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https://www.readbyqxmd.com/read/28544838/granulomatosis-with-polyangiitis-presenting-with-acute-aortic-and-mitral-regurgitation-case-report-and-big-data-analysis
#1
Sadeer G Al-Kindi, M Amer Al-Aiti, Michael Yang, Richard A Josephson
Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can have multisystem involvement, though cardiac involvement is very rare. The case is described of a 53-year-old woman who presented with acute aortic and mitral valve regurgitation requiring surgical intervention. Pathology from the excised aortic valve showed geographic necrosis concerning for GPA. Subsequent rheumatologic testing was positive for anti-serine proteinase 3 (PR3) antibody, consistent with GPA. A year after the valve surgery the patient was found to have a vegetation of the mitral valve and elevated PR3 antibody levels, and was successfully treated with an intensification of immunosuppression...
January 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28543916/hcv-rna-genomic-sequences-and-hcv-e2-glycoprotein-in-sural-nerve-biopsies-from-hcv-infected-patients-with-peripheral-neuropathy
#2
Sabino Russi, Domenico Sansonno, Salvatore Monaco, Sara Mariotto, Sergio Ferrari, Fabio Pavone, Gianfranco Lauletta, Franco Dammacco
AIMS: Peripheral neuropathy (PN), the major neurological complication of chronic HCV infection, is frequently associated with mixed cryoglobulinemia (MC) and small-vessel systemic vasculitis. While humoral and cell-mediated immune mechanisms are suspected to act together in an aberrant immune response that results in peripheral nerve damage, the role of HCV remains largely speculative. The possible demonstration of HCV in peripheral nerve tissue would obviously assume important pathogenic implications...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28541581/a-multicentre-study-to-improve-clinical-interpretation-of-proteinase-3-and-myeloperoxidase-anti-neutrophil-cytoplasmic-antibodies
#3
Xavier Bossuyt, Niels Rasmussen, Pieter van Paassen, Bernard Hellmich, Bo Baslund, Pieter Vermeersch, Daniel Blockmans, Jan-Willem Cohen Tervaert, Elena Csernok, Jan Damoiseaux
Objective.: The objective of this multicentre study was to improve the clinical interpretation of PR3- and MPO-ANCAs as an adjunct for the diagnosis of ANCA-associated vasculitis (AAV) by defining thresholds and test result intervals based on predefined specificities and by calculating test result interval-specific likelihood ratios (LRs). Methods.: Eight different PR3- and MPO-ANCA immunoassays from seven companies were evaluated using 251 diagnostic samples from AAV patients and 924 diseased controls...
May 24, 2017: Rheumatology
https://www.readbyqxmd.com/read/28540906/an-overlap-of-granulomatosis-with-polyangiitis-and-eosinophilic-granulomatosis-with-polyangiitis
#4
Sujit Surendran, Chandramohan Gundappa, Arun Gandhi, Anila Abraham Kurien, Edwin Fernando
We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA)...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540625/pituitary-dysfunction-in-granulomatosis-with-polyangiitis
#5
REVIEW
Daniela Esposito, Penelope Trimpou, Dario Giugliano, Mats Dehlin, Oskar Ragnarsson
PURPOSE: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). METHODS: A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed...
May 24, 2017: Pituitary
https://www.readbyqxmd.com/read/28539558/a-case-of-new-onset-rheumatoid-vasculitis-becoming-evident-in-the-course-of-treatment-for-pneumocystis-jirovecii-pneumonia
#6
Daisuke Ikuma, Kazuhiro Yokota, Kojiro Sato, Toshihide Mimura
  When patients with autoimmune diseases, such as rheumatoid arthritis (RA), are treated with potent immunosuppressive therapy, the risk of opportunistic diseases inevitably increases. If patients have the misfortune to suffer from both opportunistic and active autoimmune diseases, correct diagnosis could sometimes be difficult since both diseases have inflammatory nature. The choice of treatment is another challenge in that aggressive immunosuppressive therapy can fuel the opportunistic infection. Here we report a case of RA patient with new onset rheumatoid vasculitis that was diagnosed in the process of treatment of Pneumocystis jirovecii pneumonia...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28539536/remission-induction-therapy-with-rituximab-for-microscopic-polyangiitis-a-feasibility-study
#7
Ayako Saito, Yoichi Takeuchi, Saeko Kagaya, Yoshie Ojima, Hirotaka Fukami, Hiroyuki Sato, Ken Matsuda, Tasuku Nagasawa
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is systemic vascular inflammation. Microscopic polyangiitis (MPA) is a major type of AAV in Japan. MPA often affects the kidneys and lungs, leading to death if untreated. Induction therapy (i.e., initial treatment) for MPA has not been optimized, although methylprednisolone and cyclophosphamide are commonly used. Recently, rituximab (RTX) (a monoclonal antibody against the protein CD20) has also been used to treat refractory AAV. RTX at 375 mg/m(2)/week for 4 weeks (i...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28538395/palisaded-neutrophilic-and-granulomatous-dermatitis-as-a-novel-cause-of-hypercalcemia-a-case-report
#8
Michihito Kono, Tomoka Hasegawa, So Nagai, Toshio Odani, Kazumasa Akikawa, Yukiko Nomura, Hidetsugu Sato, Keisuke Kikuchi, Norio Amizuka, Hideaki Kikuchi
RATIONALE: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a benign, inflammatory dermatosis with distinct histopathological features often observed in patients with systemic diseases. There were no reports of PNGD without underlying systemic diseases as an underlying cause of hypercalcemia. Herein, we report a case of a 62-year-old man with hypercalcemia due to PNGD, but with no underlying systemic diseases, including tuberculosis, sarcoidosis, or vasculitis. PATIENT CONCERNS: Laboratory tests showed an elevated C-reactive protein level, an elevated corrected calcium level, a normal 25-hydroxyvitamin D level, and an elevated 1,25-dihydroxyvitamin D level...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28538210/correlation-of-hamp-gene-polymorphisms-and-expression-with-the-susceptibility-and-length-of-hospital-stays-in-taiwanese-children-with-kawasaki-disease
#9
Ying-Hsien Huang, Kuender D Yang, Yu-Wen Hsu, Hsing-Fang Lu, Henry Sung-Ching Wong, Hong-Ren Yu, Hsing-Chun Kuo, Fu-Chen Huang, Mao-Hung Lo, Kai-Sheng Hsieh, Su-Fen Chen, Wei-Chiao Chang, Ho-Chang Kuo
Kawasaki disease (KD) is a form of systemic vasculitis. Regarding its pathogenesis, HAMP gene encoding hepcidin, which is significant for iron metabolism, has a vital function. In this study, we recruited a total of 381 KD patients for genotyping. Data from 997 subjects (500 subjects from cohort 1; 497 subjects from cohort 2) were used for analysis. Using TaqMan allelic discrimination, we determined five tag SNPs (rs916145, rs10421768, rs3817623, rs7251432, and rs2293689). Treatment outcome data related to such clinical phenotypes as coronary artery lesions (CAL), coronary artery aneurysms (CAA), and intravenous immunoglobulin (IVIG) effects were also collected...
May 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28538036/multiple-cranial-nerve-palsies-in-giant-cell-arteritis
#10
Michael Ross, Lulu Bursztyn, Rosanne Superstein, Mark Gans
Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries often with ophthalmic involvement, including ischemic optic neuropathy, retinal artery occlusion, and ocular motor cranial nerve palsies. This last complication occurs in 2%-15% of patients, but typically involves only 1 cranial nerve. We present 2 patients with biopsy-proven GCA associated with multiple cranial nerve palsies.
May 18, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28537941/pathophysiology-of-anca-associated-vasculitis
#11
Turki Al-Hussain, Maged H Hussein, Walter Conca, Hadeel Al Mana, Mohammed Akhtar
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized as inflammation of small-sized to medium-sized blood vessels and encompasses several clinicopathologic entities including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited ANCA-associated vasculitis. Over the past several decades, significant progress has been made in understanding the pathophysiology of ANCA-associated vasculitis. Although neutrophils contain a multitude of granular proteins, clinically significant autoantibodies are only recognized against myeloperoxidase and proteinase 3, both of which are present in the azurophilic granules...
May 22, 2017: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/28536934/-off-label-biological-therapies-in-patients-with-large-vessel-vasculitis-and-or-polymyalgia-rheumatica-safety-and-efficacy-analysis-of-a-nationwide-german-registry-graid2
#12
J C Henes, H Schulze-Koops, M Witt, H P Tony, F Mueller, M Grunke, M Czihal, T Dörner, F Proft
OBJECTIVE: To evaluate the safety and clinical outcome of biological therapies in patients with large vessel vasculitis (LVV) or polymyalgia rheumatica (PMR) refractory to standard of care therapy in a real-life setting in Germany. METHODS: GRAID 2 (German Registry in Autoimmune Diseases 2) is a retrospective, noninterventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy treated with an initial off-label biological between August 2006 and December 2013...
May 23, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28536920/clinicopathological-features-of-multiple-mononeuropathy-associated-with-systemic-lupus%C3%A2-erythematosus-a-multicenter-study
#13
Elodie Rivière, Fleur Cohen Aubart, Thierry Maisonobe, François Maurier, Christophe Richez, Bruno Gombert, Marie Gousseff, Daniel Adoue, Alexis Mathian, Miguel Hié, Julien Haroche, Zahir Amoura
Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years)...
May 23, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28535620/a-case-of-false-positive-troponin-i-in-a-patient-affected-by-cryoglobulinemic-vasculitis
#14
M Bellan, M Pirisi, G Bellomo, P P Sainaghi
Troponin I (TnI) false positive results have been reported in patients affected by immune disorders. We report the case of a 74-year-old woman affected by cryoglobulinemic vasculitis, admitted to the Emergency Room because of a lipotimic episode. A marked elevation of TnI plasma concentration was confirmed in multiple determinations, despite the absence of symptoms or electrocardiogram findings suggesting myocardial infarction. TnI plasma concentration was reported normal after re-testing with a different commercial kit...
May 22, 2017: Reumatismo
https://www.readbyqxmd.com/read/28533861/-cutaneous-leukocytoclastic-vasculitis-about-85-cases
#15
Amina Aounallah, Aicha Arouss, Najet Ghariani, Wafa Saidi, Badreddine Sriha, Mohamed Denguezli, Colandane Belajouza, Rafia Nouira
Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28533099/surgical-aortic-valve-replacement-following-early-sapien-xt-valve-failure-a-first
#16
Tanveer Ahmad, Prakash Ludhani, Ronen Gurvitch, John Goldblatt, James Tatoulis
BACKGROUND: Early degeneration of prosthetic aortic valve in transcatheter aortic valve replacement (TAVR) is a rare complication. METHOD: We report the case of a 75-year-old woman who presented with severe calcific stenosis of Edwards SAPIEN-XT valve implanted only four years previously. She is a Jehovah's Witness and has background of Sjogren's syndrome with secondary cryoglobulinaemic vasculitis. She was not a suitable candidate for valve-in-valve TAVR in view of early prosthetic valve failure by calcification...
May 9, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28529340/vasculitis-a-clear-argument-for-targeting-complement-in-anca-vasculitis
#17
Ralph Kettritz
No abstract text is available yet for this article.
May 22, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28528615/chronic-tender-ulcers-on-the-calf-and-both-forearms
#18
Michael C Cameron, Mitsuya Katayama, Nishit S Patel, Philip D Shenefelt, Charurut Somboonwit
An elderly woman presented with a 3-month history of nonhealing, tender ulcers involving the right calf and both forearms. She denied any history of similar lesions or trauma. Two trials of oral antibiotics had led to no improvement. Her medical history was significant for rheumatoid arthritis treated with methotrexate, hydroxychloroquine, and prednisone. A review of clinical manifestations was otherwise negative for disease. Physical examination of the patient's right calf revealed two punched-out ulcers with central necrotic black eschars, underlying retiform purpuric pattern, and mild fibrinopurulent drainage (Figure 1)...
2017: Skinmed
https://www.readbyqxmd.com/read/28527983/when-a-death-apparently-associated-to-sexual-assault-is-instead-a-natural-death-due-to-idiopathic-hypereosinophilic-syndrome-the-importance-of-gamma-hydroxybutyric-acid-analysis-in-vitreous-humor
#19
Francesco Paolo Busardò, Francesca Portelli, Angelo Montana, Maria Concetta Rotolo, Simona Pichini, Emiliano Maresi
We here report a case involving a 21-year-old female, found dead in a central square of a city in the south of Italy. Initial evidences and circumstances were suggestive of a death associated with a sexual assault. Two peripheral blood and two vitreous humor samples were collected for the purpose of gamma-hydroxybutyric acid (GHB) testing from the dead body at two different post-mortem intervals (PMIs): approximately 2 (t0) and 36 (t1) hours. The obtained results showed that, between t0 and t1, there was an increase of GHB concentrations in peripheral blood and vitreous humor of 66...
April 11, 2017: Legal Medicine
https://www.readbyqxmd.com/read/28527263/-racemose-neurocysticercosis-neuroimaging-guides-the-diagnosis
#20
Carlos Hugo Zapata, Sergio Alberto Vargas, Carlos Santiago Uribe
Neurocysticercosis is the leading cause of parasitosis of the central nervous system and acquired epilepsy in developing countries. The clinical manifestations of neurocysticercosis, especially its racemose variant, are pleomorphic and unspecific, characteristics that hinder the diagnosis and make it a challenge for the clinician.The objective of this report was to describe two cases of racemose neurocysticercosis in which neuroimaging led to the definitive diagnosis. The first case involved a patient with persistent headache and focal neurological signs...
April 1, 2017: Biomédica: Revista del Instituto Nacional de Salud
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