age, gender, and body surface area. No significant differences were observed between GCA patients with AD and GCA patients without AD concerning levels of leukocytes, neutrophils, lymphocytes, CRP, ESR, SAA, IL-8, IL-18, PDGF, IP-10, selectins, and sICAM-1. Values of IFN-α, IFN-γ, IL-5, IL-10, IL-17A, IL-1 receptor antagonist, and TNF-α were all below the detection limits in more than 70% of subjects. Lymphocytes and CRP revealed positive correlations with the diameter of the thoracic descending aorta. Immunological parameters were not useful to conclude on the presence of AD in GCA. Further studies are required to test if CRP and lymphocytes may be useful to predict future development of AD in GCA. [ABSTRACT FROM AUTHOR]

Although Takayasu arteritis (TAK) is a form of large vessel vasculitis, complications of glomerulonephritis have occasionally been observed, with mesangial proliferative glomerulonephritis as the most common. The aim of this work was to present a case-based review regarding the association of glomerulonephritis and IgA nephropathy (IgAN) with TAK. A literature search was carried out using the PubMed and Scopus databases for articles published in English, and the Ichu-shi Web for Japanese. A 34-year-old Japanese man was evaluated for proteinuria, and IgAN was diagnosed by renal biopsy. Simultaneously, aortic wall thickening and right renal artery stenosis confirmed a coexisting TAK. Prednisolone and methotrexate improved both diseases, and percutaneous transluminal renal angioplasty resulted in right renal artery reopening. Our case and literature review revealed that membranous proliferative glomerulonephritis and IgAN are common in eastern Asia, while focal segmental glomerulosclerosis and mesangial proliferative glomerulonephritis are common in other regions. The incidence of IgAN is higher in TAK cases and is mostly reported in Asia. Abdominal aortic involvement and renal artery stenosis are common in cases with preceding TAK. IgAN could be related to the cytokine network involving interleukin-6, suggesting the usefulness of tocilizumab in patients with TAK accompanied by IgAN. The type of glomerulonephritis complicated with TAK differs among regions, and patients with TAK are more likely to experience IgAN than the healthy population. [ABSTRACT FROM AUTHOR]

Although several studies have explored the geoepidemiology of autoimmune rheumatic diseases (ARD), trends of their frequency overtime are under-investigated. Herein, in a nation-wide study, we examine trends in the prevalence of various ARD over-time, taking also into account the Covid-19 pandemic. In this retrospective study in the entire Greek adult population (approximately 10.000.000 people), we searched the electronic prescription database of the e-Government Centre for Social Security Services using prespecified ICD-10 codes to capture all adult patients with Psoriatic Arthritis (PsA), Rheumatoid Arthritis (RA), Ankylosing Spondylitis (AS), Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc) and Polymyalgia Rheumatica or Giant Cell Arteritis (PMR/GCA). Two sequential 4-year periods, namely 2016–2019 and 2020–2023 were compared. Prevalence of RA, PsA, AxSpA, SLE and SSc increased significantly during 2020–2023 compared to 2016–2019. This applies to both genders and to all age groups for RA, PsA and AxSpA, to female patients in SLE and SSc and to patients 18–39 years in SLE and ≥ 60 years in SSc. Overall, there was 47% increase in prevalence for AxSpA (0.100% in 2016–19 vs 0.147% in 2020–23), 36.5% for PsA (0.148% vs 0.202%), 20.6% for RA (0.467% vs 0.563%), 19% for SLE (0.137% vs 0.163%) and 13% for SSc (0.023% vs 0.026%). A 16.3% decrease was evident in GCA/PMR, limited to those ≥ 40 years old. In a nation-wide study we confirm that ARD prevalence increases over-time, whereas a contribution of Covid-19 pandemic to our results during 2020–2023, cannot be excluded. Additional human, medical and financial resources will be needed to cover the increased needs of ARD patients. [ABSTRACT FROM AUTHOR]

Objective: Giant cell arteritis (GCA) is the most frequent vasculitis affecting adults aged > 50 years. Cardiac involvement in GCA is considered rare, and only a few cases of pericarditis have been reported. The aim of this study was to determine the characteristics and prognosis of GCA patients suffering from pericardial involvement at diagnosis. Methods: We conducted a single-centre, retrospective chart review of patients with GCA in internal medicine departments (from 2000 to 2020). Patients were identified through a centralized hospital database. We retrospectively collected demographic, clinicobiological, histological, imaging, treatment and outcome data. Patients with pericardial effusion, defined as an effusion visible on the CT-scan performed at GCA diagnosis were compared to those without pericardial involvement. Results: Among the 250 patients with GCA, 23 patients (9.2%) had pericardial effusion on CT-scan. The comparison between the groups revealed similar distribution of age, gender, cranial symptoms and ocular ischaemic complications. Patients with pericardial effusion had a higher frequency of weight loss. They also had lower haemoglobin levels and higher platelet levels (p = 0.006 and p = 0.002, respectively), and they more frequently had positive temporal artery biopsy. There were no differences concerning the treatment, relapses, follow-up duration or deaths. Conclusions: This case series sheds light on GCA as a cause of unexplained pericardial effusion or symptomatic pericarditis among adults aged > 50 years and elevated inflammatory biological markers. Fortunately, pericardial involvement is a benign GCA manifestation. In that context, the search for constitutional symptoms, cranial symptoms and associated signs of polymyalgia rheumatica is crucial for rapidly guiding GCA diagnosis. [ABSTRACT FROM AUTHOR]

Prompt initiation of pulse glucocorticoid treatment is recommended in case of visual symptoms and suspected or proven giant cell arteritis (GCA). Pulse treatment in most cases prevents involvement of an initially unaffected fellow eye. We present the case of a biopsy-proven GCA in a 79-year-old man, complicated by sequential bilateral blindness. Initial unilateral vision loss was treated by 1 g methylprednisolone intravenously for 3 days, followed by 1 g/kg prednisone daily. Despite treatment, the second eye went completely blind 11 days after the initial vision loss. We performed a systematic search on Medline and Scopus aiming at identifying all cases of GCA complicated with loss of vision in a previously unaffected eye under pulse treatment for initially monocular vision loss. We identified 11 articles reporting 21 patients that met our inclusion criteria. Contralateral vision loss occurred 1–12 days following treatment initiation, with a median of 2 days. Treatment initiation was delayed up to 8 days since the initial vision loss, with a median delay of 2 days. Anterior ischemic optic neuropathy was the dominant mechanism of vision loss. Sequential involvement of the fellow eye in case of unilateral vision loss in GCA is rare. With 12-day interval being the longest reported, we conclude that even though the first 2 days are the most critical for the visual outcome, blindness in the initially unaffected eye may rarely occur later. Nonetheless, immediate initiation of pulse treatment remains of vital importance to preserve vision in the contralateral eye. [ABSTRACT FROM AUTHOR]

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version. Cranial and large-vessel GCA cases were defined by the presence or absence of cranial signs and/or symptoms, respectively. Annual incidence was calculated by dividing the number of incident cases by the size of the study population ≥ 50 years old. Completeness of case ascertainment was assessed by a three-source capture–recapture analysis. Among the 62 included cases, 42 (68%) were women, mean (± SD) age 77.3 ± 9.1 years. The annual incidence of GCA in northeastern Paris and completeness of case ascertainment were estimated at 7.6 (95% CI 5.9–9.8) per 100,000 inhabitants ≥ 50 years old and 66% (95% CI 52–92%), respectively. Incidence increased with age, peaked at age 80–89 years, and was almost twice as high in women versus men. Large-vessel GCA cases, mean (± SD) age 68.6 ± 11.5 years, accounted for 8% of all GCA cases. In this study, GCA epidemiology was mainly driven by cases with cranial GCA signs or symptoms and incidence results were consistent with recent European and past French studies. [ABSTRACT FROM AUTHOR]

Takayasu arteritis (TA) is a large-cell vasculitis, and is not usually associated with avascular necrosis (AVN). The objective of the study was to investigate any association between TA and AVN, including the possible pathogenic effect of glucocorticoid (GCs) use. The study design was retrospective and cross sectional. TA patients were enrolled in the study. Demographic variables, disease activity, treatments, physician global assessment, Indian Takayasu Clinical activity score 2010, and Kerr criteria were recorded. Logistic regression analysis was performed to identify predictors of AVN. A total of 29 patients were assessed. AVN was observed in four (13.8%) patients with TA. Male gender and elevated C-reactive protein (CRP) were found to be significantly associated with AVN (p = 0.001 and p = 0.006, respectively). While type IIb TA was more common in patients with AVN (n = 2, 50%), type V was more likely in the absence of AVN (n = 13, 52%). Descending aorta and thoracic aorta were usually involved in patients with AVN (both, n = 3, 75%). In multivariate logistic regression, increased CRP levels were the only predictor for AVN (OR = 1.183, 95% Cl = 1.025–1.364, p = 0.021). No association was identified between AVN in TA patients and either duration or cumulative dose of GCs. The present study found that higher CRP levels and male gender were associated with AVN in patients with TA. [ABSTRACT FROM AUTHOR]

Temporal artery biopsy (TAB) is one of the diagnostic tools to confirm the diagnosis of giant cell arteritis (GCA). We aim to evaluate the clinical and technical determinants of a positive biopsy. Demographics, clinical, technical, and laboratory data of all TAB's performed between 2007 and 2019 at a single academic medical center. 107 biopsies performed for 103 patients were included; 72.9% were female, and 27.1% were male. The mean age at the time of biopsy was 67.1 ± 9.3 years. One biopsy was excluded for lack of arterial tissue content. Of the remaining 106, 19.6% were positive. The length of the biopsy and the number of arterial cross-sections were not significantly associated with its result. A positive biopsy was seen more in patients with low albumin (p = 0.010) and hypothyroidism (p = 0.017) but less in those with prior glucocorticoids treatment (p = 0.028). Predictors of a positive biopsy included male gender [OR 4.029, 95% CI (1.330–12.209), p = 0.014]; elevated ESR [OR 3.998, 95% CI (1.908–6.787), p = 0.023]; polymyalgia rheumatica (PMR) symptoms [OR 5.121, 95% CI (2.094–9.872), p = 0.001]; and advancing in age (6.5% per every additional year), [OR 1.065, 95% CI (1.005–1.130), p = 0.033]. 53.7% of the patients were eventually diagnosed with GCA; 39.2% of them were based on positive biopsy. In conclusion, old age, male gender, elevated ESR, and PMR symptoms increase the odds of positive TAB. Technical factors, such as biopsy length and the number of cross-sections, did not influence eventual biopsy results, highlighting the pivotal role of the clinical presentation of the patients in selecting patients for TAB. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitis (AAV) in general involves small blood vessels and includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a ten-year retrospective study of a single-center cohort of AAV, including 101 patients with GPA (n = 58), EGPA (n = 28), MPA (n = 15), and compared the groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6–11.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18–605.2, p < 0.001). All five patients had GPA, whereas no cases with EGPA or MPA were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically in the diagnostic workup of AAV. [ABSTRACT FROM AUTHOR]

Temporal artery biopsy (TAB) is one of the diagnostic criteria of giant cell arteritis (GCA) according to 1990 ACR criteria and remains a tool for diagnosis. Although clinicians perform TAB with an intent to confirm suspected GCA, some biopsies result in negative and some lead to non-GCA diagnoses. We aim to review the diagnoses after TAB biopsy performed for suspected GCA and also wanted to evaluate the diagnostic changes and concomitant diseases that develop over time. The patients who had undergone TAB for suspected GCA were identified using the record entry code for TAB. Patients meeting the classification criteria for GCA were designated as the GCA group and not meeting criteria were designated as a non-GCA group. Other classification criteria were implemented for the non-GCA group diseases. A total of 51 patients (Female: 62.7%, median age: 72.1 ± 7.4 years) who had undergone TAB for suspected GCA were evaluated. TAB was positive in 23 (69.6%) of the 33 patients who met the GCA classification criteria. No significant difference was found between TAB-positive and TAB-negative GCA patients in terms of clinical and laboratory parameters. In the non-GCA group, 12 patients had isolated polymyalgia rheumatica (PMR), and the diagnoses of the remaining six patients were as follows: four large vessel vasculitis (LVV) not satisfying GCA diagnostic criteria, one chronic myelomonocytic leukemia (CMML), and one amyloidosis. TAB was negative in all patients with isolated PMR. TAB showed primary amyloidosis in one patient. Out of 33 GCA patients, 21 had "isolated" GCA, four had GCA + Rheumatoid arthritis (RA), seven had GCA + PMR, and one had GCA + polymyositis. RA was diagnosed antecedent to GCA in two patients, and after GCA in the other two patients. One of the patients had developed GCA 20 years after polymyositis had been diagnosed. TAB was found to be positive in two-thirds of patients with suspected GCA. Late-onset RA and rarely other inflammatory rheumatic diseases may develop in the course of GCA. [ABSTRACT FROM AUTHOR]

The objective of the study is to investigate the clinical characteristics and long-term prognosis including flares and organ damage in patients with giant cell arteritis (GCA) from a tertiary referral centre and compare these features in different subgroups. In this retrospective observational study, patients with GCA who were followed up in our vasculitis clinic between 1998 and 2018 were evaluated by a predefined protocol. Patients with and without cranial symptoms were compared for clinical and laboratory features, flares and permanent damage findings. Vasculitis Damage Index and Large Vessel Vasculitis Index of Damage were used for damage assessment. Records of 89 patients (median follow-up time 46 months) were analysed; mean time to diagnosis after initial symptom was longer in patients with acute vision loss (11 ± 4 vs. 4.8 ± 1.1 months p = 0.002). EGG (n = 19) was younger (63 ± 2 vs. 69 ± 1 years old p = 0.01); had higher mean CRP (141.8 ± 107.3 vs. 76.6 ± 67.9 mg/dL p = 0.023) and ESR (120.8 ± 25.1 vs. 99.3 ± 24.3 mm/h p = 0.004) at diagnosis. PET–CT detected large vessel vasculitis in 42/48 (87.5%) cases of the entire cohort. Thirty-one patients had flares and proportion of flared patients was significantly higher in patients with cranial symptoms. At least one damage item (DI) was present in 54 (60.7%) patients. The development of damage was found to be associated with flares. Evaluation of our cohort revealed the importance of early diagnosis for prevention of vision loss in GCA. Patients without cranial symptoms were younger, present with higher inflammatory response and for these, PET–CT was the main diagnostic tool. Relapse rate was higher in patients with cranial symptoms. Flares and accompanying corticosteroid treatment may contribute to organ damage in GCA. [ABSTRACT FROM AUTHOR]

To describe the clinical characteristics, management, and outcome of a series of patients with giant cell arteritis (GCA) and inflammatory bowel disease (IBD). Patients with both GCA and IBD evaluated between 1/1/1996 and 12/30/2018 were retrospectively identified. Clinical characteristics, laboratory parameters, radiologic features, histopathology, management and outcomes were abstracted. A systematic literature review identifying patients with IBD and GCA was performed via a Medline and EMBASE search from inception through December 31 2019. Six patients were identified with GCA and IBD (66% male). Five (83%) had ulcerative colitis (UC) and one had Crohn's disease (CD). Diagnosis of IBD preceded GCA in four patients with an average interval of 30 years (range 14–42). Average time to IBD diagnosis in those with prior GCA diagnosis was 1.5 years. During mean follow-up of 4.3 years, GCA relapse was infrequent with only one patient with relapse observed. Systematic literature review identified six additional patients with confirmed coexistence of GCA and IBD. Similar to the current series, male sex was more common and ulcerative colitis was the predominant IBD phenotype. The current study reports findings from the largest single-institution case-series of co-existent GCA and IBD. In contrast to Takayasu arteritis with co-existent IBD, which displays a predilection for female sex and Crohn's disease phenotype, both the current study and review of literature demonstrate a stronger association of GCA with male sex and ulcerative colitis. Further studies addressing a potential pathophysiologic connection between GCA and IBD are suggested. [ABSTRACT FROM AUTHOR]

Acute peripheral facial nerve palsy is most frequently idiopathic (Bell's palsy) or virally induced, but can also be due to several other conditions. A rare cause is underlying systemic or autoimmune disease. A 79-year-old man presented with peripheral facial nerve palsy, malaise, and fever. Physical examination revealed tenderness of the left temporal artery and reduced pulsatility. 18F-FDG-PET/CT and biopsy of the temporal artery confirmed the diagnosis of giant cell arteritis (GCA). Prompt institution of corticosteroid therapy produced rapid decrease in inflammatory markers and gradual improvement of the facial nerve palsy. We searched the MEDLINE, Embase, and Scopus databases to identify previous reports of peripheral nerve palsy in GCA, other vasculitides, and autoimmune diseases. Facial nerve palsy as the presenting symptom of GCA has very rarely been reported. Although temporal artery biopsy is the gold standard for diagnosis, it may be negative in up to one-third of cases. In doubtful cases, imaging can help establish the diagnosis. Ultrasound, 3 T MRI, and 18F-FDG-PET/CT have all been previously reported to be useful. Peripheral facial nerve palsy may very rarely be the presenting symptom of GCA. Early correct diagnosis is essential for starting appropriate therapy. In patients with atypical features, 18F-FDG-PET/CT may be useful for establishing the diagnosis. [ABSTRACT FROM AUTHOR]

The long-term mortality in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) is unexpectedly decreased or at least not increased regardless of several mortality risk factors that these diseases share with other chronic immune-mediated rheumatic diseases. The genetic and immunological profile of PMR/GCA patients is unique, therefore, the hypothesis that this profile provides some survival advantage to PMR/GCA patients should be considered. The longevity is a phenomenon that was demonstrated to be familial. The familial aggregation of longevity can be studied by analysis of life expectancy in family members. Here we test the hypothesis of the aggregation of an increased longevity in the families of PMR/GCA patients. We compared the age of death of 358 parents of 179 PMR and GCA patients with corresponding data retrieved from 506 parents of 253 randomly collected age and sex-matched controls. The number of nonagenarian (≥ 90-year -old) mothers of PMR/GCA patients was significantly higher (OR = 2.34, 95%CI 1.11–11.95, p < 0.0005) vs controls. Both nonagenarian parents were found in 6 patients (3.35%) and none in the control cohort (OR = 8.77, 95%CI 2.26–405.10, p = 0.003). Our data suggest the familial aggregation of nonagenarians in PMR/GCA patients. [ABSTRACT FROM AUTHOR]

Objective: To describe the prevalence of depression among patients with primary systemic vasculitides (PSV); compare prevalence according to vasculitis type and against controls; and examine the impact of depression on PSV outcomes. Methods: We searched Medline, PubMed, Scopus and Web of Science using a predefined protocol in accordance with PRISMA guidelines. We included all studies that reported the prevalence or impact of depression in PSV. We also included polymyalgia rheumatica (PMR) given its association with giant cell arteritis (GCA). Meta-analyses of prevalence estimates were performed using random-effects models and reported as percentages (95% confidence interval). Results: We reviewed a total of 15 studies that described the prevalence of depression, categorised into small (n = 10) and large vessel vasculitis (n = 7). Pooled prevalence estimate for depression in a small vessel (predominantly ANCA-associated) vasculitis was 28% (95% CI 20–38%) with significant heterogeneity (I2 = 93%). Depression prevalence in large-vessel vasculitis (Takayasu and GCA/PMR) was 24% (95% CI 17–34%), again with significant heterogeneity (I2 = 96%). One study reported 56% prevalence of depression in medium vessel disease. The prevalence of depression in small vessel vasculitis was higher than healthy controls. In these patients, depression and depressive symptoms were associated with poorer quality of life, adherence, and work disability, but not disease activity or damage. Conclusion: Depression is highly prevalent among patients with primary systemic vasculitis and associated with poorer outcomes across a range of measures in studies of small vessel disease. Further studies are needed for depression in medium and large vessel vasculitides. [ABSTRACT FROM AUTHOR]

Permanent vision loss is one of the most serious complications of giant cell arteritis (GCA) and therefore prompt diagnosis is paramount. However, diagnosis of GCA remains challenging due to its frequently non-specific presentation. Our aim was to identify differences in the characteristics of GCA patients with, and without, current visual symptoms. A cross-sectional survey was mailed to patients with a GCA Read code entered in their GP electronic medical record. Responders were categorised as those currently reporting a visual symptom or not. We compared general and GCA-specific characteristics in these two groups. The association of diagnostic delay with subsequent experience of visual symptoms was examined using unadjusted and adjusted linear regression analysis. 318 GCA patients responded to the survey (59.6%). Responders were predominantly female (69.8%), with a mean age of 73.7 years (SD 8.2). 28% reported current visual symptoms. There was no statistically significant difference in the general characteristics between those with and without visual symptoms. Of GCA-specific characteristics, pre-GCA diagnosis of diplopia (p = 0.018), temporary (p ≤ 0.001) or permanent visual problems (p = 0.001) and hoarseness (p = 0.004) were more common among those reporting current visual symptoms. There was no association between the extent of diagnostic delay and reporting of current visual symptoms. Though we found few characteristics to distinguish between GCA patients with or without current visual symptoms, diagnostic delay was not associated with current visual symptoms. Our findings highlighted the continued difficulty for clinicians to identify GCA patients at the highest risk of visual complications. [ABSTRACT FROM AUTHOR]

Polymyalgia rheumatica (PMR) affects elderly patients and is characterized by pain and stiffness of the shoulder girdle, pelvic girdle and cervical region, which can be associated with the presence of giant cell arteritis. Data on the epidemiology of this disease in Latin America are scarce. In Colombia, the Ministry of Health introduced SISPRO, a tool to collect nation-wide information from the health system. The information collected from SISPRO is available for scientific analysis. Using SISPRO data for the years 2012–2016, an analysis was made on the prevalence and characteristics of patients diagnosed with PMR. This is a descriptive epidemiological study using the International Statistical Classification of Diseases and Related Health Problems as search terms related to PMR, based on SISPRO data. Criteria for diagnosis are not explicitly addressed in each individual case. National records report 19,901 individuals diagnosed with PMR and estimated prevalence of 2 cases per 1000 inhabitants over 50 years old (based on a total population of 47,663,162), being more frequent in women (86% of cases), with a female/male ratio of 6.2:1. This is the first study that describes the demographic characteristics of PMR in Colombia. Our results are consistent with the age-related increase in prevalence and gender ratio. Likewise, there are differences between regions, which may be related to ancestry and environmental factors, which require further studies. [ABSTRACT FROM AUTHOR]

Systemic vasculitis involving the breast is a rare clinical condition and may mimic breast cancer or mastitis clinically or radiographically. Here, we report a case of polyarteritis nodosa (PAN) with breast involvement and perform a literature review of published cases of systemic vasculitis affecting the breast to better understand this disorder. We report a case of PAN affecting the right breast in a young woman. A retrospective review was performed by searching Medline, Embase, Web of Science, the Cochrane Library, and Scopus for cases of systemic vasculitis involving the breast written in English up to June 1st, 2018. A 27-year-old woman presented with a painful mass in the right breast was diagnosed as PAN by the biopsy. She was treated with prednisone and methotrexate for 6 months, at which time her condition had stabilized and inflammatory markers had normalized. A total of 66 cases were identified, with granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), and PAN as the main types. The typical manifestation was mass (79.2%, 53/67) in the breast, and all diagnoses were made by the pathology of the breast biopsy. Glucocorticoid and immunosuppressant were the main therapies, and 74.6% (50/67) patients achieved remission during follow-up. Our case and a literature review of 66 cases of systemic vasculitis involving the breast reveal the importance of tissue biopsy to obtain a definitive diagnosis, because the vasculitis subtype strongly influences prognosis. [ABSTRACT FROM AUTHOR]

Tocilizumab (TCZ) may rarely cause hematological side effects including neutropenia and thrombocytopenia. TCZ is essentially expected to lower the fibrinogen levels to stay within the normal range, but TCZ-induced hypofibrinogenemia has been rarely reported in literature. Although it may remain asymptomatic, hypofibrinogenemia has clinical significance owing to the tendency of the condition to result in bleeding. A 65-year-old female patient with known polymyositis was, approximately 20 years after the diagnosis was made, examined due to elevated acute phase reactants leading to the diagnosis of giant cell arteritis (GCA) and TCZ treatment was initiated as she had former steroid-induced osteoporotic fractures. 1 month after the initial dose of intravenous (IV) TCZ, she presented with ecchymosis and was detected to have hypofibrinogenemia. Following the administration of the second dose, hypofibrinogenemia was detected again. In this review, we have analyzed this patient in addition to the cases in six other articles of TCZ induced hypofibrinogenemia which we found out based on our search strategy. Our aim is to point out a rare side effect of TCZ, hypofibrinogenemia, thus to emphasize a possible bleeding disorder and discuss probable underlying mechanisms. [ABSTRACT FROM AUTHOR]

There are no universally accepted diagnostic criteria for large-vessel vasculitides (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK). Currently, available classification criteria cannot be used for the diagnosis of GCA and TAK. Early diagnosis of these two diseases is quite challenging in clinical practice and may be accomplished only by combining the patient symptoms, physical examination findings, blood test results, imaging findings, and biopsy results, if available. Awareness of red flags which lead the clinician to investigate TAK in a young patient with persistent systemic inflammation is helpful for the early diagnosis. It should be noted that clinical presentation may be highly variable in a subgroup of GCA patients with predominant large-vessel involvement (LVI) and without prominent cranial symptoms. Imaging modalities are especially helpful for the diagnosis of this subgroup. Differential diagnosis between older patients with TAK and this subgroup of GCA patients presenting with LVI may be difficult. Various pathologies may mimic LVV either by causing systemic inflammation and constitutional symptoms, or by causing lumen narrowing with or without aneurysm formation in the aorta and its branches. Differential diagnosis of aortitis is crucial. Infectious aortitis including mycotic aneurysms due to septicemia or endocarditis, as well as causes such as syphilis and mycobacterial infections should always be excluded. On the other hand, the presence of non-infectious aortitis is not unique for TAK and GCA. It should be noted that aortitis, other large-vessel involvement or both, may occasionally be seen in various other autoimmune pathologies including ANCA-positive vasculitides, Behçet's disease, ankylosing spondylitis, sarcoidosis, and Sjögren's syndrome. Besides, aortitis may be idiopathic and isolated. Atherosclerosis should always be considered in the differential diagnosis of LVV. Other pathologies which may mimic LVV include, but not limited to, congenital causes of aortic coarctation and middle aortic syndrome, immunoglobulin G4-related disease, and hereditary disorders of connective tissue such as Marfan syndrome and Ehler-Danlos syndrome. [ABSTRACT FROM AUTHOR]

The objective of this study was to evaluate glucocorticoid (GC) use in patients with polymyalgia rheumatica (PMR), giant cell arteritis (GCA) or both diseases (PMR + GCA) under rheumatological care. Data from patients with PMR (n = 1420), GCA (n = 177) or PMR + GCA (n = 261) from the National Database of the German Collaborative Arthritis Centers were analyzed regarding GCs and related comorbidities (osteoporosis, diabetes and cardiovascular disease), stratified by disease duration (DD). Longitudinal data were analyzed for all patients with a DD ≤ 2 years at database entry (n = 1397). Three-year data were available for 256 patients. Predictors of GC use ≥ 3 years were examined by logistic regression analyses. A total of 76% received GCs, and 19% (PMR) to 40% (GCA) received methotrexate. Median GC doses were 12.5 mg (PMR), 11.3 mg (GCA), and 20.0 mg/day (PMR + GCA) in a 0-6-month DD. Median GC doses ≤ 5 mg/day were reached at a 13-18-month DD in PMR patients and at a 19-24-month DD in GCA or PMR + GCA patients. In the multivariate analysis, baseline methotrexate (OR 2.03, [95% CI 1.27-3.24]), GCs > 10 mg/day (OR 1.65, [1.07-2.55]), higher disease activity (OR 1.12, [1.02-1.23]) (median 0.6 years DD), and female sex (OR 1.63 [1.09-2.43]) were predictive for GC therapy at ≥ 3 years. Of the examined comorbidities, only osteoporosis prevalence increased within 3 years. GC use for ≥ 3 years was reported in one-fourth of all the patients. A difficult-to-control disease activity within the first year was a good predictor of long-term GC need. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) has been previously associated with cranial mononeuritis (usually optic neuritis). We hereby describe a 68-year-old man who presented due to fever and diplopia of acute onset. Physical examination revealed left abducens nerve palsy and a hearing defect in the right ear. Brain imaging and cerebrospinal fluid analysis were not diagnostic. GCA was suspected, and treatment with high-dose methylprednisolone was initiated, leading to marked improvement. Temporal artery biopsy confirmed the presence of GCA. While considering corticosteroid tapering, the patient experienced hoarseness due to right laryngeal nerve palsy. Addition of cyclophosphamide to the treatment resulted in full response. GCA mainly affects large vessels, but one or more cranial nerve palsies may also occur. Following a review of the literature, this is the first report of three cranial nerve palsies in the setting of histologically proven GCA. The role of cyclophosphamide in this entity is also discussed. [ABSTRACT FROM AUTHOR]

Spinal cord infarction is extremely rare in patients with giant cell arteritis (GCA). There are only four case reports in the literature. We describe a 65-year-old man who presented with sudden paraplegia and back pain of 4-days duration with sensory loss below the umbilicus and bilateral scalp necrosis. Magnetic resonance imaging finding was consistent with dorsal spinal cord infarction. Biopsy of the temporal artery confirmed the diagnosis of GCA. The patient was treated with high dose of corticosteroids, which resulted in healing of the scalp ulcerations in 3 weeks, but the paraplegia was irreversible. To our knowledge, this is the first report of spinal cord infarction and simultaneous occurrence of bilateral scalp necrosis in a histopathologically proven GCA. Although literature about spinal cord involvement in GCA is very limited, cord infarction is associated with high mortality and therapeutic challenges since little is understood regarding the pathogenesis that leads to infarction. [ABSTRACT FROM AUTHOR]

The article discusses the Temporal Arteritis Proforma which documents symptoms and signs of Temporal Arteritis (TA). Topics discussed include the Neuro-Ophthalmology Society of Australia's meeting held in Auckland, New Zealand in September 2015; reference of an article "Dry cough is a frequent manifestation of giant cell arteritis" by T. Zenone and others and effect of smoking status and respiratory comorbidities.

Pericardial effusion in patients with giant cell arteritis has been described in case reports. The exact prevalence of this manifestation is unknown. The objective of this study was to determine the prevalence and characteristics of pericardial effusion in patients with giant cell arteritis. Retrospective data were collected from 114 patients at a single hospital with giant cell arteritis diagnosed between October 1999 and July 2013. Pericardial effusion was found in four patients (3.5 %, 95 % confidence intervals 1-8.7). In three cases, pericardial effusion was present at initial presentation and was asymptomatic. In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced. Prognosis was good in all cases. The present study confirms that pericardial effusion could occur in patients with giant cell arteritis. However, pericardial effusion is a rare manifestation of giant cell arteritis. Clinical course of pericardial effusion in the setting of giant cell arteritis is usually benign and rarely symptomatic. Recognition of this manifestation may contribute to early diagnosis of the disease. [ABSTRACT FROM AUTHOR]