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Systemic vasculitis

Si Chen, Xiaoting Wen, Jing Li, Yuan Li, Liubing Li, Xinping Tian, Hui Yuan, Fengchun Zhang, Yongzhe Li
Takayasu arteritis (TA) is a chronic large-vessel vasculitis of unclear pathogenesis. A recent genome-wide association study (GWAS) has revealed that the FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX genes confer susceptibility to TA. We investigated the linkage between presumptive TA-related genes (FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX) and TA in the Han Chinese population. We performed a large case-control multi-center study of 412 Han Chinese TA patients and 597 ethnically matched healthy controls...
October 18, 2016: Oncotarget
Sarah-Jayne Edmondson, Saina Attaran, Ulrich P Rosendahl
Wegner's granulomatosis (WG) is an autoimmune systemic vasculitis that results in necrotizing granulomas. We report a WG patient with a lung granuloma and aortic root dilatation, who underwent aortic root replacement on cardiopulmonary bypass (CPB). Intraoperatively, the patient suffered an aortic dissection, which was repaired immediately under deep hypothermic circulatory arrest (DHCA). Follow-up imaging showed complete granuloma resolution, despite absence of immunosuppressive therapy. Immune stimulation following CPB is well described; here, the opposite was observed and DHCA effects are discussed...
February 2016: Aorta (Stamford, Conn.)
Mar Llamas-Velasco, Victoria Alegría, Ángel Santos-Briz, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material...
October 18, 2016: American Journal of Dermatopathology
Abhinav Anand, Kruthi Malur, Juhi Kawale, Milind Y Nadkar
We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. 38 years old female who had earlier polyserositis and cerebral venous thrombosis was admitted with provisional diagnosis of SLE and developed acute abdominal pain during hospital stay. She was diagnosed as mesenteric vasculitis and initially responded to pulse methylprednisolone. However, she had relapse which subsequently responded to pulse cyclophosphamide and steroids...
July 2016: Journal of the Association of Physicians of India
Malik Y Ghannam, Mohammed Naseemuddin, Peter Weiser, John O Mason
BACKGROUND: Acute Multifocal Hemorrhagic Retinal Vasculitis (AMHRV) is a rare disease with unknown incidence that presents with abrupt onset of visual loss associated with retinal vasculitis, retinal hemorrhage, non-confluent posterior retinal infiltrates, vitreous cellular inflammation and papillitis in, otherwise, healthy adult individuals. The reported treatment options for Acute Multifocal Hemorrhagic Retinal Vasculitis are oral corticosteroids, intravitreal ganciclovir and laser photocoagulation or vitrectomy...
October 18, 2016: BMC Ophthalmology
Rosaria Talarico, Simone Barsotti, Elena Elefante, Chiara Baldini, Chiara Tani, Marta Mosca
PURPOSE OF REVIEW: The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. RECENT FINDINGS: Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis...
October 17, 2016: Current Opinion in Rheumatology
Hagit Peleg, Eldad Ben-Chetrit
PURPOSE OF REVIEW: This article addresses the prevalence and relationship between autoinflammatory diseases and vasculitis. RECENT FINDINGS: Autoimmune diseases (AIDs) are a group of syndromes characterized by episodes of unprovoked inflammation due to dysregulation of the innate immune system. Despite the common occurrence of rashes and other skin lesions in these diseases, vasculitis is reported in only a few. On the other hand, neutrophilic dermatoses are more prevalent...
October 13, 2016: Current Opinion in Rheumatology
Jiajin Yang, Heng Ge, Caroline J Poulton, Susan L Hogan, Yichun Hu, Britta E Jones, Candace D Henderson, Elizabeth A McInnis, William F Pendergraft, J Charles Jennette, Ronald J Falk, Dominic J Ciavatta
BACKGROUND: Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a systemic autoimmune disease characterized by destructive vascular inflammation. Two prominent ANCA autoantigens are myeloperoxidase (MPO) and proteinase 3 (PR3), and transcription of MPO and PRTN3, the genes encoding the autoantigens, is associated with disease activity. We investigated whether patients with AAV have alterations in histone modifications, particularly those associated with transcriptional activation, at MPO and PRTN3...
2016: Clinical Epigenetics
Yuka Morita, Kuniyuki Takahashi, Shuji Izumi, Yamato Kubota, Shinsuke Ohshima, Arata Horii
OBJECTIVE: Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. STUDY DESIGN: Retrospective chart review...
September 30, 2016: Otology & Neurotology
Kenzo J Koike, Jeffrey P Blice, Jan A Kylstra, Jonathan S Ralston, Sally E Self, Natasha M Ruth, Lucian V Del Priore
PURPOSE: To describe an unusual case of frosted branch angiitis that developed in a patient with acute onset systemic vasculitis possibly triggered by the antithyroid medication methimazole. METHODS: We conducted a thorough review of the medical records of a 16-year-old female patient who presented with frosted branch angiitis. During the initial hospital admission, the patient underwent an extensive systemic workup to determine the etiology of her disease and ophthalmologic testing including fundus photographs and fluorescein angiography...
October 5, 2016: Retinal Cases & Brief Reports
Eline Houben, Willem A Bax, Bastiaan van Dam, Walentina A T Slieker, Gideon Verhave, Fenneke C P Frerichs, Izhar C van Eijk, Wim G Boersma, Guido T M de Kuyper, Erik L Penne
Currently no validated diagnostic system for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is available. Therefore, diagnosing AAV is often challenging. We aimed to identify factors that lead to a clinical diagnosis AAV in ANCA positive patients in a teaching hospital in The Netherlands.In this study, all patients that tested positive for ANCA proteinase 3 (PR3) and/or myeloperoxidase (MPO) between 2005 and 2015 were analysed. Patients with a clinical diagnosis of AAV were compared with patients without a clinical diagnosis of AAV...
October 2016: Medicine (Baltimore)
Marinka Twilt, Troels Herlin
In children, inflammatory brain diseases (IBrainDs) are increasingly being recognized. Clinical, neuroimaging and laboratory features are overlapping, and an exact diagnosis can be significantly delayed. Novel antibodies have been discovered and should be included in the diagnostic evaluation. Specific neuroradiological tests such as conventional angiography or vessel wall enhancement can assist in supporting the diagnosis. Brain biopsy should be considered in children with IBrainD with unclear pathology. Treatment should be tailored to the underlying pathogenesis...
October 17, 2016: Ugeskrift for Laeger
Mikkel Faurschou, Magnus G Ahlström, Jesper Lindhardsen, Niels Obel, Bo Baslund
OBJECTIVE: Patients with organ- or life-threatening vasculitis receive high cumulative glucocorticoid (GC) doses during their disease course. GC have diabetogenic effects, but the risk of diabetes mellitus (DM) related to vasculitis therapy is not well characterized. We assessed the DM risk among patients diagnosed with giant cell arteritis (GCA) or granulomatosis with polyangiitis (GPA), i.e., patients with relatively common forms of systemic vasculitis. METHODS: We used Danish healthcare registries to identify 1682 patients diagnosed with GCA and 342 patients diagnosed with GPA from 1997 to 2015 and to obtain information regarding medication exposures...
October 15, 2016: Journal of Rheumatology
C Cossart, G Le Moal, M Garcia, E Frouin, E Hainaut-Wierzbicka, F Roblot
BACKGROUND: Visceral leishmaniasis is not normally expressed in skin. Herein, we describe the case of an HIV-positive patient who developed two unusual skin manifestations during an episode of visceral leishmaniasis. PATIENTS AND METHODS: A 48-year-old female patient consulted initially for infiltrated purpura of all four limbs. Skin biopsy revealed leukocytoclastic vasculitis with Leishman-Donovan bodies. Laboratory tests showed medullary, splenic, gastric and colic involvement, suggesting systemic disease, and enabling visceral leishmaniasis to be diagnosed...
October 11, 2016: Annales de Dermatologie et de Vénéréologie
Sergio Prieto-González, Georgina Espígol-Frigolé, Ana García-Martínez, Marco A Alba, Itziar Tavera-Bahillo, José Hernández-Rodríguez, Arturo Renú, Rosa Gilabert, Francisco Lomeña, Maria C Cid
Various imaging modalities, including color duplex ultrasonography, CT angiography, magnetic resonance angiography, and PET, are emerging as important aids to the diagnosis, staging, evaluation of disease activity and response to treatment in systemic vasculitis. Although large-vessel vasculitis is the main target of imaging, refinement and increasing accuracy of imaging modalities are also providing useful information in the evaluation of medium-vessel and small-vessel vasculitis.
November 2016: Rheumatic Diseases Clinics of North America
Carsten Paul Bramlage, Britta Froelich, Manuel Wallbach, Joan Minguet, Clemens Grupp, Cornelia Deutsch, Peter Bramlage, Michael Koziolek, Gerhard Anton Müller
In patients with rheumatic diseases, reliable markers for determining disease activity are scarce. One potential parameter is the level of immunoglobulin free light chains (FLCs), which is known to be elevated in the blood of patients with certain rheumatic diseases. Few studies have quantified FLCs in urine, a convenient source of test sample, in patients with different rheumatic diseases. We carried out a retrospective analysis of patients with rheumatic disease attending the University hospital of Goettingen, Germany...
October 12, 2016: Clinical Rheumatology
Melike Kalfa, Hayriye Kocanaoğulları, Gonca Karabulut, Hakan Emmungil, Celal Çınar, Zevcet Yılmaz, Sercan Gücenmez, Yasemin Kabasakal
Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason...
September 2016: Eur J Rheumatol
Alberto Francesco Cereda, Patrizia Pedrotti, Lucio De Capitani, Cristina Giannattasio, Alberto Roghi
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by hypereosinophilia. EGPA typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally systemic vasculitis. Cardiac involvement is the most important predictor of mortality; it occurs in approximately 15-60% of EGPA patients, a significant proportion of whom are asymptomatic and have normal electrocardiogram (ECG) and echocardiogram...
October 7, 2016: European Journal of Internal Medicine
Mohammed El Amine Ghembaza, Ali Lounici
No abstract text is available yet for this article.
October 11, 2016: Ocular Immunology and Inflammation
Alfredo Seijo, Sergio Giamperetti, Sonia M Ortiz Mayor, María B González, Eugenia S Ortega, Rossana C González
On the fifth day after leaving the Parque Nacional El Rey, province of Salta, Argentina, where she made rural tourism, a woman of Italian origin, aged 47, developed an acute fever followed by a petechial and purpuric rash that progressed rapidly to multiorgan failure. She died on the sixth day after hospitalization. There were references to tick bites and a skin lesion similar to tache noire was found. The autopsy showed generalized vasculitis, ascites, pulmonary edema, acute tubular necrosis and portal centrilobular necrosis...
2016: Medicina
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