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"Small vessel vasculitis"

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https://www.readbyqxmd.com/read/29133703/cns-small-vessel-vasculitis-distinct-mri-features-and-histopathological-correlation
#1
Kamble J Harsha, Sujit A Jagtap, Tirur R Kapilamoorthy, Chandrasekharan Kesavadas, Bejoy Thomas, Neelima Radhakrishnan
BACKGROUND: Central nervous system (CNS) vasculitis is an uncommon disease, which is a diagnostic and therapeutic challenge for physicians. Large and medium vessel vasculitis is relatively easy to diagnose by angiogram compared to small vessel vasculitis, where angiograms are often normal; imaging features described till date are sensitive but not specific. PURPOSE: Here, we describe distinct magnetic resonance (MR) imaging features of CNS small vessel vasculitis...
November 2017: Neurology India
https://www.readbyqxmd.com/read/29056777/relapsing-granulomatosis-with-polyangiitis-with-severe-lung-and-upper-respiratory-tract-involvement-successfully-treated-with-rituximab
#2
Anna Masiak, Zbigniew Zdrojewski
Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA; nevertheless, some patients do not respond satisfactorily to this treatment. According to EULAR-EDTA recommendations for the management of ANCA-associated vasculitis, RTX should be considered for remission-induction of new-onset as well as major relapse of organ-threatening or life-threatening GPA...
2017: Reumatologia
https://www.readbyqxmd.com/read/29043146/calciphylaxis-in-a-nondialysis-patient-treated-with-sodium-thiosulfate-and-high-dose-of-oxygen
#3
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/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/29026760/etiological-and-clinicopathological-study-of-secondary-small-vessel-vasculitis-in-elderly-a-case-series-of-12-patients
#5
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
#6
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
#7
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
#8
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/28960271/alterations-in-circulating-lymphoid-cell-populations-in-systemic-small-vessel-vasculitis-are-non-specific-manifestations-of-renal-injury
#9
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
#10
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
#11
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...
November 2017: Rheumatology International
https://www.readbyqxmd.com/read/28905856/position-paper-revised-2017-international-consensus-on-testing-of-ancas-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#12
REVIEW
Xavier Bossuyt, Jan-Willem Cohen Tervaert, Yoshihiro Arimura, Daniel Blockmans, Luis Felipe Flores-Suárez, Loïc Guillevin, Bernhard Hellmich, David Jayne, J Charles Jennette, Cees G M Kallenberg, Sergey Moiseev, Pavel Novikov, Antonella Radice, Judith Anne Savige, Renato Alberto Sinico, Ulrich Specks, Pieter van Paassen, Ming-Hui Zhao, Niels Rasmussen, Jan Damoiseaux, Elena Csernok
Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications...
November 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28880680/the-effect-and-possible-clinical-efficacy-of-in-vivo-inhibition-of-neutrophil-extracellular-traps-by-blockade-of-pi3k-gamma-on-the-pathogenesis-of-microscopic-polyangiitis
#13
Hirotaka Kimura, Yasushi Matsuyama, Sachiko Araki, Atsushi Koizumi, Yumi Kariya, Shunsuke Takasuga, Satoshi Eguchi, Hiroki Nakanishi, Junko Sasaki, Takehiko Sasaki
OBJECTIVE: Neutrophil extracellular traps (NETs) are peculiar structures composed of the externalized chromatin with intracellular proteins and formed by activated neutrophils in a reactive oxygen species (ROS)-dependent manner. Aberrant NETs are considered to be autoantigens for anti-neutrophil cytoplasmic antibodies (ANCAs) underling the development of microscopic polyangiitis (MPA). However, little is known regarding the therapeutic efficacy of in vivo inhibition of NET formation (NETosis) on MPA pathogenesis...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28817406/idiopathic-myointimal-hyperplasia-of-mesenteric-veins-an-uncommon-cause-of-ischemic-colitis-with-distinct-mucosal-features
#14
Rhonda K Yantiss, Isabelle Cui, Nicole C Panarelli, Jose Jessurun
Idiopathic myointimal hyperplasia of mesenteric veins causes chronic ischemic mucosal injury with segmental strictures that mimic inflammatory bowel disease and nonocclusive ischemic colitis. It is characterized by myointimal proliferative changes that narrow the lumina of veins combined with ischemic injury and ulcers. Most cases reported to date have been diagnosed following surgical resection. The aim of this study was to determine whether mucosal changes of idiopathic myointimal hyperplasia of mesenteric veins are sufficiently sensitive and specific to allow its recognition in biopsy material...
December 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28794554/mucocutaneous-manifestations-in-patients-with-rheumatoid-arthritis-a-cross-sectional-study-from-eastern-india
#15
Sudip Kumar Ghosh, Debabrata Bandyopadhyay, Surajit Kumar Biswas, Ivoreen Darung
BACKGROUND: Cutaneous manifestations are fairly common in rheumatoid arthritis (RA) and they can help in early diagnosis, prompt treatment, and hence reduced morbidity from the disease. AIMS: The objective of the present study was to find out the different patterns of dermatoses in a group of patients with RA from Eastern India. METHODOLOGY: Consecutive patients fulfilling the American Rheumatism Association 1987 revised criteria for the classification of RA and who had different dermatoses were included in this cross-sectional study done over a period of 8 years in a tertiary care hospital in Eastern India...
July 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28774183/annals-express-altered-mean-platelet-volume-in-children-with-henoch-schonlein-purpura-and-its-association-with-disease-activity
#16
Xiang Shi, Wen Chao Li, Li Jun Mo, Xiao Hong Li, Yu Zhen Luo, Liu Qun Qin, Zheng Yang, Wu Ning Mo
BACKGROUND: Henoch-Schonlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. A review of the literature has suggested a correlation between mean platelet volume (MPV) and several inflammatory disorders. However, to the best of our knowledge, any potential correlation between MPV and HSP has not been reported in the literature. Therefore, our study aimed to evaluate the role of MPV levels in patients with HSP. METHODS: This study included 97 children with HSP and 120 healthy individuals as controls...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28771805/cutaneous-small-vessel-vasculitis-in-two-children-with-inflammatory-bowel-disease-case-series-and-review-of-the-literature
#17
Tina Ho, Lauren A V Orenstein, Markus D Boos, Kevin P White, Nicole Fett
Cutaneous small-vessel vasculitis (CSVV) is an infrequent manifestation of pediatric inflammatory bowel disease (IBD). We report two cases of CSVV associated with ulcerative colitis, review the literature, and discuss the diagnostic evaluation of children who present with CSVV and abdominal pain. After excluding more common causes of CSVV and abdominal pain in children, including immunoglobulin A vasculitis (previously Henoch-Schönlein purpura), infectious colitis, and drug-induced vasculitis, alternative diagnoses such as CSVV secondary to IBD or systemic vasculitis with gastrointestinal involvement must be considered...
August 2, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28762062/sequential-rituximab-and-omalizumab-for-the-treatment-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#18
REVIEW
David Aguirre-Valencia, Iván Posso-Osorio, Juan-Carlos Bravo, Fabio Bonilla-Abadía, Gabriel J Tobón, Carlos A Cañas
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms...
July 31, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28756866/tuberculosis-granulomatosis-with-polyangiitis-or-both-a-case-report
#19
Á Nava-Castañeda, F Martín, S Voorduin, F Zuazo
INTRODUCTION: Tuberculosis (TB) is a chronic granulomatose infection, and granulomatosis with polyangiitis (GP) is a small vessel vasculitis, both of which affect the lungs. The combination of these diseases is rare. Both have similar clinical features, making the differential diagnosis difficult. CASE REPORT: It concerns a 37 year-old female undergoing treatment for pulmonary TB, who presented with left ocular proptosis, eyelid and conjunctival edema and erythema...
July 27, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28697842/-research-advances-in-immunological-pathogenesis-of-immunoglobulin-a-vasculitis
#20
REVIEW
Ya-Ting Liu, Si-Guang Lu
Immunoglobulin A (IgA) vasculitis is the most common leukocytoclastic small-vessel vasculitis in children and mainly involves the small vessels in the skin, joints, digestive tract, and kidneys. Its pathogenesis is still unclear. Currently, it is believed that environmental factors can cause autoimmune dysfunction and lead to the deposition of IgA-containing immune complexes on the wall of arterioles on the basis of genetic factors. This article reviews the research advances in the role of immune factors in the pathogenesis of IgA vasculitis...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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