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https://www.readbyqxmd.com/read/28641275/a-case-series-of-surgically-diagnosed-idiopathic-aortitis-in-a-canadian-centre-a-retrospective-study
#1
Diane L Murzin, Eric C Belanger, John P Veinot, Nataliya Milman
BACKGROUND: Idiopathic aortitis became recognized relatively recently, and the body of knowledge concerning this condition is scarce. We aimed to determine the frequency of idiopathic aortitis in aortic specimens, the clinical, laboratory and radiologic characteristics at diagnosis and during follow-up, and the approach to investigation, treatment and monitoring taken by the treating physicians. METHODS: We identified cases of aortitis diagnosed on pathological specimens of the aorta between Jan...
June 19, 2017: CMAJ Open
https://www.readbyqxmd.com/read/28640813/identification-of-a-sj%C3%A3-gren-s-syndrome-susceptibility-locus-at-oas1-that-influences-isoform-switching-protein-expression-and-responsiveness-to-type-i-interferons
#2
He Li, Tove Ragna Reksten, John A Ice, Jennifer A Kelly, Indra Adrianto, Astrid Rasmussen, Shaofeng Wang, Bo He, Kiely M Grundahl, Stuart B Glenn, Corinne Miceli-Richard, Simon Bowman, Sue Lester, Per Eriksson, Maija-Leena Eloranta, Johan G Brun, Lasse G Gøransson, Erna Harboe, Joel M Guthridge, Kenneth M Kaufman, Marika Kvarnström, Deborah S Cunninghame Graham, Ketan Patel, Adam J Adler, A Darise Farris, Michael T Brennan, James Chodosh, Rajaram Gopalakrishnan, Michael H Weisman, Swamy Venuturupalli, Daniel J Wallace, Kimberly S Hefner, Glen D Houston, Andrew J W Huang, Pamela J Hughes, David M Lewis, Lida Radfar, Evan S Vista, Contessa E Edgar, Michael D Rohrer, Donald U Stone, Timothy J Vyse, John B Harley, Patrick M Gaffney, Judith A James, Sean Turner, Ilias Alevizos, Juan-Manuel Anaya, Nelson L Rhodus, Barbara M Segal, Courtney G Montgomery, R Hal Scofield, Susan Kovats, Xavier Mariette, Lars Rönnblom, Torsten Witte, Maureen Rischmueller, Marie Wahren-Herlenius, Roald Omdal, Roland Jonsson, Wan-Fai Ng, Gunnel Nordmark, Christopher J Lessard, Kathy L Sivils
Sjögren's syndrome (SS) is a common, autoimmune exocrinopathy distinguished by keratoconjunctivitis sicca and xerostomia. Patients frequently develop serious complications including lymphoma, pulmonary dysfunction, neuropathy, vasculitis, and debilitating fatigue. Dysregulation of type I interferon (IFN) pathway is a prominent feature of SS and is correlated with increased autoantibody titers and disease severity. To identify genetic determinants of IFN pathway dysregulation in SS, we performed cis-expression quantitative trait locus (eQTL) analyses focusing on differentially expressed type I IFN-inducible transcripts identified through a transcriptome profiling study...
June 22, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28637108/-henoch-sch%C3%A3-nlein-purpura-presenting-as-intussusception
#3
Keun Young Kim
Henoch-Schönlein purpura (HSP) is systemic vasculitis disease with various clinical manifestations. Gastrointestinal symptoms in patients with HSP are usually common, with an incidence rate of 62-90%. Most of these gastrointestinal symptoms occur after typical skin purpura, which is a very important clinical evidence for making a diagnosis of HSP. It is difficult to diagnose HSP without skin rash. About 25% of patients may experience gastrointestinal symptoms as their first symptoms. Herein, we report a case of ileo-colic intussusception associated with HSP in a 5-years-old girl presented with diffuse abdominal distension...
June 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28633740/parvovirus-b19-infection-in-an-adult-presenting-with-connective-tissue-disease-like-symptoms-a-report-of-the-clinical-and-histological-findings
#4
J E Liles, S C Shalin, B A White, L B Trigg, J R Kaley
Parvovirus B19 infections in adults are usually associated with nonspecific and mild symptoms. However, cases presenting with a lupus-like syndrome have been described, leading to the hypothesis that parvovirus infection can induce connective tissue disease. Various histopathologic features of cutaneous manifestations of parvovirus have been reported, including features which overlap with those of connective tissue disease. Herein, we discuss an unusual case of Parvovirus  B19 infection in a middle-aged woman...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28632810/hydralazine-associated-cutaneous-casculitis-presenting-with-aerodigestive-tract-involvement
#5
Laura E Levin, Cynthia Magro, James Horowitz, Joanna Harp
Hydralazine-induced small vessel vasculitis is a rare entity with a limited number of cases reported in the dermatologic literature. A characteristic pattern of acral pseudoembolic vesiculopustules with necrosis and ulceration has been suggested along with involvement of the aerodigestive tract, indicating mucosal involvement is an important feature of this disease. We report the case of a patient with hydralazine-induced vasculitis who exemplified this characteristic presentation associated with severe involvement of the aerodigestive tract and gastrointestinal tract bleeding...
May 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28631704/-beh%C3%A3-et-s-disease-intracardiac-thrombosis-a-description-of-two-cases-and-a-review-of-literature
#6
Z S Alekberova, P S Ovcharov, T A Lisitsyna, A V Volkov, T V Popkova
Behçet's disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28631698/-hepatitis-c-virus-associated-cryoglobulinemic-vasculitis-a-20-year-experience-with-treatment
#7
T M Ignatova, L V Kozlovskaya, N B Gordovskaya, O A Chernova, S Yu Milovanova, P I Novikov, T P Nekrasova, T V Beketova, N A Mukhin
AIM: To summarize the experience of a multidisciplinary therapy hospital in treating patients with hepatitis C virus (HCV)-associated cryoglobulinemic vasculitis (CV). SUBJECTS AND METHODS: Seventy-two patients (mean age, 49.4±10.3 years) with HCV-associated CV were examined and followed up for an average period of 2.8±3.6 years. The efficiency of traditional (corticosteroids ± cyclophosphamide) and selective (rituximab) immunosuppressive therapy (IST) was estimated in 31 and 15 observations, respectively, and that of antiviral therapy (AVT) in 25...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28631066/rheumatoid-vasculitis-a-diminishing-yet-devastating-menace
#8
REVIEW
Shweta Kishore, Lisa Maher, Vikas Majithia
PURPOSE OF REVIEW: Rheumatoid vasculitis (RV) is an unusual complication of long-standing rheumatoid arthritis, which is characterized by the development of necrotizing or leukocytoclastic vasculitis involving small or medium-sized vessels. In this review, we aim to provide an update on the epidemiology, pathogenesis, clinical presentation, and management of this challenging extra-articular manifestation. RECENT FINDINGS: RV is heterogenous in its clinical presentation depending on the organ and size of blood vessels involved...
July 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28630794/a-case-of-presumed-tuberculosis-uveitis-with-occlusive-vasculitis-from-an-endemic-region
#9
Berna Başarır, Yalçın Karaküçük, Çiğdem Altan, Banu Şatana, Bulut Ocak, Aslı İnal
In this report, we present a case with presumed unilateral tuberculosis uveitis from an endemic region. A 23-year-old male presented with decreased vision in his left eye for 15 days. Visual acuities were 1.0 in his right eye and 0.3 in his left eye. Ophthalmologic examination was normal for the right eye. Slit-lamp examination revealed 2+ cells in the vitreous without anterior chamber reaction in his left eye. Fundus examination revealed occlusive vasculitis and granuloma. His history revealed that he had a respiratory infection with fever 3 months ago while visiting his native country, Rwanda, and was treated with non-specific antibiotic therapy...
June 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28630775/a-rare-case-of-paraneoplastic-aortitis-associated-with-chronic-myelomonocytic-leukemia
#10
Sylwia Sasinowska, Pamela Traisak, Michael McCormack, Hala Eid
Aortitis is a broad term describing inflammation of the aorta. The most common causes of aortitis are the large-vessel vasculitides giant cell arteritis and Takayasu arteritis. Other etiologies include aortitis associated with other autoimmune disorders, infectious causes, and paraneoplastic and idiopathic cases. We describe a rare case of a large-vessel arteritis occurring in association with chronic myelomonocytic leukemia (CMML). A 68-year-old female with recent diagnosis of CMML presented to our office for evaluation of abnormal chest computed tomography (CT) that showed inflammation surrounding the entirety of thoracic and abdominal aorta, consistent with aortitis...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28627981/early-vitrectomy-for-dense-vitreous-hemorrhage-in-adults-with-non-traumatic-and-non-diabetic-retinopathy
#11
Ting Zhang, Jingjing Zhang, Xiaolei Sun, Jingyi Tian, Weiyun Shi, Gongqiang Yuan
Objective To evaluate the etiologies for dense vitreous hemorrhage in adults with non-traumatic and reveal management of early vitrectomy for the disease. Methods Study included 105 eyes from 105 patients. Outcome measures were etiologies of vitreous hemorrhage, formation of retinal and/or disk neovascular membrane (NVM), incidence of retinal tear and detachment, visual acuity (VA) and postoperative complications. Results Mean time between presentation and surgery was 7.1 days. The most common etiologies were retinal vein occlusion (RVO) (58...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28626961/comparing-polyarteritis-nodosa-in-children-and-adults-a-single-center-study
#12
Abdulsamet Erden, Ezgi D Batu, Hafize E Sönmez, Alper Sarı, Berkan Armagan, Zehra S Arıcı, Emre Bilgin, Umut Kalyoncu, Ömer Karadağ, Yelda Bilginer, Ali Ihsan Ertenli, Seza Özen
OBJECTIVE: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. METHODS: Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. RESULTS: Five children had cutaneous and all the rest of the patients had systemic PAN...
June 19, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28626447/autoimmune-thyroiditis-and-glomerulopathies
#13
REVIEW
Domenico Santoro, Carmela Vadalà, Rossella Siligato, Michele Buemi, Salvatore Benvenga
Autoimmune thyroiditis (AIT) is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA) vasculitis, and amyloidosis...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28626187/efficacy-of-intravenous-cyclophosphamide-pulse-therapy-for-p-glycoprotein-expressing-b-cell-associated-active-true-renal-lupus-vasculitis-in-lupus-nephritis
#14
Akio Kawabe, Shizuyo Tsujimura, Kazuyoshi Saito, Yoshiya Tanaka
True renal lupus vasculitis (TRLV), a vascular lesion usually associated with proliferative lupus nephritis (LN), is resistant to conventional treatments. The expression of P-glycoprotein (P-gp) on activated lymphocytes causes drug resistance. We herein report a patient with TRLV, minimal change LN, overexpression of P-gp on peripheral B cells, and accumulation of P-gp(+) B cells at the site of TRLV. High-dose corticosteroids combined with intravenous cyclophosphamide pulse therapy resulted in clinical remission and the long-term normal renal function...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28624373/acute-tubulointerstitial-nephritis-in-an-hla-b27-positive-patient-with-axial-spondyloarthritis-being-treated-with-adalimumab
#15
David Castro Corredor, María Dolores Sánchez de la Nieta, Isabel María de Lara Simón
Antagonists of tumor necrosis factor-alpha (ATNF) are used for the treatment of multiple diseases such as psoriatic arthritis, Crohn's disease, ankylosing spondylitis and juvenile idiopathic arthritis, usually, when they are refractory to first-line treatment(1). The use of ATNF has been associated with the induction of autoimmune diseases such as systemic lupus erythematosus-like disease, vasculitis, sarcoidosis-like diseases and, recently, acute granulomatous tubulointerstitial nephritis. We report a case of acute nongranulomatous tubulointerstitial nephritis in an HLA-B27-positive patient with axial spondyloarthritis and Crohn's disease being treated with adalimumab...
June 14, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#16
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
June 16, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28619740/gamma-delta-t-cell-lymphoma-of-skin-eye-and-brain-presenting-with-visual-loss
#17
Nicholas Jones, Adam Gibb, Luciane Irion, Sarah Coupland
A young man presented with rapid, predominantly right-sided visual loss with a background of multifocal skin lesions. Visual acuity was right hand movements, left 6/5 Snellen, deteriorating to 6/38. He showed panuveitis with bilateral multifocal retinal infiltrates and retinal vasculitis. Multifocal brain lesions were identified. Biopsy of both skin and vitreous showed atypical lymphocytes, and immunohistochemistry confirmed T-cell lymphoma of gamma-delta subtype. Management with the CODOX-M/IVAC polychemotherapy regimen achieved rapid response including resolution of intraocular changes and substantial improvement of visual acuity to right 6/7...
June 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28614577/successful-treatment-of-cutaneous-small-vessel-vasculitis-with-leflunomide
#18
Julia D Stiegler, Naveed Sami
No abstract text is available yet for this article.
June 14, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28614191/anti-cd20-blocker-rituximab-in-kidney-transplantation
#19
Puneet Sood, Sundaram Hariharan
Rituximab is a chimeric anti-CD20 monoclonal protein used in various clinical scenarios in kidney transplant recipients. However, its evidence-based use there remains limited due to lack of controlled studies, limited sample size, short follow-up and poorly defined endpoints. Rituximab is indicated for CD20+ PTLD. It may be beneficial for treating recurrent MN and recurrent allograft ANCA vasculitis and possibly for recurrent FSGS. Rituximab, in combination with IVIG/PP, appears to decrease antibody level and increase the odds of transplantation in sensitized recipients...
June 13, 2017: Transplantation
https://www.readbyqxmd.com/read/28614161/multiple-cerebral-infarcts-in-a-young-patient-associated-with-marijuana-use
#20
Leila Costa Volpon, Camila Lacerda Muniz de Melo Sousa, Silvia Keiko Kavaguti Moreira, Sara Reis Teixeira, Ana Paula de Carvalho Panzeri Carlotti
: Cerebrovascular events associated with marijuana use have been reported previously. This association is plausible, but not well-established yet. A 14-year-old girl, long-term heavy cannabis user, presented with generalized tonic-clonic seizures and decreased level of consciousness a few hours after smoking cannabis. Brain magnetic resonance imaging showed multiple areas of acute, subacute and chronic ischemic lesions in the left frontal lobe, basal ganglia, and corpus callosum. History of other illicit drug use and other known causes of stroke were ruled out...
June 13, 2017: Journal of Addiction Medicine
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