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Large vessel vasculitis

Si Chen, Xiaoting Wen, Jing Li, Yuan Li, Liubing Li, Xinping Tian, Hui Yuan, Fengchun Zhang, Yongzhe Li
Takayasu arteritis (TA) is a chronic large-vessel vasculitis of unclear pathogenesis. A recent genome-wide association study (GWAS) has revealed that the FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX genes confer susceptibility to TA. We investigated the linkage between presumptive TA-related genes (FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX) and TA in the Han Chinese population. We performed a large case-control multi-center study of 412 Han Chinese TA patients and 597 ethnically matched healthy controls...
October 18, 2016: Oncotarget
Richard F Spaide
PURPOSE: To investigate the structural optical coherence tomography and related microvascular flow characteristics in eyes with retinal vasculitis. METHODS: Regions involved with perivascular infiltration in eyes with retinal vasculitis, but no evidence of large vessel occlusion were evaluated with optical coherence tomography (OCT), OCT angiography, and fluorescein angiography. RESULTS: Ten eyes of 5 patients with a variety of etiologies of retinal vasculitis were evaluated...
October 18, 2016: Retina
Hagit Peleg, Eldad Ben-Chetrit
PURPOSE OF REVIEW: This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. RECENT FINDINGS: Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of the innate immune system. Despite the common occurrence of rashes and other skin lesions in these diseases, vasculitis is reported in only a few. On the other hand, neutrophilic dermatoses are more prevalent...
October 13, 2016: Current Opinion in Rheumatology
Sergio Prieto-González, Georgina Espígol-Frigolé, Ana García-Martínez, Marco A Alba, Itziar Tavera-Bahillo, José Hernández-Rodríguez, Arturo Renú, Rosa Gilabert, Francisco Lomeña, Maria C Cid
Various imaging modalities, including color duplex ultrasonography, CT angiography, magnetic resonance angiography, and PET, are emerging as important aids to the diagnosis, staging, evaluation of disease activity and response to treatment in systemic vasculitis. Although large-vessel vasculitis is the main target of imaging, refinement and increasing accuracy of imaging modalities are also providing useful information in the evaluation of medium-vessel and small-vessel vasculitis.
November 2016: Rheumatic Diseases Clinics of North America
Elizabeth Manuely González Revilla, Araceli Abad Fernandez, María Teresa Río Ramirez, Sara Calero Pardo, María Antonia Juretschke Moragues
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures...
2016: Respiratory Medicine Case Reports
B Hellmich
Imaging methods, such as joint and color duplex sonography, magnetic resonance imaging (MRI) and positron emission tomography (PET) nowadays facilitate the diagnosis of polymyalgia rheumatica and large vessel vasculitides and have now been included in the new classification criteria. In patients with typical symptoms, color duplex sonography of the temporal artery can replace a biopsy of the temporal artery for the diagnosis of giant cell arteritis (GCA); however, the role of these methods for patient follow-up and assessment of prognosis is unclear...
September 8, 2016: Der Internist
Li-Li Pan, Juan Du, Na Gao, Hua Liao, Jin Wan, Wei-Ping Ci, Chun Yang, Tian Wang
Takayasu's arteritis (TAK) is a type of large vessel vasculitis which involves the aorta and its major branches. Interleukin (IL)-9 or IL-9-producing Th9 cells were found to be involved in pathogenesis of autoimmune arteritis such as giant cell arteritis, but IL-9 or Th9 cells in TAK were not well known. Here, this study aims to analyze the levels of serum IL-9 and their major source Th9 cells in TAK. With the help of cytometric bead array (CBA), a total of 21 patients with TAK were examined for serum levels of cytokines IL-4, IL-6, IL-8, IL-17, IL-10, TNF-α, IFN-γ, and IL-9...
September 15, 2016: Clinical Rheumatology
N Martins, J Polido-Pereira, A M Rodrigues, F Soares, P Batista, J A Pereira da Silva
Giant Cell Arteritis (GCA) is a large vessels vasculitis that is typically characterised by headache, scalp tenderness, jaw claudication and visual disturbances. Temporal arteries color Doppler ultrasonography (CDUS) is a sensitive and non-invasive image technique used in the diagnosis of this disease. This work highlights the importance of CDUS in the diagnostic workup of GCA and also demonstrates it´s usefullness in the evaluation and documentation of the response to corticosteroids therapy in an atypical case of ACG...
April 2016: Acta Reumatológica Portuguesa
Siwalik Banerjee, Philip P Stapleton, Bhaskar Dasgupta
No abstract text is available yet for this article.
August 2016: International Journal of Rheumatic Diseases
Giovanni M Rossi, Giacomo Emmi, Domenico Corradi, Maria L Urban, Federica Maritati, Federica Landini, Paola Galli, Alessandra Palmisano, Augusto Vaglio
Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006-2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-mediated disorders and to analyse which disorders most often coexist with IMF...
August 23, 2016: Clinical Reviews in Allergy & Immunology
Enrico Tombetti, Justin Mason
No abstract text is available yet for this article.
October 2016: Expert Review of Clinical Immunology
I Töpel, N Zorger, M Steinbauer
Aortitis is a term which encompasses inflammatory changes to the aortic wall from various pathogenic etiologies. Large vessel vasculitis, such as Takayasu arteritis and giant cell arteritis represent the most common entities; however, there is also an association with other rheumatological diseases. Chronic idiopathic periaortitis represents a distinct disease entity and infectious aortitis is a rare but life-threatening disease. Due to the diverse clinical pictures vascular surgeons often face a significant challenge in terms of making an accurate initial diagnosis...
2016: Gefässchirurgie: Zeitschrift Für Vaskuläre und Endovaskuläre Chirurgie
Masataka Umeda, Jin Ikenaga, Tomohiro Koga, Toru Michitsuji, Toshimasa Shimizu, Shoichi Fukui, Ayako Nishino, Yoshikazu Nakasima, Sin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Yasuko Hirai, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
A 78-year-old woman diagnosed with cyclic neutropenia 5 years previously had been treated with recombinant granulocyte-colony stimulating factor (G-CSF). She developed fever, tenderness and distension of temporal arteries after the treatment with G-CSF. Magnetic resonance imaging and ultrasonography revealed wall thickening of the temporal arteries. She was therefore diagnosed with giant cell arteritis (GCA). Small vessel vasculitis has been reported as a complication of G-CSF. However, the development of large vessel vasculitis after G-CSF treatment is quite rare...
2016: Internal Medicine
Aurel Doru ChiriŢă, Irina Mărgăritescu
Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Karla Maria Treitl, Stefan Maurus, Nora Narvina Sommer, Hendrik Kooijman-Kurfuerst, Eva Coppenrath, Marcus Treitl, Michael Czihal, Ulrich Hoffmann, Claudia Dechant, Hendrik Schulze-Koops, Tobias Saam
OBJECTIVES: To evaluate the feasibility of T1w-3D black-blood turbo spin echo (TSE) sequence with variable flip angles for the diagnosis of thoracic large vessel vasculitis (LVV). METHODS: Thirty-five patients with LVV, diagnosed according to the current standard of reference, and 35 controls were imaged at 3.0T using 1.2 × 1.3 × 2.0 mm(3) fat-suppressed, T1w-3D, modified Volumetric Isotropic TSE Acquisition (mVISTA) pre- and post-contrast. Applying a navigator and peripheral pulse unit triggering (PPU), the total scan time was 10-12 min...
August 10, 2016: European Radiology
Juan P Vinicki, Rosario García-Vicuña, Miguel Arredondo, Juan P López-Bote, Jesús A García-Vadillo, Santos Castañeda, José M Álvaro-Gracia
OBJECTIVES: To describe the results obtained in clinical practice with the use of biological therapy (BT) in patients diagnosed with Takayasu arteritis (TA) and giant cell arteritis (GCA). METHODS: Retrospective single center study of TA/GCA patients who received BT (infliximab [IFX], etanercept [ETN] and tocilizumab [TCZ]). In TA, active disease was defined according to a previous National Institutes of Health study. In GCA, active disease was defined with a modified criteria and clinical manifestations secondary to temporal artery involvement or polymyalgia rheumatica symptoms...
August 4, 2016: Reumatología Clinica
Elisabeth De Smit, Eoin O'Sullivan, David A Mackey, Alex W Hewitt
BACKGROUND: Giant cell arteritis (GCA) is a systemic granulomatous vasculitis, primarily affecting medium-large arteries. It has a predilection for the aorta and its major branches, including the carotid and vertebral arteries. Ophthalmic artery involvement frequently leads to irreversible visual loss, and therefore GCA is one of the few true ophthalmic emergencies. GCA, although classified as a large vessel vasculitis, is known to affect smaller-sized vessels, resulting in a multiplicity of signs in the eye, some of which are often missed...
August 5, 2016: Graefe's Archive for Clinical and Experimental Ophthalmology
Siddesh Shambhu, Lisbet Suarez
Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries in individuals over the age of 50. It is typically characterised by the granulomatous involvement of large and medium sized blood vessels branching of the aorta with particular tendencies for involving the extracranial branches of the carotid artery. Generally the diagnosis is straightforward when characteristic symptoms such as headache, jaw claudication, or other ischemic complications are present. Atypical presentations of GCA without "overt" cranial ischemic manifestations have become increasingly recognised but we report for the first time a case of GCA presenting as mild upper abdominal pain and generalized weakness in the context of hyponatremia as the presenting manifestation of vasculitis that was subsequently diagnosed by MRI scanning...
2016: Case Reports in Rheumatology
Hubert de Boysson, Grégoire Boulouis, Nelly Dequatre, Sophie Godard, Antoine Néel, Caroline Arquizan, Olivier Detante, Coralie Bloch-Queyrat, Mathieu Zuber, Emmanuel Touzé, Boris Bienvenu, Achille Aouba, Loïc Guillevin, Olivier Naggara, Christian Pagnoux
BACKGROUND AND PURPOSE: We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. METHODS: We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. RESULTS: The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0...
September 2016: Stroke; a Journal of Cerebral Circulation
Cory A Perugino, Zachary S Wallace, Nandini Meyersohn, George Oliveira, James R Stone, John H Stone
OBJECTIVES: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined...
July 2016: Medicine (Baltimore)
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