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small vessel vasculitis

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https://www.readbyqxmd.com/read/29045715/towards-precision-medicine-in-anca-associated-vasculitis
#1
Kornelis S M van der Geest, Elisabeth Brouwer, Jan-Stephan Sanders, Maria Sandovici, Nicolaas A Bos, Annemieke M H Boots, Wayel H Abdulahad, Coen A Stegeman, Cees G M Kallenberg, Peter Heeringa, Abraham Rutgers
ANCA-associated vasculitis (AAV) is characterized by inflammation and destruction of small and medium-sized vessels. Current management strategies for AAV have been validated in large groups of patients. However, recent insights indicate that distinct patient subsets may actually exist within AAV, thereby justifying the development of more personalized treatment strategies. In this review, we discuss current evidence for a better classification of AAV based on ANCA type. We describe how thus defined categories of AAV patients may differ in genetic background, clinical presentation, immune pathology, response to treatment and disease outcome...
October 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/29043146/calciphylaxis-in-a-nondialysis-patient-treated-with-sodium-thiosulfate-and-high-dose-of-oxygen
#2
Anawin Sanguankeo, Natanong Thamcharoen, Sikarin Upala
BACKGROUND: Calciphylaxis in a nondialysis patient is a rare condition and is characterized by calcific deposition in tissue. We present a case of calciphylaxis in a nondialysis patient who was diagnosed by clinical presentation and skin biopsy and was treated with sodium thiosulfate with improvement of skin lesions. CASE: A 43-year-old female with type 2 diabetes and atrial fibrillation taking oral anticoagulation medication presented with reddish drainage from the right buttock...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29038676/adult-onset-henoch-schonlein-purpura-duodenitis
#3
Emmanuel Ofori, Daryl Ramai, Mel A Ona, Charilaos Papafragkakis, Madhavi Reddy
Henoch-Schonlein purpura (HSP) is an immune-mediated vasculitis. HSP presents with purple spots on the skin (purpura), arthralgia, digestive problems, and kidney injury. HSP is most commonly seen in children, and rarely presents in adults. The pathogenesis involves the deposition of immune complexes in small to medium size blood vessels which leads to necrosis and inflammation. Most patients recover after symptomatic treatment, while more severe cases are treated with steroids. We report a 28-year-old female patient who presented with two episodes of hematemesis, worsening abdominal pain, and bloody diarrhea...
November 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29032440/vasculitis-and-vasculitis-like-manifestations-in-monogenic-autoinflammatory-syndromes
#4
REVIEW
Avinash Jain, Durga Prasanna Misra, Aman Sharma, Anupam Wakhlu, Vikas Agarwal, Vir Singh Negi
Monogenic autoinflammatory syndromes are a rare group of disorders characterized by periodic episodes of systemic inflammation of endogenous origin. Sometimes, these diseases may present with features akin to vasculitis. We conducted a literature review on such vasculitic manifestations in described monogenic autoinflammatory syndromes utilizing the Online Mendelian Inheritance in Man (OMIM), Medline, and Scopus databases. Our search identified that Familial Mediterranean fever (FMF) can manifest with features of either small, medium, large, or variable-vessel vasculitis...
October 14, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29032100/-severity-of-illness-index-in-autoimmune-diseases-have-there-any-usefulness-in-medical-practice
#5
A Saunier, M-A Vandenhende, P Morlat, F Bonnet
INTRODUCTION: Assessing disease activity in patients suffering from autoimmune diseases is complex. Symptoms are multiple, often subjective and there are no reliable biomarkers. Many activity scores have been implemented to compare treatment efficacy in clinical trials. Their use in clinical practice is largely unknown. We performed a practical survey to analyze the use of activity scores in clinical practice to consider treatment response and to assess the determinants of their use. METHODS: A sample of French internists answered a questionnaire about activity scores of systemic lupus erythematosus, Sjögren's syndrome, autoimmune myositis and necrotizing vasculitis of small vessels...
October 11, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29026760/etiological-and-clinicopathological-study-of-secondary-small-vessel-vasculitis-in-elderly-a-case-series-of-12-patients
#6
Ajay Kumar Mishra, Ramya Iyadurai, Anu Anna George, Ebenezer Rajdurai, V Surekha
BACKGROUND: Inflammation involving the postcapillary venular wall is defined as small vessel vasculitis. Small vessel vasculitis has various clinical manifestations. Etiologically, it can be primary or secondary. Literature regarding secondary vasculitis in elderly is scanty. AIM AND OBJECTIVES: In this case series, we aimed to assess the clinical features and etiologies of biopsy-proven secondary small vessel vasculitis in the elderly. METHODOLOGY: Twelve elderly patients with biopsy-proven small vessel vasculitis were included in this study...
January 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29026588/granulomatosis-with-polyangiitis-can-cause-periaortitis-and-pericarditis
#7
Mari Miyawaki, Seitaro Oda, Kenichiro Hirata, Hideaki Yuki, Daisuke Utsunomiya, Hidetaka Hayashi, Yasumiko Sakamoto, Shinichiro Okamoto, Kazuhiko Fujii, Yasuyuki Yamashita
Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and necrotizing vasculitis predominantly affecting small-sized vessels. Although categorized as a small vessel vasculitis, GPA can cause periaortitis and pericarditis. Physicians should recognize all categories of vasculitis can affect any size arteries.
October 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29021484/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-complicated-by-periaortitis-and-cranial-hypertrophic-pachymeningitis-a-report-of-an-autopsy-case
#8
Hajime Kaga, Atsushi Komatsuda, Masaya Saitoh, Mizuho Nara, Ayumi Omokawa, Masaru Togashi, Shin Okuyama, Hideki Wakui, Naoto Takahashi
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic inflammatory disorder categorized as small-vessel vasculitis. We herein report an elderly Japanese man with AAV (granulomatous with polyangiitis affecting the eyes, nose, lungs, and kidneys) who also showed periaortitis at the diagnosis and developed cranial hypertrophic pachymeningitis (HP) during steroid maintenance therapy. His consciousness disturbance caused by HP improved after steroid pulse therapy, but he died of aspiration pneumonia...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28983940/outline-of-guidelines-for-the-management-of-vasculitis-and-vascular-disorders-in-japan-2016-revised-edition
#9
Takaharu Ikeda, Fukumi Furukawa, Tamihiro Kawakami, Naoko Ishiguro, Miwa Uzuki, Shoichi Ozaki, Kensei Katsuoka, Takeshi Kono, Seiji Kawana, Masanari Kodera, Takashi Sawai, Yasuyuki Sawada, Mariko Seishima, Akiko Tanikawa, Ko-Ron Chen, Minoru Hasegawa
The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small-vessel vasculitis is only a pathological name...
October 6, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28982784/clinical-and-imaging-characteristics-of-arteriopathy-subtypes-in-children-with-arterial-ischemic-stroke-results-of-the-vips-study
#10
M Wintermark, N K Hills, G A DeVeber, A J Barkovich, T J Bernard, N R Friedman, M T Mackay, A Kirton, G Zhu, C Leiva-Salinas, Q Hou, H J Fullerton
BACKGROUND AND PURPOSE: Childhood arteriopathies are rare but heterogenous, and difficult to diagnose and classify, especially by nonexperts. We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. MATERIALS AND METHODS: The Vascular Effects of Infection in Pediatric Stroke (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (2010-2014)...
October 5, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28980290/atherosclerosis-in-antineutrophil-cytoplasmic-autoantibody-anca-associated-vasculitis
#11
Katarzyna Życińska, Anna Borowiec
The primary systemic vasculitides are heterogeneous, multisystem disorders characterized by inflammation and necrosis of small and medium blood vessels. Patients with antineutrophil cytoplasmic autoantibodies associated vasculitis have increased mortality rate as a consequence of cardiovascular disease. Measures to reduce the risk of cardiovascular disease should be integral to the management of systemic vasculitis. The preventive therapy for accelerated atherosclerosis in systemic vasculitis should be based on aggressive approach against inflammation and against risk factors of premature atherosclerosis such as hypertension, smoking, inactivity, obesity and unhealthy diet...
October 5, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28961286/asymptomatic-cutaneous-polyarteritis-nodosa-treatment-options-and-therapeutic-guidelines
#12
Kate Ross, Jessika Contreras, David Aung-Din, Mary Lien
Cutaneous polyarteritis nodosa (CPAN) is a rare cutaneous small- to medium-vessel vasculitis of unknown etiology. Clinically it ranges in manifestation from livedo reticularis to large cutaneous ulcers and necrosis. Prognosis is favorable and progression to systemic polyarteritis nodosa is rare. There are multiple treatment options, none of which have proven to be definitively effective. Cutaneous polyarteritis nodosa has been associated with abnormal antibody testing with elevations of antiphospholipid cofactor antibody, lupus anticoagulant, anticardiolipin antibody, and anti-β2-glycoprotein I-dependent cardiolipin antibodies, as well as elevated anti-phosphatidylserine-prothrombin complex antibody...
August 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28960271/alterations-in-circulating-lymphoid-cell-populations-in-systemic-small-vessel-vasculitis-are-non-specific-manifestations-of-renal-injury
#13
Barbara Fazekas, Ana Moreno-Olivera, Yvelynne Kelly, Paul O'Hara, Susan Murray, Alan Kennedy, Niall Conlon, Jennifer Scott, Ashanty M Melo, Fionnuala B Hickey, Dearbhaile Dooley, Eoin C O'Brien, Sarah Moran, Derek G Doherty, Mark A Little
Innate lymphocyte populations, such as innate lymphoid cells (ILCs), γδ T cells, invariant natural killer T (iNKT) cells and mucosal associated invariant T (MAIT) cells are emerging as important effectors of innate immunity and are involved in various inflammatory and autoimmune diseases. The aim of this study was to assess the frequencies and absolute numbers of innate lymphocytes as well as conventional lymphocytes and monocytes in peripheral blood from a cohort of ANCA associated vasculitis (AAV) patients...
September 28, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28957962/neutrophil-extracellular-traps-in-vasculitis-friend-or-foe
#14
Daniel Söderberg, Mårten Segelmark
PURPOSE OF REVIEW: Neutrophil extracellular traps (NETs) can be found at the sites of vascular lesions and in the circulation of patients with active small vessel vasculitis. Neutrophils from vasculitis patients release more NETs in vitro, and NETs have properties that can harm the vasculature both directly and indirectly. There are several ways to interfere with NET formation, which open for new therapeutic options. However, there are several types of NETs and different mechanisms of NET formation, and these might have different effects on inflammation...
September 27, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28956128/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-prevalence-treatment-and-outcomes
#15
REVIEW
Lei Shi
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a primary small-vessel vasculitis group with three distinct clinical entities, including GPA, MPA, and EGPA. The incidence of AAV has increased since the 1980s and remained stable since the early 2000s. Distinct phenotypes of AAV may also differ in various geographical regions. Elderly people are susceptible to developing AAV, and AAV is used to be a fatal disease before the introduction of glucocorticoids and immunosuppressants. Different treatment protocols should be employed for patients with different disease severity levels...
September 27, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28954096/vascular-sclerosing-effects-of-bleomycin-on-cutaneous-veins-a-pharmacopathologic-study-on-experimental-animals
#16
Khalid M AlGhamdi, Ashok Kumar, Abdelkader E Ashour, Ammar C Al-Rikabi, Abdullah Hasan AlOmrani, Shaik Shaffi Ahamed
Background: Varicose veins and the complications of venous disease are common disorders in humans. Objective: To study the effects of bleomycin as a potential new sclerosing agent and its adverse events in treating varicose veins. Methods: Bleomycin-loaded liposomes 0.1ml was injected in the dorsal ear veins of white New Zealand rabbits. Sodium tetradecyl sulfate was used as a positive control. Normal saline was used as negative control. The blood vessels of the treated ears were photographed before and at one hour and two, eight and 45 days after treatment...
July 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28953791/neurosyphilis-the-white-matter-disintegration-two-case-reports
#17
Monika Czarnowska-Cubała, Adam Włodarczyk, Joanna Szarmach, Katarzyna Gwoździewicz, Joanna Pieńkowska, Mariusz S Wiglusz, Wiesław Jerzy Cubała, Krzysztof Krysta
BACKGROUND: There is evidence for neurosyphilis being associated with the central nervous system vasculitis involving medium and small vessels. As the hemispheric white matter is the major target of these vascular alterations the white matter axonal and myelination disruption may be observed employing measure for the rate of water molecule diffusion. High apparent diffusion coefficient (ADC) correspond to unimpeded water diffusion and indicating white matter disintegration. CASE REPORTS: In a retrospective study exploringcentral nervous system magnetic resonance (MR) images of two subjects presenting with neurosyphilis the ADC values were found to be increased as related to normal values being accompanied with normal appearing white matter of hemispheres...
September 2017: Psychiatria Danubina
https://www.readbyqxmd.com/read/28936074/syndromes-of-rapidly-progressive-cognitive-decline-our-experience
#18
Sadanandavalli Retnaswami Chandra, Lakshminarayanapuram Gopal Viswanathan, Anupama Ramakanth Pai, Rahul Wahatule, Suvarna Alladi
BACKGROUND: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. AIMS: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center...
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28934973/liver-chemistry-in-new-onset-henoch-sch%C3%A3-nlein-syndrome
#19
Giulia Rosti, Gregorio P Milani, Emanuela A Laicini, Emilio F Fossali, Mario G Bianchetti
BACKGROUND: Henoch-Schönlein syndrome is a systemic small-vessel leukocytoclastic vasculitis that usually present with cutaneous, gastrointestinal, articular and renal manifestations. Little is known on liver involvement in this syndrome. This study investigated liver chemistry and creatine kinase in Henoch-Schönlein children. CASE PRESENTATION: Alanine aminotransferase, aspartate aminotransferase, γ-glutamyltransferase, lactate dehydrogenase, total bilirubin, prothrombin time and creatine kinase were assessed in 75 consecutive pediatric patients (41 boys and 34 girls aged from 2...
September 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28929294/neurologic-complications-of-infective-endocarditis-recent-findings
#20
REVIEW
Marie Cantier, Mikael Mazighi, Isabelle Klein, J P Desilles, Michel Wolff, J F Timsit, Romain Sonneville
PURPOSE OF REVIEW: The purpose of this paper is to provide recent insights in management of neurologic complications of left-sided infective endocarditis (IE). RECENT FINDINGS: Cerebral lesions observed in IE patients are thought to involve synergistic pathophysiological mechanisms including thromboembolism, sepsis, meningitis, and small-vessel cerebral vasculitis. Brain MRI represents a major tool for the detection of asymptomatic events occurring in the majority of patients...
September 19, 2017: Current Infectious Disease Reports
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