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Systemic vasculitis

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https://www.readbyqxmd.com/read/28339745/chronic-nasal-staphylococcus-aureus-carriage-identifies-a-subset-of-newly-diagnosed-granulomatosis-with-polyangiitis-patients-with-high-relapse-rate
#1
Anna Salmela, Niels Rasmussen, Jan Willem Cohen Tervaert, David R W Jayne, Agneta Ekstrand
Objective.: The aim of this study was to evaluate whether chronic nasal carriage of Staphylococcus aureus (SA) is related to relapses in patients with newly diagnosed ANCA-associated vasculitis (AAV). Methods.: In two clinical trials (n = 200), for early systemic (n = 83) and generalized (n = 117) AAV, nasal swabs were obtained monthly and at the time of a relapse. Chronic nasal SA carriage (CNSAC) was defined as ⩾ 75% of cultures being SA positive, with non-carriers being SA negative in all cultures and remaining patients being intermittent carriers...
February 20, 2017: Rheumatology
https://www.readbyqxmd.com/read/28339364/clinical-and-prognostic-features-of-korean-patients-with-mpo-anca-pr3-anca-and-anca-negative-vasculitis
#2
Juyoung Yoo, Ho Jae Kim, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA negative vasculitis, and investigated clinical and prognostic features. METHODS: We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had having either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission...
March 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28334541/-autoantibodies-in-systemic-connective-tissue-disease-and-anca-associated-vasculitis-their-relationship-to-interstitial-lung-diseases-and-prognosis
#3
Martina Doubková, Jana Pokorná
Lung disease, including interstitial lung disease (ILD), is a frequent complication of systemic connective tissue disorders (CTD) and ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis (AAV). Pulmonary manifestations are prognostic factor of CTDs and vasculitis. Autoantibodies assessment is a part of differential diagnosis algorithm of lung diseases. Autoantibodies importance is mainly clinical-diagnostic. Using detection of some autoantibodies it is possible to determine prognosis of lung involvement, especially in CTDs...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28330942/primary-angiitis-of-the-central-nervous-system-magnetic-resonance-imaging-spectrum-of-parenchymal-meningeal-and-vascular-lesions-at-baseline
#4
Grégoire Boulouis, Hubert de Boysson, Mathieu Zuber, Loïc Guillevin, Eric Meary, Vincent Costalat, Christian Pagnoux, Olivier Naggara
BACKGROUND AND PURPOSE: Primary angiitis of the central nervous system remains challenging. To report an overview and pictorial review of brain magnetic resonance imaging findings in adult primary angiitis of the central nervous system and to determine the distribution of parenchymal, meningeal, and vascular lesions in a large multicentric cohort. METHODS: Adult patients from the French COVAC cohort (Cohort of Patients With Primary Vasculitis of the Central Nervous System), with biopsy or angiographically proven primary angiitis of the central nervous system and brain magnetic resonance imaging available at the time of diagnosis were included...
March 22, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28329555/dermatomyositis-clinically-presenting-with-cutaneous-ulcers-with-histopathologic-evidence-of-perforating-collagenosis
#5
Rachel Rosenstein, Kathryn Martires, Mitalee Christman, Vitaly Terushkin, Shane A Meehan, Nicole Seminara, Brian D Golden, Andrew G Franks
Dermatomyositis is a systemic, autoimmune diseasewith a variety of clinical features that often includemyositis and characteristic cutaneous findings. Asubset of patients with dermatomyositis developcutaneous ulcers, often in the setting of vasculitis orvasculopathy. We present a case of dermatomyositiswith cutaneous ulcers that show perforatingcollagenosis on histopathologic examination.Acquired reactive perforating collagenosistypically occurs in the setting of diabetes mellitus,chronic renal failure, and other pruritic conditions,and this case represents a rare association withdermatomyositis, which may ultimately be helpful inelucidating the pathophysiology of this perforatingdisorder...
December 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28328827/clinical-study-on-single-organ-cutaneous-small-vessels-vasculitis-socsvv
#6
Maciej Pastuszczak, Magdalena Celińska-Löwenhoff, Joanna Sułowicz, Anna Wojas-Pelc, Jacek Musiał
Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28324788/primary-central-nervous-system-vasculitis-and-its-mimicking-diseases-clinical-features-outcome-comorbidities-and-diagnostic-results-a-case-control-study
#7
J Becker, P A Horn, K Keyvani, I Metz, C Wegner, W Brück, F M Heinemann, J C Schwitalla, P Berlit, M Kraemer
OBJECTIVES: To compare clinical features and outcome, imaging characteristics, biopsy results and laboratory findings in a cohort of 69 patients with suspected or diagnosed primary central nervous system vasculitis (PCNSV) in adults; to identify risk factors and predictive features for PCNSV. PATIENTS AND METHODS: We performed a case-control-study including 69 patients referred with suspected PCNSV from whom 25 were confirmed by predetermined diagnostic criteria based on biopsy (72%) or angiography (28%)...
March 8, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28322578/interview-with-amr-h-sawalha-epigenetics-and-autoimmunity
#8
Amr H Sawalha
Amr H Sawalha is Professor of Internal Medicine and Marvin and Betty Danto Research Professor of Connective Tissue Research at the University of Michigan, Department of Internal Medicine, Division of Rheumatology. He also holds faculty appointments at the Center for Computational Medicine and Bioinformatics and the Graduate Program in Immunology at the University of Michigan. He was recently appointed as Guest Professor at Central South University in Changsha, China. He received his medical degree from Jordan University of Science and Technology and completed his residency training in internal medicine at the University of Oklahoma Health Sciences Center, and his fellowship in rheumatology at the University of Michigan...
March 21, 2017: Epigenomics
https://www.readbyqxmd.com/read/28321351/bilateral-birdshot-retinochoroiditis-and-retinal-astrocytoma
#9
Sunil Mamtora, Yun Wong, Dugald Bell, Teresa Sandinha
Background. This case highlights the importance of recognising multiple pathologies within the eye which may not necessarily be linked. Both birdshot retinochoroiditis and astrocytoma are rare conditions. The case underlines the need for early identification and treatment of birdshot retinochoroiditis with steroids and disease modifying drugs. Astrocytoma in the absence of tuberous sclerosis is also uncommon. Case Presentation. A 36-year-old male presented with 3-month history of bilateral progressive flashing lights and floaters...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28321078/helicobacter-cinaedi-bacteremia-mimicking-a-flare-of-systemic-lupus-erythematosus
#10
Ruriko Nishida, Nobuyuki Shimono, Noriko Miyake, Yong Chong, Shinji Shimoda, Hiroshi Tsukamoto, Koichi Akashi
A 40-year-old woman with systemic lupus erythematosus (SLE) presented with high-grade fever and severe thrombocytopenia. Acalculous cholecystitis and thrombocytopenia were initially suspected to be complicated with SLE and vasculitis. Contrary to our expectation, however, the patient was finally diagnosed with Helicobacter cinaedi bacteremia. SLE patients show various symptoms, especially when their condition is complicated with vasculitis, which mimics H. cinaedi bacteremia. It is therefore difficult to provide a definite diagnosis...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#11
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28316335/complement-in-anca-associated-vasculitis-mechanisms-and-implications-for-management
#12
REVIEW
Min Chen, David R W Jayne, Ming-Hui Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of potentially life-threatening autoimmune diseases. The main histological feature in the kidneys of patients with AAV is pauci-immune necrotizing crescentic glomerulonephritis with little immunoglobulin and complement deposition in the glomerular capillary walls. The complement system was not, therefore, initially thought to be associated with the development of AAV. Accumulating evidence from animal models and clinical observations indicate, however, that activation of the complement system - and the alternative pathway in particular - is crucial for the development of AAV, and that the complement activation product C5a has a central role...
March 20, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28298242/migratory-large-vessel-vasculitis-preceding-acute-myeloid-leukemia-a-case-report
#13
Dinusha Chandratilleke, Anthea Anantharajah, Mauro Vicaretti, Warwick Benson, Lucinda J Berglund
BACKGROUND: Large vessel vasculitis is a rare disorder usually occurring in the context of the autoimmune conditions of giant cell arteritis and Takayasu's arteritis. Case reports have described large vessel vasculitis occurring in individuals with myelodysplastic syndrome, preceding transformation to acute myeloid leukemia. CASE PRESENTATION: A 56-year-old Afghanistan-born woman presented with fever, a tender left carotid artery, and raised inflammatory markers...
March 16, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28293454/can-rheumatoid-vasculitis-predate-a-diagnosis-of-rheumatoid-arthritis
#14
Sarah Sacks, Alan Steuer
We report regarding a male patient who presented with a systemic vasculitis that was consistent with a diagnosis of polyarteritis nodosa. At presentation, he had no features of inflammatory arthritis but had a high rheumatoid factor titer and low C4 level. Withdrawal of immunosuppression after 6 years resulted in the development of classical rheumatoid arthritis (RA). This case supports previous reports that revealed that vasculitis may predate the development or occur very early in the course of articular RA...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28290145/successful-surgical-intervention-for-rectal-perforation-due-to-polyarteritis-nodosa-report-of-a-case
#15
Keiji Yoshiya, Yu Imamura, Yu Nakaji, Daisuke Taniguchi, Rinne Takeda, Koji Ando, Yuichiro Nakashima, Motohiro Shimizu, Kippei Ohgaki, Norihiro Furusyo, Takuya Matsumoto, Hiroshi Saeki, Yoshinao Oda, Eiji Oki, Yoshihiko Maehara
BACKGROUND: Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis with diffuse organ involvements, resulting in a high mortality rate due to multiple organ failure. Although the small bowel is the frequently targeted organ of PAN-associated vasculitis, rectal involvement is very rare, and only one case of rectal bleeding has been previously reported. The mortality rate of PAN with gastrointestinal (GI) perforation is reportedly much higher than that of without severe GI involvement...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28289183/c1-inhibitor-decreases-the-release-of-vasculitis-like-chemotactic-endothelial-microvesicles
#16
Maria Mossberg, Anne-Lie Ståhl, Robin Kahn, Ann-Charlotte Kristoffersson, Ramesh Tati, Caroline Heijl, Mårten Segelmark, L M Fredrik Leeb-Lundberg, Diana Karpman
The kinin system is activated during vasculitis and may contribute to chronic inflammation. C1-inhibitor is the main inhibitor of the kinin system. In this study, we investigated the presence of the kinin B1 receptor on endothelial microvesicles and its contribution to the inflammatory process. Compared with controls (n=15), patients with acute vasculitis (n=12) had markedly higher levels of circulating endothelial microvesicles, identified by flow cytometry analysis, and significantly more microvesicles that were positive for the kinin B1 receptor (P<0...
March 13, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28285286/necrotizing-polyarteritis-nodosa-like-vasculitis-in-a-child-with-systemic-lupus-erythematosus
#17
Ritambhra Nada, Joseph L Matthews, Sagar Bhattad, Anju Gupta, Surjit Singh
A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema...
February 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28281443/central-nervous-system-tuberculosis
#18
John M Leonard
Central nervous system tuberculosis (CNS-TB) takes three clinical forms: meningitis (TBM), intracranial tuberculoma, and spinal arachnoiditis. TBM predominates in the western world and presents as a subacute to chronic meningitis syndrome with a prodrome of malaise, fever, and headache progressing to altered mentation and focal neurologic signs, followed by stupor, coma, and death within five to eight weeks of onset. The CSF formula typically shows a lymphocytic pleocytosis, and low glucose and high protein concentrations...
March 2017: Microbiology Spectrum
https://www.readbyqxmd.com/read/28273992/anca-associated-mononeuritis-multiplex-with-overlap-in-vasculitic-syndromes
#19
Ravi Anadure, Coimbatore Narayanan, Govindraj Varadraj, Bevinahalli Nandeesh
Mononeuritis multiplex is a common manifestation of many illnesses which includes Hansen's disease and certain types of systemic vasculitis. The Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). We describe a patient who presented with mononeuritis multiplex and had features of overlap between EGPA and MPA...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28273603/rare-presentation-of-wegener-s-granulomatosis-in-the-pituitary-gland-case-report-and-literature-review
#20
Samantha M Baird, Upasna Pratap, Catriona McLean, Candice P Law, Nicholas Maartens
INTRODUCTION: Wegener's granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system. PRESENTATION OF CASE: A 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland...
February 20, 2017: International Journal of Surgery Case Reports
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