arteritis and TRAPS

Giant cell arteritis (GCA) is a prototypic autoimmune disease with a highly selective tissue tropism for medium and large arteries. Extravascular GCA manifests with intense systemic inflammation and polymyalgia rheumatica; vascular GCA results in vessel wall damage and stenosis, causing tissue ischemia. Typical granulomatous infiltrates in affected arteries are composed of CD4+ T cells and hyperactivated macrophages, signifying the involvement of the innate and adaptive immune system. Lesional CD4+ T cells undergo antigen-dependent clonal expansion, but antigen-nonspecific pathways ultimately control the intensity and duration of pathogenic immunity...

Giant cell arteritis (GCA) is an autoimmune disease affecting large vessels in patients over 50 years old. It is an exemplary model of a classic inflammatory disorder with IL-6 playing the leading role. The main comorbidities that may appear acutely or chronically are vascular occlusion leading to blindness and thoracic aorta aneurysm formation, respectively. The tissue inflammatory bulk is expressed as acute or chronic delayed-type hypersensitivity reactions, the latter being apparent by giant cell formation...

In steroid-responsive meningitis-arteritis (SRMA), inflammatory dysregulation is driven by neutrophilic granulocytes resulting in purulent leptomeningitis. Neutrophils can generate neutrophil extracellular traps (NET). Uncontrolled NET-formation or impaired NET-clearance evidently cause tissue and organ damage resulting in immune-mediated diseases. The aim of the study was to verify that NET-formation is detectable in ex vivo samples of acute diseased dogs with SRMA by visualizing and measuring NET-markers in serum and cerebrospinal fluid (CSF) samples...

Takayasu's arteritis, first described by Dr. Mikito Takayasu in 1908, is a systemic vasculitis that mostly affects the aorta and its major branches. Although the etiology of the disease is yet unknown, genetic and environmental factors may both play a role. One hundred years after the discovery of Takayasu's arteritis, inflammation is finally widely recognized as a fundamental condition common to all vascular diseases, and clinical trials have proven the efficacy of molecularly targeted drugs that block each step of the NLRP3 inflammasome/interleukin (IL)-1β/IL-6 cascade in patients with atherosclerotic vascular disease and elevated C-reactive protein (CRP)...

OBJECTIVE: Neutrophils are important in host defense. However, neutrophils are also linked to inflammation and organ damage. The purpose of this study was to assess whether markers of neutrophil activation are increased in polymyalgia rheumatica. METHODS: Levels of immune complexes (IC), calprotectin, and neutrophil extracellular traps (NETs) were measured in plasma of healthy individuals (n = 30) and patients with PMR (n = 60), at flare and upon treatment with glucocorticoids using ELISA...

The term "vasculitis" refers to a group of rare immune-mediated diseases characterized by the dysregulated immune system attacking blood vessels located in any organ of the body, including the skin, lungs, and kidneys. Vasculitides are classified according to the size of the vessel that is affected. Although this observation is not specific to small-, medium-, or large-vessel vasculitides, patients show a high circulating neutrophil-to-lymphocyte ratio, suggesting the direct or indirect involvement of neutrophils in these diseases...

Extracellular traps (ETs) are DNA networks formed by immune cells to fight infectious diseases by catching and attacking pathogenic microorganisms. Uncontrolled ET formation or impaired ET clearance can cause tissue and organ damage. Steroid-responsive meningitis-arteritis (SRMA) represents an immune-mediated, presumably non-infectious, purulent leptomeningitis and fibrinoid-necrotizing arteritis and periarteritis of young-adult dogs. Chronic and recurrent cases of SRMA are characterized by lymphohistiocytic inflammatory cell infiltration in the meninges and perivascular tissue...

Recent studies have confirmed the role of plasma donor-derived cell-free DNA (ddcfDNA) as a reliable non-invasive biomarker for allograft injury after kidney transplantation. Whereas the variability of plasma ddcfDNA levels among recipients has limited their clinical use. This study aimed to explore the intrinsic factors associated with plasma ddcfDNA elevation by investigating the impact of Banff lesions and inflammatory infiltrates on ddcfDNA levels in kidney transplant recipients. From March 2017 to September 2019, a total of 106 kidney transplant recipients with matched allograft biopsies were included, consisting of 13 recipients with normal/nonspecific changes, 13 recipients with borderline changes, 60 with T cell-mediated rejection, and 20 with antibody-mediated rejection...

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OBJECTIVES: To explore the presence of neutrophil extracellular traps (NETs) in inflamed temporal artery biopsies (TABs) of patients with giant cell arteritis (GCA). METHODS: Ten patients with GCA [5 with limited and 5 with associated generalized vascular involvement, as defined by 18F-fluorodeoxyglucose (FDG) positron-emission tomography with computed tomography (PET/CT)] and 8 with polymyalgia rheumatica (PMR) were studied. The presence, location, quantitation, and decoration of NETs with IL-6, IL-1β, and IL-17A were assessed in TABs at the time of disease diagnosis by tissue immunofluorescence and confocal microscopy...

Selenium (Se) deficiency and poor plasma Se levels can cause cardiovascular diseases by decreasing selenoprotein levels. Neutrophil extracellular traps (NETs) may be the vicious cycle center of inflammation in vasculitis. Here, we show that Se deficiency induced arteritis mainly by reducing selenoprotein S (SelS), and promoted the progression of arteritis by regulating the recruitment of neutrophils and NET formation. Silencing SelS induced chicken arterial endothelial cells (PAECs) to secrete cytokines, and activated neutrophils to promote NET formation...

The aorta and the large conductive arteries are immunoprivileged tissues and are protected against inflammatory attack. A breakdown of immunoprivilege leads to autoimmune vasculitis, such as giant cell arteritis, in which CD8+ Treg cells fail to contain CD4+ T cells and macrophages, resulting in the formation of tissue-destructive granulomatous lesions. Here, we report that the molecular defect of malfunctioning CD8+ Treg cells lies in aberrant NOTCH4 signaling that deviates endosomal trafficking and minimizes exosome production...

The importance of disease-mediated invasions and the role of parasite spillover as a substantial threat to the conservation of global biodiversity are now well known. Although competition between invasive sliders Trachemys scripta elegans and indigenous European turtles has been extensively studied, the impact of this invasive species on diseases affecting native populations is poorly known. During winter 2012-2013 an unusual event was detected in a population of Emys orbicularis (Linnaeus, 1758) inhabiting a pond system in Galicia (NW Spain)...

OBJECT: Tumor necrosis factor (TNF)-α inhibitors are effective at treating certain inflammatory and autoimmune disorders. They are generally safe; potential adverse events include infections (bacterial, fungal, and viral), congestive heart failure exacerbations, and the potential for demyelinating diseases and possibly certain malignancies. We present the first documented case of fungal internal carotid artery (ICA) mycotic aneurysm in a patient being treated with a TNF-α inhibitor...

INTRODUCTION: Meningiomas are benign primary meningeal tumors. Their diagnosis may be incidental or in response to a work-up for neurological or ophthalmological symptoms. PATIENTS AND METHODS: The clinical course of five patients with ophthalmological symptoms leading to the diagnosis of meningioma is described. RESULTS: The case reports consist of five women (48 to 54 years old - mean 52 years at the onset of symptoms), all suffering from a progressive unilateral decrease in visual acuity with a normal initial fundus examination and ipsilateral visual field changes...

Sometimes, acute aortic dissection diagnosis, a significant cardiological and surgical emergency, is particularly difficult. Therefore, Takayasu arteritis associated dissection fold may be a real diagnostic "trap", in spite of all modern exploratory techniques.

BACKGROUND: Dissecting aneurysm of the posterior inferior cerebellar artery (PICA) uninvolved with the vertebral artery is rare. The exact pathohistological diagnosis might result in 'unknown' because the underlying pathoanatomical features are, for a variety of reasons, not always identified. CASE DESCRIPTION: We report herein two cases of dissecting aneurysm harbored in different segments of the distal posterior inferior cerebellar artery. In our cases, after trapping the PICA at both just proximal and distal to the aneurysm, the abnormal portion was successfully resected with/without an end-to-end anastomosis...

We describe here successful surgical treatment of a rare case of a rapidly enlarged aneurysm due to Takayasu's arteritis. A 22-year-old woman presented with a 1-month history of hoarseness and left neck pain. Contrast-enhanced computerized tomography showed an unruptured partially thrombosed aneurysm, and angiography revealed a large aneurysm of the common carotid artery. After hospitalization, the symptoms, interestingly, resolved spontaneously. While angiography at 2 weeks after admission showed the aneurysm to have reduced in size, 2 months later it showed the left common carotid artery aneurysm to be substantially enlarged in size and aneurysmal formation of the right external carotid artery...

Immature dendritic cells (DCs) are scattered throughout peripheral tissues and act as sentinels that sample the antigenic environment. After activation, they modify their chemokine receptor profile and migrate toward lymphoid tissues. On arrival, they have matured into chemokine-producing DCs that express co-stimulatory molecules and can prime naive T cells. Normal temporal arteries contain immature DCs that are located at the media-adventitia border. In temporal arteries affected by giant cell arteritis, DCs are highly enriched and activated and have matured into fully differentiated cells producing the chemokines, CCL18, CCL19, and CCL21...

INTRODUCTION: The rheumatic disorders and vasculites are inflammatory processes affecting connective tissue in different organs. The inflamed cells liberate destructive enzymes which harm tissues, producing IgE, IgM, IgG antibodies and autoimmune complexes. OBJECTIVE: To review the current classification of these conditions and describe the commonest neurological complications in children. Infantile systemic lupus erythematosus is the commonest; it affects the dermis, joints, blood vessels, heart, kidney and brain...