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https://www.readbyqxmd.com/read/27893661/acute-coronary-vasospasm-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-following-nsaid-administration-a-case-report
#1
Naïl Benallegue, Pierre Lozach, Cristina Belizna, Christian Lavigne, Geoffrey Urbanski
Eosinophilic with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare systemic disease characterized by a small-vessel necrotizing vasculitis. Cardiac manifestations are broad-ranging and are associated with a poor prognosis. Coronary vasospasm is uncommon.Here, we report a case of an acute coronary vasospasm in a patient with EGPA after corticosteroids withdrawal and nonsteroidal antiinflammatory drug (NSAID) introduction. This patient was initially misdiagnosed as bradykinin-mediated angioedema...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27857032/eosinophilic-granulomatosis-with-polyangiitis-presenting-with-skin-rashes-eosinophilic-cholecystitis-and-retinal-vasculitis
#2
Mingbing Zeng, Xialin Liu, Yizhi Liu
BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. CASE REPORT The patient was hospitalized for high fever and skin rashes and then developed right upper abdominal pain, decreased visual acuity, coma, and convulsions...
November 18, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27825424/radiological-and-clinical-features-of-eosinophilic-granulomatosis-with-polyangiitis
#3
Xiao-Li Xu, Wei Song, Xin Sui, Lan Song, Qian-Ni DU, Xiao Wang
Eosinophilic granulomatosis with polyangiitis(EGPA),also known as Churg-Strauss syndrome,is a clinically rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs),characterized by asthma,disseminated necrotizing vasculitis,extravascular granulomas,peripheral eosinophilia,and tissue eosinophilia. This article reviews the pathology,imaging,and clinical features of EGPA.
October 10, 2016: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/27812073/eosinophilic-granulomatosis-with-polyangiitis-presenting-with-multiple-intracerebral-haemorrhages-a-case-report
#4
Zafar Ullah, Zain Ul Abideen, Raja Farhat Shoaib, Arsalan Ahmad, Qamar Zaman, Rashid Nazir
Eosinophilic granulomatosis with polyangiitis also known as Churgg-Strauss syndrome is a systemic multi system vasculitis. Neurological involvement is mostly in the form of peripheral nervous system disease. Central nervous system involvement is relatively uncommon with most cases being secondary to ischaemic infarctions. Intra cerebral haemorrhage is rare and is usually in the form of solitary haemorrhagic lesions. Multiple intra cerebral haemorrhages are exceptionally rare with only one case documented in medical literature...
November 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27804171/development-and-validation-of-case-finding-algorithms-for-the-identification-of-patients-with-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-in-large-healthcare-administrative-databases
#5
Antoine G Sreih, Narender Annapureddy, Jason Springer, George Casey, Kevin Byram, Andy Cruz, Maya Estephan, Vince Frangiosa, Michael D George, Mei Liu, Adam Parker, Sapna Sangani, Rebecca Sharim, Peter A Merkel
PURPOSE: The aim of this study was to develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (Churg-Strauss, EGPA). METHODS: Two hundred fifty patients per disease were randomly selected from two large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). Sixteen case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody type...
December 2016: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/27727077/comprehensive-evaluation-of-cardiac-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-egpa-with-cardiac-magnetic-resonance
#6
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
https://www.readbyqxmd.com/read/27671089/revisiting-the-systemic-vasculitis-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-a-study-of-157-patients-by-the-groupe-d-etudes-et-de-recherche-sur-les-maladies-orphelines-pulmonaires-and-the-european-respiratory-society-taskforce-on-eosinophilic
#7
Vincent Cottin, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A Sinico, Pasupathy Sivasothy, Michael E Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne Gondouin, Bertrand Dunogué, Gérard Chatté, Christophe Briault, Christian Pagnoux, David Jayne, Loïc Guillevin, Jean-François Cordier
OBJECTIVE: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. METHODS: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. RESULTS: The study population included 157 patients (mean age 49...
September 23, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27625985/think-twice-diagnostic-delay-in-a-patient-with-acute-chest-pain
#8
Cæcilie Larsen Bang, Celeste Michala Porsbjerg
Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA). We present a case of acute chest pain in a 58-year-old male with severe asthma, which regressed after sublingual administration of nitroglycerine. At the time of hospital admission, there were non-specific ST-changes on the ecg, coronary enzymes were increased, and the patient was concluded to have a non-ST-elevation myocardial infarction, and treated as such. A subacute cardiac catheterization showed no signs of significant coronary stenosis...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27606476/chronic-skin-lichenification-as-unusual-presentation-of-eosinophilic-granulomatosis-with-polyangitis-case-report-and-literature-review
#9
F Sbrana, B Loggini, S Galimberti, M Coceani, M Latorre, V Seccia, S L'Abbate, M Mosca, E M Pasanisi, C Baldini
Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually developed a full-blown ANCA-MPO positive EGPA in combination with blood hypereosinophilia, eosinophilic vasculitis at skin biopsy, subclinical asthma and chronic rhinosinusitis...
April 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/27591039/anca-negative-eosinophilic-granulomatosis-with-polyangitis-egpa-manifesting-as-a-large-intracardiac-thrombus-and-glomerulonephritis-with-angionecrosis
#10
Yuichi Saito, Sho Okada, Nobusada Funabashi, Yoshio Kobayashi
A 59-year-old woman with a history of bronchial asthma presented with a prolonged fever and eosinophilia. There was transient proteinuria and troponin level was elevated. Antineutrophil cytoplasmic antibody was negative and she did not fulfil criteria for eosinophilic granulomatosis with polyangitis (EGPA). Echocardiography showed a large apical mass in the left ventricle, but there was no systolic dysfunction, local asynergy or ventricular remodelling. On MRI, apical mass was compatible with a thrombus and endocardial region was diffusely damaged...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27587545/respiratory-manifestations-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss
#11
Vincent Cottin, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A Sinico, Pasupathy Sivasothy, Michael E Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne Gondouin, Bertrand Dunogué, Gérard Chatté, Amandine Briault, David Jayne, Loïc Guillevin, Jean-François Cordier
The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×10(9) L(-1) at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1...
September 1, 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27549670/a-diagnostic-algorithm-for-eosinophilic-granulomatosis-with-polyangiitis-initially-diagnosed-as-lumbar-disc-hernia-or-lumbar-spinal-stenosis-personal-experience-and-review-of-the-literature
#12
Kosei Nagata, Shinichi Yamamoto, Kota Miyoshi, Masaki Sato, Yusuke Arino, Yoji Mikami
Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic vasculitis and is difficult to diagnose. EGPA has a number of symptoms including peripheral dysesthesia caused by mononeuropathy multiplex, which is similar to radiculopathy due to lumbar disc hernia or lumbar spinal stenosis. Therefore, EGPA patients with mononeuropathy multiplex often visit orthopedic clinics, but orthopedic doctors and spine neurosurgeons have limited experience in diagnosing EGPA because of its rarity...
August 2016: Acta Medica Okayama
https://www.readbyqxmd.com/read/27536681/the-prevalence-and-management-of-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-in-china
#13
REVIEW
Zhi-Ying Li, Tian-Tian Ma, Min Chen, Ming-Hui Zhao
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. SUMMARY: This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients...
March 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27464538/-the-clinical-analysis-of-46-cases-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#14
Q Zhang, H Q Zhou, Y H Li, J Guo, P H Xu, M H Lu, X L Liu, L Wang, S G Li
OBJECTIVE: To investigate the clinical features of patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and to explore the disease activity of AAV patients and the relationship with prognosis. METHODS: The clinical data of 46 cases of AAV patients in the First Affiliated Hospital of PLA General Hospital were analyzed retrospectively.The clinical and laboratory features of each clinical subtype were compared.The disease activity of AAV and the relationship between disease activity and prognosis were evaluated...
July 19, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27464484/anca-associated-vasculitis-clinical-utility-of-using-anca-specificity-to-classify-patients
#15
REVIEW
Divi Cornec, Emilie Cornec-Le Gall, Fernando C Fervenza, Ulrich Specks
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a heterogeneous group of rare syndromes characterized by necrotizing inflammation of small and medium-sized blood vessels and the presence of ANCAs. Several clinicopathological classification systems exist that aim to define homogeneous groups among patients with AAV, the main syndromes being microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Two main types of ANCA can be detected in patients with AAV...
October 2016: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/27461086/long-term-outcomes-in-antineutrophil-cytoplasmic-autoantibody-positive-eosinophilic-granulomatosis-with-polyangiitis-patients-with-renal-involvement-a-retrospective-study-of-14-chinese-patients
#16
Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied. METHODS: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed. RESULTS: The most common initial symptom of EGPA was asthma (57...
July 26, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27272254/impact-of-renal-involvement-on-survival-in-anca-associated-vasculitis
#17
Manish Rathi, Benzeeta Pinto, Aadhaar Dhooria, Vinay Sagar, Tarun Mittal, Roopa Rajan, Varun Dhir, Susheel Kumar, Kusum Sharma, Ritambhra Nada, Surjit Singh, Ranjana Walker Minz, Aman Sharma
BACKGROUND: Renal involvement is a serious complication of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). We describe the pattern of renal involvement and its correlation with outcomes. PATIENTS AND METHODS: Medical records of 92 patients seen in rheumatology clinic and diagnosed as AAV between January 2007 and June 2014 were analysed. Patients were classified as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA) and undifferentiated AAV...
September 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27191665/optimal-therapy-and-prospects-for-new-medicines-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#18
Christian Pagnoux, Matthieu Groh
INTRODUCTION: The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome) is lower than that of other antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV's), and only a few randomized controlled trials have been conducted for this rare disease. However, recent international efforts have helped delineate the best treatment approach. AREAS COVERED: At present, EGPA conventional therapy is by default similar to that of other AAVs...
October 2016: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27166610/protective-role-of-hla-drb1-13-02-against-microscopic-polyangiitis-and-mpo-anca-positive-vasculitides-in-a-japanese-population-a-case-control-study
#19
Aya Kawasaki, Narumi Hasebe, Misaki Hidaka, Fumio Hirano, Ken-Ei Sada, Shigeto Kobayashi, Hidehiro Yamada, Hiroshi Furukawa, Kunihiro Yamagata, Takayuki Sumida, Nobuyuki Miyasaka, Shigeto Tohma, Shoichi Ozaki, Seiichi Matsuo, Hiroshi Hashimoto, Hirofumi Makino, Yoshihiro Arimura, Masayoshi Harigai, Naoyuki Tsuchiya
Among antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), granulomatosis with polyangiitis (GPA) and proteinase 3-ANCA-positive AAV (PR3-AAV) are prevalent in European populations, while microscopic polyangiitis (MPA) and myeloperoxidase-ANCA-positive AAV (MPO-AAV) are predominant in the Japanese. We previously demonstrated association of DRB1*09:01-DQB1*03:03 haplotype, a haplotype common in East Asians but rare in the European populations, with MPA/MPO-AAV, suggesting that a population difference in HLA-class II plays a role in the epidemiology of this disease...
2016: PloS One
https://www.readbyqxmd.com/read/27155202/chronic-skin-lichenification-as-unusual-presentation-of-eosinophilic-granulomatosis-with-polyangitis-case-report-and-literature-review
#20
Francesco Sbrana, Barbara Loggini, Sara Galimberti, Michele Coceani, Marta Latorre, Veronica Seccia, Sara L'Abbate, Marta Mosca, Emilio Maria Pasanisi, Chiara Baldini
Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually developed a full-blown ANCA-MPO positive EGPA in combination with blood hypereosinophilia, eosinophilic vasculitis at skin biopsy, subclinical asthma and chronic rhinosinusitis...
January 12, 2016: Acta Reumatológica Portuguesa
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