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eosinophilic granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/28339364/clinical-and-prognostic-features-of-korean-patients-with-mpo-anca-pr3-anca-and-anca-negative-vasculitis
#1
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/28339359/cardiac-involvement-as-a-presenting-feature-of-eosinophilic-granulomatosis-with-polyangiitis-in-childhood
#2
Timothy D Reynolds, Catherine Armstrong, Athimalaipet V Ramanan
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March 15, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28281453/iga-antibodies-to-myeloperoxidase-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss
#3
Esha Oommen, Amber Hummel, Lisa Allmannsberger, David Cuthbertson, Carette Simon, Christian Pagnoux, Gary S Hoffman, Dieter E Jenne, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Larry Moreland, Philip Seo, Antoine Sreih, Steven R Ytterberg, Peter A Merkel, Ulrich Specks, Paul A Monach
OBJECTIVES: To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity. METHODS: Serum samples from patients with EGPA followed in a multi-center longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test...
March 1, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28273992/anca-associated-mononeuritis-multiplex-with-overlap-in-vasculitic-syndromes
#4
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/28261989/five-factor-score-more-than-1-is-associated-with-relapse-during-the-first-2-year-follow-up-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis
#5
Dae Sik Kim, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
AIM: We investigated what variables at diagnosis might be associated with the relapse of eosinophilic granulomatosis with polyangiitis (EGPA) and whether five factor score (FFS) at diagnosis might predict it. METHODS: We reviewed the medical records of 30 patients with EGPA having results of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA) and proteinase 3 (PR3)-ANCA and having achieved remission during the first therapeutic regimen administration within follow-up duration for at least more than 2 years...
March 5, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28260325/-a-case-of-eosinophilic-granulomatosis-with-polyangiitis-with-similar-clinical-presentation-of-acute-coronary-syndrome
#6
C D Zhang, S M Chen, C Sun
No abstract text is available yet for this article.
February 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/28255916/hospital-morbidity-database-for-epidemiological-studies-on-churg-strauss-syndrome
#7
Krzysztof Kanecki, Aneta Nitsch-Osuch, Paweł Gorynski, Patryk Tarka, Piotr Tyszko
Churg-Strauss syndrome or more accurately eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis with a characteristic late-onset allergic rhinitis and asthma. The use of hospital morbidity database is an important element of the epidemiological analysis of this rare disease. The present study was undertaken to assess the incidence of EGPA and factors related to its epidemiology in Poland; the first analysis of the kind in Poland, enabling a comparison in the European context...
March 3, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28255551/multiple-perforations-and-fistula-formation-following-corticosteroid-administration-a-case-report
#8
Jing-Ni He, Zhong Tian, Xu Yao, Hang-Yu Li, Yun Yu, Yuan Liu, Jin-Gang Liu
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPGA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction...
February 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28251013/antiproteinase-3-positive-eosinophilic-granulomatosis-with-polyangiitis-presenting-with-heart-failure-and-intraventricular-thrombosis
#9
Dan Zhu, Yiming Luo, Xiangyuan Liu, Lingyun Zu
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28229830/neutrophil-extracellular-traps-formation-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-association-with-eosinophilic-inflammation
#10
Joanna Natorska, Michał Ząbczyk, Jakub Siudut, Piotr Krawiec, Lucyna Mastalerz, Anetta Undas
OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with an inflammation and the presence of antineutrophil cytoplasmic antibodies (ANCA). Thus, we investigated the impact of ANCAs and eosinophilic inflammation on neutrophil activation and extracellular traps (NETs) formation. METHODS: We recruited 29 patients in the remission of EGPA (17 ANCA-negative and 12 ANCA-positive, including 7 p-ANCA-positive and 5 c-ANCA-positive patients). Healthy donors' neutrophils were stimulated with EGPA patients' serum...
February 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28229825/anca-associated-pauci-immune-glomerulonephritis-always-pauci-immune
#11
Valeria Scaglioni, Marina Scolnik, Luis J Catoggio, Silvia B Christiansen, Carlos F Varela, Gustavo Greloni, Guillermo J Rosa-Diez, Enrique R Soriano
OBJECTIVES: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is considered "pauci-immune" with absent or mild glomerular tuft staining for immunoglobulin (Ig) and/or complement. However, it is not unusual to see some immune deposits (ID) within glomeruli on immunofluorescence (IF). We determined to evaluate the prevalence and clinical significance of immune deposits in ANCA-associated GN. METHODS: We included all patients with ANCA associated vasculitis with renal biopsies between January 2002 and May 2014: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and renal limited vasculitis...
January 31, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28225490/clinical-characteristics-and-outcome-of-spanish-patients-with-anca-associated-vasculitides-impact-of-the-vasculitis-type-anca-specificity-and-treatment-on-mortality-and-morbidity
#12
MULTICENTER STUDY
Roser Solans-Laqué, Guadalupe Fraile, Monica Rodriguez-Carballeira, Luis Caminal, Maria J Castillo, Ferran Martínez-Valle, Luis Sáez, Juan J Rios, Xavier Solanich, Joaquim Oristrell, Francisco Pasquau, Eva Fonseca, Monica Zamora, Jose L Callejas, Begoña Frutos, Monica Abdilla, Patricia Fanlo, Isabel García-Sánchez, Miguel López-Dupla, Bernardo Sopeña, Almudena Pérez-Iglesias, Josep A Bosch
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28208913/eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hepatitis-b-infection-a-rare-association
#13
Nidhi Kaeley, Narula Kamakshi
Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. They include infections, allergic desensitization, vaccinations like hepatitis B and influenza vaccination, cocaine abuse, drugs like leukotriene receptor antagonists and exposure to pigeons...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28157661/eosinophilic-endomyocardial-disease
#14
Victor Y Liu, Raymundo A Quintana-Quezada, L Maximilian Buja
Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. The disease affects several organ systems and shares many features with more common conditions, making it a difficult diagnosis.
March 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28152445/heart-failure-not-responsive-to-standard-immunosuppressive-therapy-is-successfully-treated-with-high-dose-intravenous-immunoglobulin-therapy-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-egpa
#15
Antonio Pecoraro, Ludovica Crescenzi, Laura Carucci, Arturo Genovese, Giuseppe Spadaro
Glucocorticoids and immunosuppressive drugs represent the first-line treatment of eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome), even though the combined therapy is not successful in achieving the disease remission in some patients with neurological or cardiac involvement. We describe a case of an EGPA male patient with impaired left ventricular function not responsive to glucocorticoid and immunosuppressive therapy. We observed that high-dose (2g/kg/4weeks) intravenous immunoglobulin (IVIG) therapy significantly improved cardiac function, which was deteriorated after reducing IVIG dose at 0...
January 30, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28148583/anca-associated-vasculitis
#16
Max Yates, Richard Watts
The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome)...
February 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28131439/-antineutrophil-cytoplasmic-antibodies-associated-with-infective-endocarditis-literature-review
#17
V Langlois, I Marie
Antineutrophil cytoplasmic antibodies (ANCA) associated with infective endocarditis are a rare disorder. The condition can mimic primary systemic vasculitis (i.e. granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis). Thus, a wrong diagnosis of valvular involvement related to primary systemic vasculitis can be made in patients exhibiting ANCA associated with infective endocarditis. Because treatment of both conditions is different, this wrong diagnosis will lead to dramatic consequences in these latter patients...
January 25, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28119348/a-pragmatic-approach-to-vasculitis-in-the-gastrointestinal-tract
#18
REVIEW
Runjan Chetty, Stefano Serra
Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach...
January 24, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28108440/autoimmune-hepatitis-and-eosinophilic-granulomatosis-with-polyangiitis-a-rare-association
#19
Saroj Lohani, Salik Nazir, Niranjan Tachamo, Pavani Pagolu
We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. Her history was significant for asthma with recurrent exacerbations, polyneuropathy and recurrent sinus infections. Liver function tests revealed cholestasis. Antinuclear antibody and antismooth muscle cell antibody were positive. Liver biopsy revealed active chronic hepatitis with cholestasis. A diagnosis of autoimmune hepatitis was made. All other causes of cholestatic jaundice were ruled out...
January 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28094120/omalizumab-associated-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#20
Salik Nazir, Niranjan Tachamo, Shoaib Bilal Fareedy, Muhammad Sohail Khan, Saroj Lohani
No abstract text is available yet for this article.
March 2017: Annals of Allergy, Asthma & Immunology
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