Giant cell arteritis (GCA), also called Horton or granulomatous arteritis, is a largeand medium-sized blood vessel systemic vasculitis characterized by the granulomatous involvement of the aorta and its major branches (1,2). Polymyalgia rheumatica (PMR) is a disease characterized by severe bilateral pain and aching involving the neck, shoulder, and pelvic girdles associated with morning stiffness (1). GCA and PMR mainly occur in white individuals age 50 years. Due to the progressive aging of the population in Western countries, both conditions have emerged as relatively common diseases in the elderly (1,2).

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.24459

Epidemiology of giant cell arteritis and polymyalgia rheumatica.

Background— In giant cell arteritis (GCA), vasculitic damage of the aorta and its branches is combined with a syndrome of intense systemic inflammation. Therapeutically, glucocorticoids remain the gold standard because they promptly and effectively suppress acute manifestations; however, they fail to eradicate vessel wall infiltrates. The effects of glucocorticoids on the systemic and vascular components of GCA are not understood. Methods and Results— The immunoprofile of untreated and glucocorticoid-treated GCA was examined in peripheral blood and temporal artery biopsies with protein quantification assays, flow cytometry, quantitative real-time polymerase chain reaction, and immunohistochemistry. Plasma interferon-γ and interleukin (IL)-17 and frequencies of interferon-γ-producing and IL-17-producing T cells were markedly elevated before therapy. Glucocorticoid treatment suppressed the Th17 but not the Th1 arm in the blood and the vascular lesions. Analysis of monocytes/macrophages in the circulation an...

Th17 and Th1 T-Cell Responses in Giant Cell Arteritis

Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment.

Takayasu arteritis and giant cell arteritis: a spectrum within the same disease?

Thoracic aortic aneurysm (TAA) is the life-threatening complication of Marfan syndrome (MFS), a connective tissue disorder caused by mutations in the fibrillin-1 gene. TAA is characterized by degradation of elastic fiber, suggesting the involvement of matrix metalloproteinase (MMP)-2 and -9, the activation of which is regulated by TIMP (tissue inhibitor of MMP) types 1 and 2. We hypothesized that MMP-2 and -9 were upregulated during TAA formation in Marfan syndrome, causing loss of elastic fibers and structural integrity. We studied mice, from 3 to 12 months, heterozygous for a mutant Fbn1 allele encoding a cysteine substitution in fibrillin-1 (Fbn1(C1039G/+), designated as "Marfan" mice) (n=120), the most common class of mutation in Marfan syndrome. The littermates, Fbn1(+/+) served as controls (n=120). In Marfan aneurysmal thoracic aorta, mRNA and protein expression of MMP-2 and -9 were detected at 3 months and peaked at 6 months of age, accompanied by severe elastic fiber fragmentation and degradation. From 3 to 9 months, the MMP-2/TIMP-2 ratio increased by 43% to 63% compared with the controls. Dilated thoracic aorta demonstrated increased elasticity but distention caused a pronounced loss of contraction, suggesting weakening of the aortic wall. Breaking stress of the aneurysmal aorta was 70% of the controls. Contraction in response to depolarization and receptor stimulation decreased in the aneurysmal thoracic aorta by 50% to 80%, but the expression of alpha-smooth muscle actin between the 2 strains was not significantly different. This report demonstrates the upregulation of MMP-2 and -9 during TAA formation in Marfan syndrome. The resulting elastic fiber degeneration with deterioration of the aortic contraction and mechanical properties may explain the pathogenesis of TAA.

Loss of Elastic Fiber Integrity and Reduction of Vascular Smooth Muscle Contraction Resulting From the Upregulated Activities of Matrix Metalloproteinase-2 and -9 in the Thoracic Aortic Aneurysm in Marfan Syndrome

Background— Both matrix metalloproteinase-2 (MMP-2) and -9 (MMP-9) have been postulated to play roles in the pathophysiology of giant cell arteritis (GCA) because of their ability to degrade elasti

Metalloproteinase-2 and -9 in Giant Cell Arteritis Involvement in Vascular Remodeling

No detection of parvovirus B19 or herpesvirus DNA in giant cell arteritis

Objective: The percentage of negative temporal artery biopsies (TABs) remains very high in patients with suspected giant cell arteritis (GCA). The aim of our study was to identify the clinical predictors of TAB results to improve the effectiveness of this diagnostic procedure.Methods: We performed a cross‐sectional study of 125 consecutive patients who underwent TAB in the Department of Internal Medicine of a public tertiary hospital, from January 1997 to March 2002. We compared patients with a positive and a negative biopsy result.Results: Forty‐six of the 125 biopsies (36.8%) were positive for GCA and 79 (63.2%) negative. Temporal artery (TA) thickening (8.01), decreased pulse (5.58), jaw claudication (4.87), and scalp tenderness (4.29) presented the highest positive likelihood ratios (LR+). Erythrocyte sedimentation rate (ESR) (0.11), headache (0.39), TA thickening (0.42), and decreased pulse (0.48) had the lowest negative likelihood ratios (LR−). Only TA abnormalities [odds ratio (OR) 12.3] and scalp ...

Pere Huguet-Redecilla

Papers

Metalloproteinase-2 and -9 in Giant Cell Arteritis Involvement in Vascular Remodeling

No detection of parvovirus B19 or herpesvirus DNA in giant cell arteritis

Proposal to decrease the number of negative temporal artery biopsies