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The estimated incidence of LVI, detected by imaging in a clinical setting, was higher among patients with GCA than the reference population. The aorta was the most commonly affected vascular territory. The negative association with giant cells may suggest particular mechanisms in this subset of GCA.

Background Over the years it has become increasingly clear that vascular involvement in giant cell arteritis (GCA) is widespread, that vasculitic changes may become evident many years after onset of the disease, and that these anomalies may not always be symptomatic. Objectives To investigate the cumulative incidence of large vessel involvement (LVI), defined as aneurysm/ectasia/stenosis of the aorta and its main branches, in patients with biopsy-proven GCA, and to describe the distribution of LVI. Furthermore, to identify predictive factors and to evaluate to what extent the presence of LVI is systematically investigated at the time of diagnosis and during follow-up. Methods Patients with biopsy-proven GCA in the region of Kristianstad, the County of Skåne in southern Sweden, diagnosed between 1997 and 2010, were investigated. Patients were identified using the regional register of the Department of Pathology in Skåne as previously described (1). A retrospective review of case records and histopathology reports of all the identified patients was performed. Results A total of 169 patients were identified, of whom 24 (14.2%) had LVI with the following distribution: aortic ectasia n=17 (ascendens n=10, descendens n=3, abdominal n=4), aortic aneurysm n=10 (ascendens n=3, descendens n=2, abdominal n=5), aortic arch branches (cervical and upper-extremities) n=3, visceral arteries (truncus coeliacus, mesenteric arteries, renal arteries) n=5, lower extremity arteries (iliac, femoral, crural) n=10, intracranial vessels n=1. Two patients had concomitant aortic aneurysm and aortic dissection, two had ectasia at several levels of aorta and four had concomitant aortic aneurysm and ectasia. Fourteen (58%) had isolated aortic involvement and six (25%) had concomitant affection of the aorta and its tributaries. There were no major differences between those with LVI and those without regarding sex (71% vs 76% females) or age at diagnosis (mean 76.5 vs 75.8 years). LVI manifestations were initially noted a median of 3.7 years [interquartile range (IQR) 0.7-7.5] after the diagnosis of GCA. Presence of giant cells in the biopsy was significantly less frequent among patients with LVI (23% vs 52%; p=0.01). There was also a trend towards lower levels of CRP [median 75 (IQR 46-111) vs 90 mg/L (IQR 50-146); p=0.31] and ESR [mean 66 (SD 27) vs 75 mm1h (SD 28); p=0.15] at diagnosis among those with LVI, although this did not reach statistical significance. In general, patients were not systematically investigated regarding the presence of LVI at the time of diagnosis or during the course of the disease. Conclusions LVI of a number of different arteries is seen in GCA. The lower proportion with giant cells in the biopsy and the lower initial inflammatory response suggest that this is a subset of GCA with particular characteristics and disease mechanisms. It is likely that the occurrence of LVI in GCA is severely underrated due to lack of physician awareness of such complications. References Mohammad A et al. Ann Rheum Dis 2014...

Background Giant cell arteritis (GCA) is a systemic vasculitis of the elderly, viewed by many as a disease with multiple and overlapping clinical phenotypes. Retrospective studies have shown differences in clinical presentation between these phenotypes. To reflect the heterogeneity of GCA, and novel diagnostic methods, new classification criteria have been proposed. Methods This is a retrospective study of newly diagnosed patients with GCA at the outpatient rheumatology clinics at Skåne University Hospital (Malmö and Lund) between 2012 and 2018. All patients were evaluated using two sets of classification criteria—the American College of Rheumatology (ACR) classification criteria from 1990, and a proposed revision of these criteria requiring objective findings (positive biopsy or imaging) for classification. Patients were further classified as one of four widely used clinical phenotypes. Results A total of 183 patients with a new diagnosis of GCA were identified. The diagnosis was confirmed by 1–2 experienced rheumatologists in 116 of these patients at review of medical records. The ACR criteria were more sensitive than the revised criteria (93.1% vs 72.4%), while the revised criteria had higher specificity (94.0% vs 28.4%). The revised criteria tended to have higher sensitivity in the phenotype with constitutional symptoms compared to cranial GCA (p = 0.08). Conclusion The specificity of the ACR classification criteria for GCA can be improved by using revised criteria requiring objective findings of vasculitis. In addition, the wider symptoms covered by the revised criteria may improve classification of patients with a phenotype characterized by constitutional symptoms.

Background: Giant cell arteritis (GCA) is a systemic disease with extensive vascular involvement. There is limited and conflicting information on the relation between patient characteristics at diagnosis and future disease phenotypes. We aimed to investigate predictors of time dependent large vessel involvement (LVI) in a population-based cohort of patients with GCA. Methods: GCA patients with positive temporal artery biopsies (TAB) between 1997and 2010 were identified through a regional pathology register. A structured review of histopathology reports and relevant imaging studies was performed. Cases with LVI through July 2016 were identified. Patients were followed to first LVI, death, migration from the area or July 29, 2016. Event free survival by clinical and histopathologic features was estimated using the Kaplan-Meier method. Potential predictors of LVI were examined using Cox regression models.Results: A total of 274 patients were included. The mean age at GCA diagnosis was 75.7 years. Fifty-one patients (19 %) had documented LVI during the follow-up, corresponding to an incidence rate of 2.4/100 person-years. The median time from GCA diagnosis to the diagnosis of LVI was 4.5 years (interquartile range 0.6-7.4). Thirty-four patients had aortic involvement (67% of those with LVI; 12% of all GCA cases). Survival free of LVI was longer in patients with giant cells in the TAB (75th percentile 14.0 vs 6.7 years; p=0.014). In age-adjusted analysis, the presence of giant cells in the TAB was associated with reduced risk of LVI (hazard ratio 0.48; 95 % confidence interval 0.27-0.86). Conclusions: The negative association with giant cells in the TAB suggests that patients with LVI constitute a subset of GCA with particular disease mechanisms. This underlines the heterogeneity of GCA, which should be further explored in prospective studies.

Giant cell arteritis (GCA) is the most common form of primary vasculitis, affecting primarily the large and middle sized arteries in people aged 50 years or older. It is associated with a spectrum of neurological manifestations, the most frequent being neuro-ophtalmic, others are mononeuritis simplex, mononeuritis multiplex, diffuse peripheral polyneuropathy, acute confusional states, spinal cord infarction and TIA/stroke. Radiculopathy/plexopathy is rarely described.This report present a case with lumbosacral radiculopathy, perceived as steroid-induced myopathy. Abstract Case HistoryA 84-year old female with polymyalgia rheumatica (PMR) with disease onset at the age of 62 and with ongoing prednisolone (PRD) 12.5 mg/d, was referred to the rheumatology department in December 2012 due to progressive proximal muscle weakness of the lower extremities over the last 2 years, considered as steroid-induced myopathy.At disease onset September 1991, the patient suffered acute onset of pains in the calf muscles and shortly after also pains in the neck and upper arms, erythrocyte sedimentation rate (ESR) was 90 mm/1 h, no other laboratory measurements was done. PMR was diagnosed by her general practitioner and PRD was instituted with a prompt response. PRD was then tapered during one year to 5 mg/d when she flared leading to escalation to 15-20 mg/d. This pattern was repeated again and again over the years. She managed to become PRD free once for three years before a relapse of PMR symptoms and PRD was re-established. Neither at disease onset nor later did she display any ischemic symptoms.

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