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Systemic vasculitis

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https://www.readbyqxmd.com/read/28936058/multifocal-choroiditis-with-retinal-vasculitis-optic-neuropathy-and-keratoconus-in-a-young-saudi-male
#1
Yousef Dhafiri, Khalid Al Rubaie, Omar Kirat, William N May, Quan D Nguyen, Igor Kozak
The purpose of this study is to describe an association of unilateral multifocal choroiditis (MFC), retinal vasculitis, optic neuropathy, and bilateral keratoconus in a young Saudi male. A 27-year-old male patient with stable bilateral keratoconus presented with a painless vision loss in his left eye. Ophthalmic examinations revealed multiple foci of idiopathic chorioretinitis, retinal vasculitis, and mild optic disc leakage on fluorescein angiography, all of which resolved on systemic therapy with mycophenolate mofetil and prednisone after 3 months...
April 2017: Middle East African Journal of Ophthalmology
https://www.readbyqxmd.com/read/28934973/liver-chemistry-in-new-onset-henoch-sch%C3%A3-nlein-syndrome
#2
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/28934765/qualitative-study-the-experience-and-impact-of-living-with-behcet-s-syndrome
#3
Vicky Tai, Karen Lindsay, Joanne L Sims, Fiona M McQueen
AIM: Behcet's syndrome is a rare chronic multisystemic vasculitis of unknown aetiology, is unpredictable and can cause life-threatening complications. This qualitative study aims to explore the experiences of patients living with Behcet's syndrome in New Zealand. METHODS: Eight English-speaking patients participated in in-depth semi-structured interviews about their experiences of living with Behcet's syndrome. Interviews were recorded and transcribed. Data were analysed using a general inductive thematic approach...
September 22, 2017: New Zealand Medical Journal
https://www.readbyqxmd.com/read/28927316/cardiovascular-magnetic-resonance-detects-silent-heart-disease-missed-by-echocardiography-in-systemic-lupus-erythematosus
#4
S Mavrogeni, L Koutsogeorgopoulou, G Markousis-Mavrogenis, A Bounas, M Tektonidou, S-N C Lliossis, D Daoussis, S Plastiras, G Karabela, E Stavropoulos, G Katsifis, V Vartela, G Kolovou
Background Accurate diagnosis of cardiovascular involvement in systemic lupus erythematosus (SLE) remains challenging, due to limitations of echocardiography. We hypothesized that cardiovascular magnetic resonance can detect cardiac lesions missed by echocardiography in SLE patients with atypical symptoms. Aim To use cardiovascular magnetic resonance in SLE patients with atypical symptoms and investigate the possibility of silent heart disease, missed by echocardiography. Patients/methods From 2005 to 2015, 80 SLE patients with atypical cardiac symptoms/signs (fatigue, mild shortness of breath, early repolarization and sinus tachycardia) aged 37 ± 6 years (72 women/8 men), with normal echocardiography, were evaluated using a 1...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28920811/salt-bath-as-a-treatment-for-idiopathic-dermatitis-in-captive-nile-hippopotamus-hippopotamus-amphibius
#5
Kelly E Helmick
Ulcerative skin lesions were observed in two captive adult female hippos (Hippopotamus amphibius) from a zoological collection. Lesions appeared as cracking, peeling, or vesicles of the skin affecting the lateral and ventral aspects of the neck, limbs, thorax, and abdomen, dorsum, toes, and perineal region. Some lesions drained blood, serum, or purulent material. Histologic evaluation of sloughed skin consisted of deep dermal collagen with bacterial cellulitis and vasculitis and superficial fungal colonization...
September 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/28919362/a-homozygote-trex1-mutation-in-two-siblings-with-different-phenotypes-chilblains-and-cerebral-vasculitis
#6
Rabia Miray Kisla Ekinci, Sibel Balci, Atil Bisgin, Derya Ufuk Altintas, Mustafa Yılmaz
Three prime repair exonuclease 1 degrades single and double stranded DNA with 3'-5' nuclease activity and its mutations are related to type 1 IFN mediated autoinflammation due to accumulated intracellular nucleic acids. To date, several cases of systemic lupus erythematosus, Aicardi-Goutieres syndrome, familial chilblain lupus, retinal vasculopathy-cerebral leukodystrophy have been reported with TREX1 mutations. Chilblain lupus is a skin disease characterized by blue-reddish coloring, swelling or ulcers on acral regions of body such as fingertips, heels, nose and auricles...
September 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28918956/healthcare-utilization-and-expenditures-for-united-states-medicare-beneficiaries-with-systemic-vasculitis
#7
Carolyn T Thorpe, Joshua M Thorpe, Tao Jiang, Dylan Atkinson, Yihuang Kang, Loren J Schleiden, Delesha M Carpenter, Julie Anne G McGregor, Susan L Hogan
OBJECTIVE: The Medicare federal insurance program is the most common United States insurer of patients with systemic vasculitis (SV). We compared healthcare utilization and expenditures for Medicare beneficiaries with versus without SV. METHODS: This national, retrospective study used 2010 claims and enrollment data for a 100% cohort of Medicare Part A and B beneficiaries with ≥1 claim including a diagnosis for a form of SV (n = 176,498), and a randomly selected group of non-SV beneficiaries (n = 46,561)...
August 10, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28918917/leukocytapheresis-in-rheumatoid-arthritis
#8
REVIEW
Toshihiko Hidaka, Yayoi Hashiba, Kazuyoshi Kubo, Yasufumi Kai, Keiichi Maeda
In this article, we discussed leukocytapheresis (LCAP) for rheumatoid arthritis (RA). Recently, a simple and practical on-line continuous LACP system has been developed. It is equipped with a direct hemoperfusion column (Cellsorba(®), Asahikasei Medical Co., Ltd.) packed with fine-diameter polyester fibers, which are commonly used to adsorb white blood cells to prevent a graft-versus-host reaction during blood transfusion. Clinical trials revealed that LCAP is a effective and safe therapy for patients with drug-resistant RA or RA complicated with vasculitis...
August 30, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28917219/reuma-pt-contribution-to-the-knowledge-of-immune-mediated-systemic-rheumatic-diseases
#9
Maria José Santos, Helena Canhão, Ana Filipa Mourão, Filipa Oliveira Ramos, Cristina Ponte, Cátia Duarte, Anabela Barcelos, Fernando Martins, José António Melo Gomes
Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making...
September 14, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28917218/erythema-elevatum-diutinum-in-crohn-s-disease-associated-spondyloarthritis-a-rare-vasculitis-an-unusual-association
#10
Maria João Gonçalves, Vasco C Romão, Luís Soares-de-Almeida, Helena Canhão, José Carlos Romeu, Heinz Kutzner, José Alberto Pereira-da-Silva
Erythema elevatum diutinum is a rare neutrophilic dermatoses with vasculitis, which presents as persistent, symmetrical, purple or brownish papules and nodules, mainly in the extensor surface of the limbs. We describe a case of erythema elevatum diutinum and polyarthritis as initial manifestations of Crohn's disease associated spondyloarthritis. A 51-year-old man, from São Tomé e Príncipe, with previous history of treated tuberculosis and chronic hepatitis B infection, was admitted due to 4 months history of polyarthritis, hyperpigmented papules on the extensor surfaces, occasional episodes of bloody mucous diarrhea and significant weight loss...
September 14, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28914375/central-nervous-system-involvement-in-patients-with-granulomatosis-with-polyangiitis-a-single-center-retrospective-study
#11
George E Fragoulis, Sophia Lionaki, Aliki Venetsanopoulou, Panayiotis G Vlachoyiannopoulos, Haralampos M Moutsopoulos, Athanasios G Tzioufas
The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between GPA patients with and without CNS involvement...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28912865/elevated-levels-of-soluble-fractalkine-and-increased-expression-of-cx3cr1-in-neuropsychiatric-systemic-lupus-erythematosus
#12
Ling Guo, Xiaoye Lu, Yuan Wang, Chunde Bao, Shunle Chen
The aim of the present study was to determine the levels of soluble fractalkine (sFKN) and expression of CX3CR1 in neuropsychiatric systemic lupus erythematosus (NPSLE). Disease activity of SLE was assessed using the SLE Disease Activity Index (SLEDAI). The mRNA expression levels of CX3CR1 and FKN were quantified using reverse transcription-quantitative polymerase chain reaction. Levels of sFKN in the serum and cerebrospinal fluid (CSF) were measured using enzyme-linked immunosorbent assays. The mRNA expression levels of CX3CR1 in peripheral blood mononuclear cells from patients with NPSLE, non-NPSLE and Behcet's disease were significantly higher than that of rheumatoid arthritis and healthy persons...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28906413/differences-in-clinical-features-observed-between-childhood-onset-versus-adult-onset-systemic-lupus-erythematosus-a-systematic-review-and-meta-analysis
#13
REVIEW
Pravesh Kumar Bundhun, Alka Kumari, Feng Huang
BACKGROUND: Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis. METHODS: Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28901810/the-incidence-of-ocular-tuberculosis-in-australia-over-the-past-10-years-2006-2015
#14
Erica Darian-Smith, Ming-Lee Lin, Lyndell L Lim, Peter McCluskey, Anthony J Hall
PURPOSE: To determine the incidence and clinical phenotype of ocular tuberculosis in Australia based on the mandatory jurisdictional health notification records for TB. METHODS: A whole population retrospective case series (Australia). Patients diagnosed with ocular tuberculosis were identified over the past 10 years (1 January 2006 to 31 December 2015) as recorded by individual Health Department jurisdictions per mandatory health notifications. The incidence rates were calculated based on the available Australian census data...
September 13, 2017: Ophthalmic Epidemiology
https://www.readbyqxmd.com/read/28900133/fcn1-m-ficolin-which-directly-associates-with-immunoglobulin-g1-is-a-molecular-target-of-intravenous-immunoglobulin-therapy-for-kawasaki-disease
#15
Daisuke Okuzaki, Kaori Ota, Shin-Ichi Takatsuki, Yukari Akiyoshi, Kazuyuki Naoi, Norikazu Yabuta, Tsutomu Saji, Hiroshi Nojima
Kawasaki disease (KD), an acute systemic vasculitis of early childhood, is of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is an effective treatment, but its molecular target remains elusive. DNA microarray analysis of peripheral blood mononuclear cells (PBMCs) revealed that at least 21 genes are drastically down-regulated after IVIG treatment in most KD patients. qRT-PCR analysis confirmed that the mRNA levels of five of these genes were considerably reduced in almost all KD patients after IVIG treatment...
September 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28899799/neutrophil-extracellular-traps-nets-in-autoimmune-diseases-a-comprehensive-review
#16
REVIEW
Keum Hwa Lee, Andreas Kronbichler, David Duck-Young Park, YoungMin Park, Hanwool Moon, Hyungdo Kim, Jun Hyug Choi, YoungSeo Choi, Songjoo Shim, Il Suk Lyu, Byung Hwan Yun, Yeonseung Han, Donghee Lee, Sang Yoon Lee, Byung Hun Yoo, Kyung Hwan Lee, Tai Lim Kim, Heonki Kim, Joo Sung Shim, Wonseok Nam, Heesung So, SooYeon Choi, Sangmok Lee, Jae Il Shin
The structures named neutrophil extracellular traps (NETs) are fibrous networks which protrude from the membrane of activated neutrophils. NETs are found in a variety of conditions, such as infection, malignancy, atherosclerosis, and autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), psoriasis, and gout. The impact of NETs on the development mechanisms of autoimmune diseases are proposed to arise from an imbalance between "NETosis" which is a process of NET formation and NET degradation...
September 9, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28895653/clinical-interventions-for-takayasu-arteritis-a-systematic-review
#17
REVIEW
Rafael L Pacheco, Carolina de Oliveira Cruz Latorraca, Alexandre Wagner Silva de Souza, Daniela V Pachito, Rachel Riera
INTRODUCTION: Takayasu arteritis (TA) is a rare systemic vasculitis that affects large vessels often resistant to treatment and associated with high morbidity and mortality. Treatment is defied by the relapsing nature of the disease and frequent adverse effects of corticosteroids and immunosuppressors, rendering failure of treatment in a significant portion of patients. Considering the low quantity and quality of published studies focusing on treatment of TA, synthesis and critical assessment of the available evidence is fundamental to establish recommendations for clinical practice...
September 12, 2017: International Journal of Clinical Practice
https://www.readbyqxmd.com/read/28891482/encephalopathy-in-henoch-sch%C3%A3-nlein-purpura
#18
Huijun Shen, Jianhua Mao, Qiang Shu, Lizhong Du
BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP. CASE CHARACTERISTICS: Three children with features of HSP presented with seizures and CNS dysfunction. OBSERVATION: All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage. MESSAGE: HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment...
August 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28889017/mycoplasmas-brain-invaders
#19
REVIEW
Rubén S Rosales, Roberto Puleio, Guido R Loria, Salvatore Catania, Robin A J Nicholas
Mycoplasmas of humans and animals are usually associated with respiratory, autoimmune, genital and joint diseases. Human mycoplasmas have also been known to affect the brain. Severe central nervous system (CNS) diseases, such as encephalitis, have been linked to Mycoplasma pneumoniae and ureaplasma infections. Less well known is the sheep and goat pathogen, Mycoplasma agalactiae, which has been found in large quantities in the brain where it may be responsible for non-purulent encephalitis as well as ataxia in young animals...
September 5, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28886140/male-specific-association-of-the-fcgr2a-his167arg-polymorphism-with-kawasaki-disease
#20
Young-Chang Kwon, Jae-Jung Kim, Sin Weon Yun, Jeong Jin Yu, Kyung Lim Yoon, Kyung-Yil Lee, Hong-Ryang Kil, Gi Beom Kim, Myung-Ki Han, Min Seob Song, Hyoung Doo Lee, Kee-Soo Ha, Sejung Sohn, Ryota Ebata, Hiromichi Hamada, Hiroyuki Suzuki, Kaoru Ito, Yoshihiro Onouchi, Young Mi Hong, Gi Young Jang, Jong-Keuk Lee
Kawasaki disease (KD) is an acute systemic vasculitis that can potentially cause coronary artery aneurysms in some children. KD occurs approximately 1.5 times more frequently in males than in females. To identify sex-specific genetic variants that are involved in KD pathogenesis in children, we performed a sex-stratified genome-wide association study (GWAS), using the Illumina HumanOmni1-Quad BeadChip data (249 cases and 1,000 controls) and a replication study for the 34 sex-specific candidate SNPs in an independent sample set (671 cases and 3,553 controls)...
2017: PloS One
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