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https://www.readbyqxmd.com/read/28741986/serum-ferritin-as-an-activity-marker-for-granulamotosis-with-polyangiitis
#1
Hamit Kucuk, Ozkan Varan, Berna Goker, Berivan Bitik, Mehmet Akif Ozturk, Seminur Haznedaroglu, Abdurrahman Tufan
BACKGROUND: Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis. METHODS: Medical records of granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28739610/silicone-implant-incompatibility-syndrome-siis-in-a-57-year-old-woman-with-unilateral-silicone-breast-implant
#2
Juliane Schierbeck, Jesper Rømhild Davidsen, Sanne Grindsted Nielsen, Camilla Bille
Since the 1960s, silicone implants have been used for breast augmentations, both cosmetically and in reconstructive surgery. Tissue exposed to silicone can react with multiple adverse advents. Autoimmune/inflammatory syndrome induced by adjuvants due to silicone exposure from ruptured silicone implants can lead to different interstitial lung manifestations predominantly with granuloma evolvement, leading to the so-called silicone implant incompatibility syndrome (SIIS). This case describes a 57-year-old woman with multiple lung infiltrations and a left-sided breast implant...
July 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28739362/stroke-in-ehlers-danlos-syndrome-kyphoscoliotic-type-dissection-or-vasculitis
#3
Annegret Quade, Martin Wiesmann, Joachim Weis, Ingo Kurth, Houman Jalaie, Marianne Rohrbach, Martin Häusler
BACKGROUND: Patients with the kyphoscoliotic type of Ehlers-Danlos syndrome have an increased risk of vascular complications such as aortic dissection and perforation. Cerebral ischemia has only rarely been documented. PATIENT DESCRIPTION: This 13-year-old girl with the kyphoscoliotic type of Ehlers-Danlos syndrome had a large right middle cerebral artery distribution infarction. Full intravenous heparinization was started in response to presumed arterial dissection...
May 29, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28736547/thyroid-involvement-in-hepatitis-c-virus-infected-patients-with-without-mixed-cryoglobulinemia
#4
REVIEW
Clodoveo Ferri, Michele Colaci, Poupak Fallahi, Silvia Martina Ferrari, Alessandro Antonelli, Dilia Giuggioli
Thyroid involvement is a common condition that can be recorded during the natural course of different systemic rheumatic diseases, including the mixed cryoglobulinemia (MC) syndrome or cryoglobulinemic vasculitis. MC is triggered by hepatitis C virus (HCV) chronic infection in the majority of cases; it represents the prototype of autoimmune-lymphoproliferative disorders complicating a significant proportion of patients with chronic HCV infection. HCV is both hepato- and lymphotropic virus responsible for a great number of autoimmune/lymphoproliferative and/or neoplastic disorders...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28735554/magnetic-resonance-imaging-in-central-nervous-system-vasculitis-in-a-patient-affected-by-crioglobulin-negative-hepatitis-c-virus-infection-a-likely-correlation
#5
Silvia Squarza, Alberto Galli, Maurizio Cariati, Federico Alberici, Valentina Bertolini, Fabio Frediani, Carla Uggetti
A 56-year-old man with behavioural disorders and facial-brachio-crural right hemiparesis presented with a brain lesion studied with computed tomography, magnetic resonance imaging and brain biopsy, leading to the diagnosis of cerebral vasculitis. Hepatitis C virus (HCV) infection in a phase of activity, without cryoglobulins, was also detected. Brain biopsy, laboratory analysis and response to a specific therapy supported the diagnosis of central nervous system vasculitis that was HCV related.
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28734234/association-of-primary-central-nervous-system-vasculitis-with-the-presence-of-specific-human-leucocyte-antigen-gene-variant
#6
Markus Kraemer, Jana Becker, Peter A Horn, Jan Claudius Schwitalla, Kathy Keyvani, Imke Metz, Christiane Wegner, Wolfgang Brück, Marc Schlamann, Falko M Heinemann, Peter Berlit
OBJECTIVES: The etiology and genetic susceptibility of primary central nervous system vasculitis (PCNSV) are still unclear. PATIENTS AND METHODS: We analyzed the DNA of 25 Caucasian patients with PCNSV for human leucocyte antigen genes HLA-A, HLA-B, HLA-DRB1, and HLA-DQB1, respectively. HLA-frequencies of the 25 patients with PCNSV were compared with HLA-frequencies of matched Caucasian controls. RESULTS: No statistically significant associations were found for HLA-B, HLA-DR1 and HLA-DQB1 variant...
June 23, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28733791/current-clinical-evidence-of-tocilizumab-for-the-treatment-of-anca-associated-vasculitis-a-prospective-case-series-for-microscopic-polyangiitis-in-a-combination-with-corticosteroids-and-literature-review
#7
REVIEW
Ryota Sakai, Tsuneo Kondo, Takahiko Kurasawa, Eiko Nishi, Ayumi Okuyama, Kentaro Chino, Akiko Shibata, Yusuke Okada, Hirofumi Takei, Hayato Nagasawa, Koichi Amano
The purpose of this study is to report the efficacy and safety of a combination of tocilizumab (TCZ) and high-dose corticosteroid (CS) in two patients with microscopic polyangiitis (MPA) and review the published current clinical evidence on TCZ in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), except for large vessel vasculitis (LVV) and polymyalgia rheumatica (PMR). Two MPA patients were treated with TCZ at 8 mg/kg every month for 1 year and CS (prednisolone 1 mg/kg/day for 2 weeks, followed by tapering) in a prospective single-arm, single-center, cohort, open-label pilot study (UMIN clinical trials: 000012072)...
July 21, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28732837/immune-diffuse-alveolar-hemorrhage-clinical-presentation-and-outcome
#8
Silvia Quadrelli, Diana Dubinsky, Marco Solis, Demelza Yucra, Marcos Hernández, Hugo Karlen, Alejandro Brigante
BACKGROUND: Diffuse alveolar haemorrhage (DAH) is a manifestation of several immune and nonimmune diseases. OBJECTIVE: The objective of this study was to characterize the clinical characteristics and short-term outcomes of patients with immune-mediated DAH requiring hospital admission. METHODS: A retrospective study from December 2010 to December 2015, was conducted by analyzing the clinical records of 39 patients with DAH with a proven immunological origin...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732547/crescentic-glomerular-nephritis-associated-with-rheumatoid-arthritis-a-case-report
#9
K Balendran, L D S U Senarathne, R D Lanerolle
BACKGROUND: Rheumatoid arthritis is a systemic disorder where clinically significant renal involvement is relatively common. However, crescentic glomerular nephritis is a rarely described entity among the rheumatoid nephropathies. We report a case of a patient with rheumatoid arthritis presenting with antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis. CASE PRESENTATION: A 54-year-old Sri Lankan woman who had recently been diagnosed with rheumatoid arthritis was being treated with methotrexate 10 mg weekly and infrequent nonsteroidal anti-inflammatory drugs...
July 21, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28731865/-joining-the-spots-in-adults-and-young-tots-a-clinicopathological-study-of-henoch-sch%C3%A3-nlein-purpura-iga-vasculitis
#10
Priyanka P Kedia, Rajalakshmi Tirumalae, Divya Puttegowda, Meryl Antony
INTRODUCTION: Henoch-Schönlein Purpura (HSP; IgA vasculitis), the most common vasculitis of childhood, has a wide spectrum of clinical manifestations ranging from palpable purpura with abdominal pain and arthritis to the more morbid renal involvement. AIMS AND OBJECTIVES: To study and correlate the clinical presentation, laboratory values, skin and renal histopathology and immunofluoroscence findings in HSP. MATERIAL AND METHODS: A total of 44 cases of HSP from March 2011 to February 2014 were studied for the above features along with their clinical outcomes...
August 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28731673/-kawasaki-disease-interdisciplinary-and-intersocieties-consensus-clinical-guidelines-brief-version
#11
(no author information available yet)
Kawasaki disease is an acute self-limiting systemic vasculitis. It is the most common cause of acquired heart disease, with the risk of developing coronary artery aneurysms, myocardial infarction and sudden death. Diagnosis is based on the presence of fever in addition to other clinical criteria. The quarter of the Kawasaki disease patients have "incomplete" presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28731667/-henoch-schonlein-purpura-involving-the-penis-a-case-report
#12
Borja Croche Santander, Elena Campos, Adela Sánchez, Laura Marcos, Isabel Díaz, Cristóbal Toro
Schonlein-Henoch purpura accounts for the majority of cases of systemic vasculitis in children. Classical presentation is characterized by palpable purpura, glomerulonephritis, arthralgias and abdominal pain. Although genitourinary manifestations, in form of testicular and scrotal involvement, have been widely described, penile involvement remains an extremely rare complication. We report a case of a 6-year-old boy who presented with purpuric rash on the glans, prepuce and penile shaft, with painful edema in the penile region...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28728567/settings-and-artefacts-relevant-for-doppler-ultrasound-in-large-vessel-vasculitis
#13
REVIEW
L Terslev, A P Diamantopoulos, U Møller Døhn, W A Schmidt, S Torp-Pedersen
Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used in arthritic conditions and provide recommendations for optimal adjustments. This is addressed through relevant Doppler physics, focusing, for example, on the Doppler shift equation and how angle correction ensures correctly displayed blood velocity...
July 20, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28726127/clippers
#14
REVIEW
Nicholas L Zalewski, W Oliver Tobin
PURPOSE OF REVIEW: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker. RECENT FINDINGS: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases...
September 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28724596/severe-destructive-nasopharyngeal-granulomatosis-with-polyangiitis-with-superimposed-skull-base-pseudomonas-aeruginosa-osteomyelitis
#15
Mitchell S von Itzstein, Jithma P Abeykoon, Daniel D Summerfield, Jennifer A Whitaker
Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28724595/hypertensive-encephalopathy-mimicking-cerebral-vasculitis-with-pontine-oedema-cerebellar-white-matter-lesions-and-multiple-cerebral-infarctions
#16
Daniela Ceccarelli, David Hargroves, Ibrahim Balogun, Thomas Webb
A 47-year-old man with poorly controlled hypertension presented with headaches, right-sided weakness and dysarthria. CT and MRI scans of the brain showed widespread abnormalities including significant pontine oedema, basal ganglia and corona radiata infarctions and cerebellar white matter high signal. Imaging of the intracerebral vasculature also demonstrated wall irregularities. Initially a central nervous system inflammatory disorder was thought to be the most likely diagnosis, possibly acute demyelinating encephalomyelitis or cerebral vasculitis, and the patient was treated with high-dose intravenous steroids...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28724574/the-microvascular-niche-instructs-t-cells-in-large-vessel-vasculitis-via-the-vegf-jagged1-notch-pathway
#17
Zhenke Wen, Yi Shen, Gerald Berry, Farhad Shahram, Yinyin Li, Ryu Watanabe, Yaping Joyce Liao, Jörg J Goronzy, Cornelia M Weyand
Microvascular networks in the adventitia of large arteries control access of inflammatory cells to the inner wall layers (media and intima) and thus protect the immune privilege of the aorta and its major branches. In autoimmune vasculitis giant cell arteritis (GCA), CD4 T helper 1 (TH1) and TH17 cells invade into the wall of the aorta and large elastic arteries to form tissue-destructive granulomas. Whether the disease microenvironment provides instructive cues for vasculitogenic T cells is unknown. We report that adventitial microvascular endothelial cells (mvECs) perform immunoregulatory functions by up-regulating the expression of the Notch ligand Jagged1...
July 19, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28724375/bilateral-idiopathic-retinal-vasculitis-following-coxsackievirus-a4-infection-a-case-report
#18
Izumi Mine, Manzo Taguchi, Yutaka Sakurai, Masaru Takeuchi
BACKGROUND: Coxsackieviruses are members of a group of viruses called the enteroviruses, which may cause respiratory and gastrointestinal symptoms, erythema, meningoencephalitis, myocarditis, pericarditis, and myositis. Unilateral acute idiopathic maculopathy caused by coxsackievirus A16 has been associated with hand, foot, and mouth disease, but only a few reports describe retinitis associated with coxsackievirus serotype B3 or B4. We report a case of bilateral multifocal obstructive retinal vasculitis that developed after coxsackievirus A4 infection...
July 19, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28722366/-large-vessel-vasculitis-pathogenesis-diagnostic-and-medical-management
#19
Alireza Sadrkhah, Jörg D Seebach, Yannick Muller
Large vessels vasculitis includes two diseases : 1) giant cell arteritis, formerly known as Horton's arteritis and 2) Takayasu arteritis. In this article, we will describe and compare the epidemiology, pathogenesis, diagnostic criteria and medical management of both vasculitis. T helper (Th) 1 and Th17 responses, genetic links and the role of viral (varicella zoster) and bacterial infection (Mycoplasma pneumoniae or Chlamydia pneumoniae) will be discussed. Classification criteria, inflammation biomarkers and progress in imaging tools will also be described...
April 5, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28722365/-segmental-arterial-mediolysis-a-differential-diagnosis-of-vasculitis
#20
Yassaman Alipour Tehrany, Sylvain Terraz, Jörg D Seebach
Segmental arterial mediolysis (SAM) is a rare non-atherosclerotic, non-inflammatory disease of unknown aetiology mostly affecting medium-sized arteries of the splanchnic circulation. Histologically, SAM is characterized by vacuolization and lysis of the arterial media leading to dissection, stenosis/occlusion or aneurysms. Symptoms vary from unspecific abdominal pain to hemorrhagic shock due to vessel rupture. The clinical and radiological presentation can mimic vasculitis, fibromuscular dysplasia or collagen vascular diseases...
April 5, 2017: Revue Médicale Suisse
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