Long-term survival of patients with giant cell arteritis in the american college of rheumatology giant cell arteritis classification criteria cohort

1996 ◽  
Vol 100 (2) ◽  
pp. 193-196 ◽  
Author(s):  
Eric L. Matteson ◽  
Kenneth N. Gold ◽  
Daniel A. Bloch ◽  
Gene G. Hunder
2004 ◽  
Vol 101 (2) ◽  
pp. 219-226 ◽  
Author(s):  
Naoki Shinojima ◽  
Masato Kochi ◽  
Jun-Ichiro Hamada ◽  
Hideo Nakamura ◽  
Shigetoshi Yano ◽  
...  

Object. Glioblastoma multiforme (GBM) remains incurable by conventional treatments, although some patients experience long-term survival. A younger age, a higher Karnofsky Performance Scale (KPS) score, more aggressive treatment, and long progression-free intervals have been reported to be positively associated with long-term postoperative patient survival. The aim of this retrospective study was the identification of additional favorable prognostic factors affecting long-term survival in surgically treated adult patients with supratentorial GBM. Methods. Of 113 adult patients newly diagnosed with histologically verified supratentorial GBM who were enrolled in Phase III trials during the period between 1987 and 1998, six (5.3%) who survived for longer than 5 years were defined as long-term survivors, whereas the remaining 107 patients served as controls. All six were women and were compared with the controls; they were younger (mean age 44.2 years, range 31–60 years), and their preoperative KPS scores were higher (mean 85, range 60–100). Four of the six patients underwent gross-total resection. In five patients (83.3%) the progression-free interval was longer than 5 years and in three a histopathological diagnosis of giant cell GBM was made. This diagnosis was not made in the other 107 patients. Conclusions. Among adult patients with supratentorial GBM, female sex and histopathological characteristics consistent with giant cell GBM may be predictive of a better survival rate, as may traditional factors (that is, younger age, good KPS score, more aggressive resection, and a long progression-free interval).


2009 ◽  
Vol 2 (2) ◽  
pp. 103-110 ◽  
Author(s):  
E. Naydenov ◽  
V. Bussarsky ◽  
S. Nachev ◽  
S. Hadjidekova ◽  
D. Toncheva

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1212.1-1212
Author(s):  
V. Yang ◽  
C. Mcmaster ◽  
C. Owen ◽  
J. Leung ◽  
R. Buchanan ◽  
...  

Background:Giant cell arteritis (GCA), if left untreated, confers the threat of serious cranial ischaemic complications including permanent visual loss. Although achieving a prompt and accurate diagnosis remains challenging, early diagnosis is viewed as being paramount in preventing significant morbidity.1 This raises the question of whether GCA patients are at greater risk of developing visual sequelae if there is a longer window between symptom onset and presentation.Objectives:To compare the frequency of lasting visual loss in patients diagnosed with GCA undergoing temporal artery biopsy (TAB) within three months and after three months of symptom onset.Methods:Patients who underwent TAB from January 2011 to November 2020 were identified from the pathology database of an Australian rheumatology referral centre. The diagnosis of GCA was established for each patient based on either positive TAB or, in the setting of negative TAB, clinical diagnosis by a rheumatologist. Baseline demographics, symptoms and major confounders – including age, sex, history of polymyalgia rheumatica or inflammatory arthritis, headache, jaw pain, fatigue, temporal artery tenderness or diminished pulse, and number of 1990 American College of Rheumatology (ACR) classification criteria for GCA2 fulfilled – were manually extracted from electronic medical records, as was the duration between onset of GCA symptoms and TAB, and the presence of visual loss before and after TAB. Logistic regression log-likelihood tests were used to examine the two cohorts presenting before and after three months.Results:There were 167 patients who underwent TAB during the study period with accessible clinical information. Of these, 31 (19%) had a delayed presentation of greater than three months from symptom onset. There were no statistical differences in patient demographics between the two groups (Table 1). No patients with delayed presentation experienced lasting, objective visual loss. In contrast, there were three cases in the cohort of patients who presented more promptly; these included two patients who developed permanent unilateral blindness, and one who experienced unilateral vision loss with some improvement at three months of follow-up.Table 1.Patient characteristics by time from symptom onset to TAB.Presentation <3 monthsPresentation ≥3 monthsp-valueAge (years)73.45±10.0669.84±10.750.080Female92 (67.65%)20 (64.52%)0.738History of polymyalgia rheumatica23 (16.91%)4 (12.90%)0.586History of inflammatory arthritis6 (4.41%)2 (6.45%)0.633Headache110 (80.88%)23 (74.19%)0.406Jaw pain37 (27.21%)5 (16.13%)0.206Fatigue28 (20.59%)6 (19.35%)0.878Temporal artery tenderness or diminished pulse46 (33.82%)11 (35.48%)0.860ACR classification criteria2.83±0.992.58±0.890.199Conclusion:GCA patients with a lengthier course of symptoms before diagnosis did not experience any enduring visual loss. This may reflect a pattern of more aggressive disease leading to earlier presentation, but further study should explore whether longer symptom duration before diagnosis necessitates a higher degree of clinical concern.References:[1]Font C, Cid MC, Coll-Vinent B, López-Soto A, Grau JM. Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis. Br J Rheumatol. 1997 Feb;36(2):251-4. doi: 10.1093/rheumatology/36.2.251. PMID: 9133940.[2]Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990 Aug;33(8):1122-8. doi: 10.1002/art.1780330810. PMID: 2202311.Disclosure of Interests:None declared


2015 ◽  
Vol 52 (4) ◽  
pp. 704 ◽  
Author(s):  
A Singh ◽  
V Mallya ◽  
F Siraj ◽  
KC Sharma

2021 ◽  
pp. jrheum.200952
Author(s):  
Jérémy Clément ◽  
Pierre Duffau ◽  
Joel Constans ◽  
Thierry Schaeverbeke ◽  
Jean-Francois Viallard ◽  
...  

Objective Tocilizumab (TCZ), an IL-6 receptor antagonist, is approved for giant cell arteritis (GCA) as a cortisone-sparing strategy and in refractory patients. This study assessed the real-world efficacy, safety, and long-term outcomes of GCA patients treated with TCZ. Methods We conducted a multicenter retrospective observational study at three French centers. All patients ≥ 50 years, meeting the American College of Rheumatology (ACR) criteria, and had received at least one dose of TCZ were included. Relapse was defined by therapeutic escalation, such as increased doses of CS, resumption of CS after weaning, or introduction or intensification of adjuvant therapy. Results Between 2013 and 2019, 43 patients were included. Patients were followed-up in median 511 days between GCA diagnosis and inclusion with 34/43 (72%) patients experiencing relapses. At inclusion, median age was 77 years and median dose of corticosteroid (CS) was 15 mg/day. After inclusion, the mean cumulative dose of CS was 2.1g/year versus 9.4g/year before inclusion (p<2.107) with 12/43 (28%) patients experiencing relapses on TCZ. Among 29 patients undergoing TCZ discontinuation, 18 (62%) experienced relapse. Factors associated with relapse after inclusion were introduction of TCZ > 6 months after diagnosis (p=0,005), absence of ischemic signs at diagnosis (p=0,006), relapse rate >0.8/year (p=0.03) and absence of CS tapering ≤ 5 mg/day (p=0,03) before inclusion. Serious adverse events occurred in 18/43 patients (42%), including four deaths. Conclusion Our results confirm the effectiveness of TCZ for CS-sparing, but after discontinuation of treatment, TCZ allows for a prolonged remission in less than 50% of patients. Attention must be paid to the tolerance of this long-term treatment in this elderly and heavily treated population.


Rheumatology ◽  
2021 ◽  
Author(s):  
Frans Wiberg ◽  
Nazanin Naderi ◽  
Aladdin J Mohammad ◽  
Carl Turesson

Abstract 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.


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