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Systemic vasculitis

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https://www.readbyqxmd.com/read/28444097/chronic-polyarthritis-as-the-first-manifestation-of-childhood-systemic-polyarteritis-nodosa
#1
Glaucia Vanessa Novak, Koken Hayashi, Kohei Sampa, Yosuke Okumura, Gabriela Ribeiro Viola Ferreira, Clovis Artur Silva
Arthritis has been reported as an acute pattern, generally evanescent with oligoarthritis, mostly affecting knees and ankles in childhood systemic polyarteritis nodosa. However, chronic polyarthritis with morning stiffness mimicking juvenile idiopathic arthritis has not been reported. We describe the case of a 4-year old girl who had additive and chronic polyarthritis with edema, tenderness, pain on motion and morning stiffness for 2 months. After 45 days, she also presented painful subcutaneous nodules and erythematous-violaceous lesions in the extensor region of upper and lower limbs...
January 2017: Einstein
https://www.readbyqxmd.com/read/28442517/red-blood-cell-distribution-width-and-neutrophil-to-lymphocyte-ratio-in-patients-with-cutaneous-vasculitis
#2
Nazan Emiroglu, Fatma Pelin Cengiz, Anıl Gulsel Bahalı, Dilek Biyik Ozkaya, Ozlem Su, Nahide Onsun
BACKGROUND: Vasculitis represents a specific pattern of inflammation of the blood vessel wall that can occur in any organ system of the body. The neutrophil to lymphocyte ratio (NLR) and red blood cell distribution width (RDW) are currently used as markers of inflammation in several diseases. OBJECTIVES: This study analyzed C-reactive protein level (CRP), erythrocyte sedimentation rate (ESR), white blood cell (WBC), NLR, and RDW in patients who had cutaneous vasculitis, or cutaneous vasculitis with systemic involvement, and in healthy controls...
March 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28440998/-extranodal-nk-t-cell-lymphoma-nasal-type-in-granulomatosis-with-polyangiitis-a-case-report
#3
Sergio Alberto Mendoza-Álvarez, Fátima Margarita Rodríguez-Dávila, Leslie Moranchel-García, Virginia Soto, Natalia Quisped
BACKGROUND: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. CLINICAL CASE: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection...
May 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28440408/identification-of-potential-gene-targets-in-systemic-vasculitis-using-dna-microarray-analysis
#4
Yiwen Feng, Miao Zheng, Shujie Gan, Lei Zhang, Zhong Wan, Yanping Zhang, Qin Qian, Jingdong Tang
The present study aimed to identify the involvement of critical genes in systemic vasculitis, to gain an improved understanding of the molecular circuity and to investigate novel potential gene targets for systemic vasculitis treatment. The dual‑color cDNA microarray data of GSE16945, consisting of peripheral mononuclear blood cell specimens from 13 patients with systemic vasculitis and 16 healthy controls, was downloaded from the Gene Expression Omnibus database. Differentially expressed genes (DEGs) were screened in systemic vasculitis compared with controls using BRB ArrayTools, followed by the construction of a protein‑protein interaction (PPI) network using the clusterProfiler package, and significant functional interaction (FI) module selection...
April 11, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28431717/chronic-periaortitis-of-ascending-aorta-with-bilateral-ostial-coronary-stenosis-in-a-young-female
#5
Gauranga Majumdar, Aditya Kapoor, Surendra K Agarwal, Navneet Srivastava, Ram Nawal Rao, Neetu Soni
An 18-year-old woman without any risk factors for coronary artery disease or systemic vasculitis and infection presented with recurrent angina at rest. Coronary angiography revealed 100% occlusion of the ostial left main coronary artery and severe ostial right coronary artery stenosis. She underwent successful coronary artery bypass graft surgery. At surgery, the external surface of the ascending aorta was covered with a soft-tissue thickened mass; histopathology of the mass revealed chronic nonspecific inflammatory aortitis...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28426486/neutrophilic-figurate-erythema
#6
Yu-Hung Wu, Pa-Fan Hsiao
Neutrophilic figurate erythema (NFE) has been rarely reported. This study aimed to identify the clinical and pathological features of NFE. We retrospectively reviewed the information from diagnostic cases from 2000 to 2013. The diagnosis of NFE includes clinically annular rash, histopathologically predominant neutrophilic perivascular and interstitial infiltrate in the dermis without evidence of vasculitis, and exclusion of other known specific entities. Fifteen cases of NFE were identified, including 11 women and 4 men...
May 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28421878/cerebrovascular-injury-in-cryptococcal-meningitis
#7
Ajay Kumar Mishra, Vanjare Harshad Arvind, Divya Muliyil, Cijoy K Kuriakose, Anu Anna George, Reka Karuppusami, Ronald Albert Benton Carey, Sunithi Mani, Samuel George Hansdak
Background Cryptococcal meningitis continues to be one of the common causes of chronic central nervous system infection worldwide. Individuals with cryptococcal meningitis can occasionally present with small vessel vasculitis causing infarcts primarily in the basal ganglia, internal capsule, and thalamus. Literature regarding patterns of cerebrovascular injury among patients with cryptococcal meningitis is scanty, and outcome following these vascular involvements is unknown. Aim To study the clinical profile, imaging findings, and details of vascular territory involved among patients admitted with cryptococcal meningitis and central nervous system infarct in a tertiary care center from India...
January 1, 2017: International Journal of Stroke: Official Journal of the International Stroke Society
https://www.readbyqxmd.com/read/28421357/incidence-of-autoimmune-diseases-in-patients-with-scabies-a-nationwide-population-based-study-in-taiwan
#8
Jui-Ming Liu, Feng-Hsiang Chiu, Chien-Yu Lin, Fung-Wei Chang, Ren-Jun Hsu
Scabies is a commonly occurring infectious immune-mediated inflammatory skin disease. Immune-mediated inflammatory processes are also observed in autoimmune diseases. There have been very few previous studies; however, that have investigated the possible association between scabies and autoimmune diseases. To address this research gap, we conducted a nationwide population-based cohort study that included a total of 4481 scabies patients and 16,559 control subjects matched by gender, age, insured region, urbanization and income...
April 18, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#9
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28420057/central-nervous-system-vasculitis-in-systemic-lupus-erythematosus-a-case-series-report-in-a-tertiary-referral-centre
#10
M Rodrigues, O Galego, C Costa, D Jesus, P Carvalho, M Santiago, A Malcata, L Inês
Central nervous system (CNS) vasculitis (CNS) in systemic erythematosus lupus (SLE) is a rare and challenging diagnosis. We report four cases of CNS vasculitis that occurred 5 to 16 years after the diagnosis of SLE. Magnetic resonance imaging (MRI) detected different features suggestive of CNS vasculitis: enhancement and thickening of the vascular wall, vascular stenosis, ischemic brain lesions and intracerebral haemorrhage unlikely to correspond to other mimic aetiologies. Three patients received combination therapy with glucocorticoids (GC) and cyclophosphamide (CYC)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28417923/anemia-in-kawasaki-disease-hepcidin-as-a-potential-biomarker
#11
REVIEW
Ying-Hsien Huang, Ho-Chang Kuo
Kawasaki disease (KD) is an autoimmune-like disease and acute childhood vasculitis syndrome that affects various systems but has unknown etiology. In addition to the standard diagnostic criteria, anemia is among the most common clinical features of KD patients and is thought to have a more prolonged duration of active inflammation. In 2001, the discovery of a liver-derived peptide hormone known as hepcidin began revolutionizing our understanding of anemia's relation to a number of inflammatory diseases, including KD...
April 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28416004/clinical-study-of-children-with-takayasu-arteritis-a-retrospective-study-from-a-single-center-in-china
#12
Ye Feng, Xuemei Tang, Mingyue Liu, Juan Zhou, Xiaodong Zhao, Qiu Li
BACKGROUND: Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. METHODS: Eleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28413538/primary-cns-vasculitis-masquerading-as-glioblastoma-a-case-report-and-review
#13
Pelluru Pavan Kumar, Alugolu Rajesh, Rukmini Mrudula Kandadai, Aniruddh Kumar Purohit, Challa Sundaram
Isolated angitis of the central nervous system (IACNS)/primary angitis of central nervous system vasculitis (PACNS) is an uncommon vascular disease, sparingly presenting as an isolated inflammatory lesion on magnetic resonance imaging (MRI). The disease usually manifests as a long-drawn and progressive ischemic event. Delay in diagnosis due to focal nature of the lesion also contributes to the poor prognosis as the dismal natural history and immunosuppressive therapy. To date, only a few cases with tumor-like isolated angitis of CNS have been reported with clear and definitive diagnostic workup...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28412068/vitreous-amyloidosis-ocular-systemic-and-genetic-insights
#14
Pradeep Venkatesh, Harathy Selvan, Sundararajan Baskar Singh, Divya Gupta, Seema Kashyap, Shreyas Temkar, Varun Gogia, Koushik Tripathy, Rohan Chawla, Rajpal Vohra
PURPOSE: To report the unique clinical and surgical characteristics encountered in eyes with vitreous amyloidosis. Systemic evaluation and visual outcome after vitrectomy are discussed. A novel mutation in the transthyretin gene (TTR) in Indian patients with familial amyloid polyneuropathy (FAP) is described. DESIGN: Retrospective, observational study. PARTICIPANTS: Ten eyes of 5 patients from 2 pedigrees with a diagnosis of vitreous amyloidosis...
April 12, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28410693/clinical-and-radiological-spectrum-of-intracranial-tuberculosis-a-hospital-based-study-in-northeast-india
#15
Baiakmenlang Synmon, Marami Das, Ashok K Kayal, Munindra Goswami, Jogesh Sarma, Lakshya Basumatary, Suvorit Bhowmick
Central nervous system tuberculosis (TB) is the most severe extra pulmonary TB having a high mortality and morbidity. OBJECTIVE: To study the various clinical, biochemical, and radiological spectrum of intracranial TB. MATERIALS AND METHOD: Ninety-three patients were enrolled in this prospective study after ethical clearance and consent from August 2013 to May 2015. The entire clinical course with complications and predictors of mortality were assessed. RESULTS: 36 females (38...
April 2017: Indian Journal of Tuberculosis
https://www.readbyqxmd.com/read/28410605/frosted-branch-angiitis-and-cerebral-venous-sinus-thrombosis-as-an-initial-onset-of-neuro-beh%C3%A3-et-s-disease-a-case-report-and-review-of-the-literature
#16
Bruno Fortaleza de Aquino Ferreira, Ever Ernesto Caso Rodriguez, Leandro Lara do Prado, Celio Roberto Gonçalves, Carlos Eduardo Hirata, Joyce Hisae Yamamoto
BACKGROUND: Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Diagnosis of Behçet's disease was based on the current 2014 International Criteria for Behçet's Disease and the International consensus recommendation criteria for neuro-Behçet's disease...
April 15, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#17
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28409662/association-of-endothelial-nitric-oxide-synthase-gene-polymorphism-with-susceptibility-and-nephritis-development-of-henoch-sch%C3%A3-nlein-purpura-in-chinese-han-children
#18
Ailing Wang, Aili Wang, Yanfeng Xiao, Jingjing Wang, Erdi Xu
OBJECTIVE: Henoch-Schönlein purpura (HSP) is the most common form of systemic small-vessel vasculitis in children. Previous studies suggested endothelial nitric oxide synthase (eNOS) plays an important role in the pathogenesis and clinical manifestations of HSP. This study aimed to investigate the potential association between 10 single-nucleotide polymorphisms (SNPs) within eNOS gene and HSP risk and nephritis development in a Chinese Han population. MATERIALS AND METHODS: A case-control study was conducted including 459 healthy children and 423 children with HSP...
April 14, 2017: Genetic Testing and Molecular Biomarkers
https://www.readbyqxmd.com/read/28407841/cardiovascular-comorbidity-in-inflammatory-rheumatological-conditions
#19
Jürgen Braun, Klaus Krüger, Bernhard Manger, Matthias Schneider, Christof Specker, Hans Joachim Trappe
BACKGROUND: Approximately 1.5 million adults in Germany suffer from an inflammatory rheumatological condition. The most common among these are rheumatoid arthritis and spondyloarthritis-above all axial spondyloarthritis, including ankylosing spondylitis (Bekhterev's disease) and psoriatic arthritis. These systemic inflammatory diseases often affect the heart as well. METHODS: This review is based on pertinent articles retrieved by a selective literature search, on current European guidelines, and on the authors' clinical experience...
March 24, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28406765/maintenance-treatment-of-anca-associated-vasculitides
#20
REVIEW
Loic Guillevin
ANCA-associated vasculitis(AAV)covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined...
April 5, 2017: Clinical and Experimental Rheumatology
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