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https://www.readbyqxmd.com/read/29778666/mycophenolate-mofetil-for-induction-and-maintenance-of-remission-in-na%C3%A3-ve-patients-with-granulomatosis-with-polyangiitis-without-renal-involvement
#1
Yasser Emad, Yasser Ragab, Johannes J Rasker
Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up...
May 16, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29754328/hbsag-negative-and-anti-hbc-positive-in-eosinophilic-granulomatosis-with-polyangiitis-a-retrospective-pilot-study
#2
Sang-Won Lee, Do Young Kim, Sang Hoon Ahn, Yong-Beom Park, Kwang-Hyub Han, Jun Yong Park
We examined whether resolved hepatitis B virus (HBV) infection was associated with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), and affected AAV activity at diagnosis and prognosis during the follow-up. We reviewed the electronic medical records of 153 AAV patients, and included 91 hepatitis B surface antigen (HBsAg)-negative patients having results of both antibody to hepatitis B core antigen (anti-HBc) and surface antigen (anti-HBs). We collected clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis and relapse rates during the follow-up...
May 12, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29730524/mortality-in-systemic-necrotizing-vasculitides-a-retrospective-analysis-of-the-french-vasculitis-study-group-registry
#3
REVIEW
Sabine Jardel, Xavier Puéchal, Alain Le Quellec, Christian Pagnoux, Mohamed Hamidou, François Maurier, Olivier Aumaitre, Achille Aouba, Thomas Quemeneur, Jean-François Subra, Vincent Cottin, Jean Sibilia, Pascal Godmer, Patrice Cacoub, Anne Laure Fauchais, Eric Hachulla, Delphine Maucort-Boulch, Loïc Guillevin, Jean-Christophe Lega
OBJECTIVE: The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010...
May 3, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29709930/a-case-of-dilated-cardiomyopathy-with-eosinophilic-granulomatosis-with-polyangiitis-in-which-active-myocardial-inflammation-was-only-detected-by-endomyocardial-biopsy
#4
Takafumi Nakayama, Shunsuke Murai, Nobuyuki Ohte
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of systemic vasculitis. Cardiac involvement is the main cause of death in patients with this disease. We herein report a case of congestive heart failure in a patient with EGPA. Neither 67 Ga scintigraphy nor cardiac magnetic resonance imaging detected inflammation of the myocardium; however, myocardial biopsy revealed numerous infiltrating inflammatory cells, thereby fulfilling the criteria of inflammatory dilated cardiomyopathy. We improved the LV systolic function by increasing the patient's prednisolone dosage...
April 27, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29652700/overall-disability-sum-score-for-clinical-assessment-of-neurological-involvement-in-eosinophilic-granulomatosis-with-polyangiitis
#5
Roberto Padoan, Maddalena Marconato, Mara Felicetti, Francesco Cinetto, Mariachiara Cerchiaro, Francesca Rizzo, Renzo Marcolongo, Leonardo Punzi, Carlo Agostini, Franco Schiavon
AIM: The aim of this study was to verify the application of Overall Disability Sum Score (ODSS) for standardized clinical assessment of neurological involvement in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and its correlation with treatment response and long-term outcomes. METHODS: Consecutive EGPA patients referred to our tertiary vasculitis center were retrospectively evaluated. Patients' neurological damage and disability were systematically assessed with Vasculitis Damage Index and ODSS...
April 13, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29643885/anca-associated-vasculitis-in-patients-from-saudi-arabia
#6
Abdurhman Saud Al Arfaj, Najma Khalil
Objective: To explore clinical and laboratory features, therapy and outcome of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) patients from our tertiary care center. Methods: This study of AAV patients seen in Rheumatology clinics at King Khalid University hospital (KKUH), King Saud University, Riyadh during the period 1990-2014 was carried out retrospectively. Demographic, clinical, haematological and immunological parameters along with therapy, complications and outcome were retrieved from patients' medical charts...
January 2018: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29611402/delta-neutrophil-index-is-associated-with-vasculitis-activity-and-risk-of-relapse-in-anca-associated-vasculitis
#7
Juyoung Yoo, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong Beom Park, Sang Won Lee
PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA)...
May 2018: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29611207/immunological-and-hematological-effects-of-il-5-r%C3%AE-targeted-therapy-an-overview
#8
REVIEW
M Hassani, L Koenderman
IL-5 is an important cytokine for priming and survival of mature eosinophils, and for proliferation and maturation of their progenitors. Hence, IL-5(Rα) targeting will be increasingly used in diseases where eosinophils are the key immune effector cells such as eosinophilic asthma (EA), hyper eosinophilic syndrome (HES), eosinophilic esophagitis (EE) and eosinophilic granulomatosis with polyangitis (EGPA). Therefore, several neutralizing monoclonal antibodies directed against IL-5 (mepolizumab and reslizumab) and its receptor IL-5Rα (benralizumab) have found or will find their way to the clinic...
April 3, 2018: Allergy
https://www.readbyqxmd.com/read/29611001/eosinophilic-granulomatosis-with-polyangiitis-newer-therapies
#9
REVIEW
Erika P Navarro-Mendoza, Gabriel J Tobón
PURPOSE OF REVIEW: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic disseminated vasculitis associated with extravascular granulomas in patients suffering from asthma and tissue eosinophilia. Current therapies to achieve remission and prevent relapse include glucocorticoids and immunosuppressants like cyclophosphamide. RECENT FINDINGS: With the right treatment, clinical prognosis is favorable, so concerted efforts have been made in recent years to find new alternatives for treating severe EGPA...
April 2, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29569004/-manifestation-of-eosinophilic-granulomatosis-with-polyangiitis-in-the-head-and-neck-area-over-time-taking-systemic-disease-activity-into-consideration
#10
Henrik Andersen, Paul Götz, Jan Phillip Bremer, Martin Laudien
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. A regular involvement of the head and neck area is known. Since it is not yet known whether the inflammatory processes in the head and neck area are parallel to the systemic disease activity, the course of systemic and local disease activity was investigated in a comparative study. MATERIAL AND METHODS: Between 1990 and 2010 the disease progression of 25 patients with a total of 90 consultations and an average observation period of 7...
March 22, 2018: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29564565/imatinib-mesylate-use-in-refractory-eosinophilic-granulomatosis-with-polyangiitis-a-literature-review-and-a-case-report
#11
REVIEW
Tatiana V Beketova, Mikhail Y Volkov, Evgeniy A Naryshkin, Tatiana M Novoselova, Evgeniy L Nasonov
Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate...
March 21, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29560002/a-rare-case-of-eosinophilic-granulomatosis-with-polyangiitis-associated-with-cryoglobulinemia-presenting-with-a-bullous-skin-eruption-of-the-lower-limbs
#12
D D K Abeyaratne, C Liyanapathirana, C L Fonseka, P W M C S B Wijekoon
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29525845/anti-neutrophil-cytoplasmic-antibodies-and-their-clinical-significance
#13
Supaporn Suwanchote, Muanpetch Rachayon, Pongsawat Rodsaward, Jongkonnee Wongpiyabovorn, Tawatchai Deekajorndech, Helen L Wright, Steven W Edwards, Michael W Beresford, Pawinee Rerknimitr, Direkrit Chiewchengchol
Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical...
April 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29469750/a-case-of-probable-trimethoprim-sulfamethoxazole-induced-circulating-antineutrophil-cytoplasmic-antibody-positive-small-vessel-vasculitis
#14
Therese Woodring, Ronnie Abraham, Stephanie Frisch
Cutaneous leukocytoclastic vasculitis (LCV) can occur as skin-limited disease or as part a systemic vasculitis. Appropriate workup includes the evaluation of antineutrophil cytoplasmic antibodies (ANCAs), with a positive titer raising concern for the associated primary vasculitides including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), or eosinophilic granulomatosis with polyangiitis (EGPA). In the absence of systemic findings, however, a drug etiology must also be considered. Tumor necrosis factor (TNF) inhibitors, propylthiouracil, levamisole-adulterated cocaine, hydralazine, and minocycline have been previously documented to induce ANCA-positive vasculitis (APV), which may present with conspicuously high ANCA titers...
August 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29465370/the-initial-predictors-of-death-in-153-patients-with-anca-associated-vasculitis-in-a-single-korean-centre
#15
Chin Hee Mun, Juyoung Yoo, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We estimated the cumulative patient survival rates, the causes of death and the initial predictors of death in Korean patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). METHODS: We reviewed the medical records of 153 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We collected clinical and laboratory data including ANCA, Birmingham vasculitis activity score (BVAS), five factor score (FFS) (2009), comorbidities, medications and prognosis (death and relapse)...
February 14, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29465366/cancer-development-in-korean-patients-with-anca-associated-vasculitis-a-single-centre-study
#16
Juyoung Yoo, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We investigated the incidence rate and type of cancer, and furthermore, estimated the standardised incidence ratios (SIRs) of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Korea. METHODS: We retrospectively included 150 patients with AAV [81 patients with microscopic polyangiitis (MPA), 38 with granulomatosis with polyangiitis (GPA) and 31 patients with eosinophilic GPA (EGPA)], and reviewed their medical records...
February 14, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29409373/epidemiology-of-primary-systemic-vasculitis-in-children-a-population-based-study-from-southern-sweden
#17
M Mossberg, M Segelmark, R Kahn, M Englund, A J Mohammad
OBJECTIVES: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. METHODS: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register...
February 7, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29404165/clinical-and-serological-features-of-eosinophilic-and-vasculitic-phases-of-eosinophilic-granulomatosis-with-poliangiitis-a-case-series-of-15-patients
#18
İnsu Yılmaz, Nuri Tutar, Zuhal Özer Şimşek, Fatma Sema Oymak, İnci Gülmez
OBJECTIVES: Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment. MATERIALS AND METHODS: We included 15 EGPA patients in the study. The clinical, serological, and therapeutic characteristics and the treatment responses of the patients were recorded...
July 2017: Turkish Thoracic Journal
https://www.readbyqxmd.com/read/29393242/focus-on-the-involvement-of-the-nose-and-paranasal-sinuses-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-nasal-cytology-reveals-infiltration-of-eosinophils-as-a-very-common-feature
#19
Veronica Seccia, Chiara Baldini, Manuela Latorre, Matteo Gelardi, Iacopo Dallan, Lodovica Cristofani-Mencacci, Stefano Sellari-Franceschini, Maria Laura Bartoli, Elena Bacci, Pierluigi Paggiaro
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. However, the actual clinical features, cellular mechanisms and impact on patients' quality of life (QoL) are still a matter of study...
2018: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29392845/recurrence-of-eosinophilic-granulomatosis-with-polyangitis-after-orthotopic-heart-transplant
#20
Ujjwal Rastogi, Ziad Sergie, Sean Pinney, Noah Moss
Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft.
February 2, 2018: American Journal of Transplantation
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