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https://www.readbyqxmd.com/read/28412705/interferon-%C3%AE-for-induction-and-maintenance-of-remission-in-eosinophilic-granulomatosis-with-polyangiitis-a-single-center-retrospective-observational-cohort-study
#1
Benjamin Seeliger, Martin Förster, Janett Happe, Thomas Forberg, Anne Moeser, Thomas Neumann, Claus Kroegel
OBJECTIVE: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA. METHODS: We conducted a retrospective monocentric cohort study including 30 patients (16 women) with active EGPA under IFN-α treatment...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28371513/early-outcomes-in-children-with-antineutrophil-cytoplasmic-antibody-anca-associated-vasculitis-aav
#2
Kimberly A Morishita, Lakshmi N Moorthy, Joanna M Lubieniecka, Marinka Twilt, Rae S M Yeung, Mary B Toth, Susan Shenoi, Goran Ristic, Susan M Nielson, Raashid A Luqmani, Suzanne C Li, Tzielan Lee, Erica F Lawson, Mikhail M Kostik, Marisa Klein-Gitelman, Adam M Huber, Aimee O Hersh, Dirk Foell, Melissa E Elder, Barbara A Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M Benseler, David A Cabral
Objective To characterize early disease course in childhood onset antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) and 12-month outcomes. Methods Eligible subjects were children diagnosed with GPA, MPA, EGPA, and ANCA-positive pauci-immune glomerulonephritis before their eighteenth birthday and entered into The Pediatric Vasculitis Initiative (PedVas) study. The primary outcome was remission (Pediatric Vasculitis Activity Score (PVAS) = 0 with corticosteroid dose (CS) <0.2mg/kg/day) at 12-months...
March 28, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28348913/a-rare-case-of-digital-ischemia-and-gangrene-in-anca-associated-vasculitis-with-review-of-the-literature
#3
Richard A Lau, Ramandeep Bains, Duminda Suraweera, Jane Ma, Emil R Heinze, Andrew L Wong, Philip J Clements
This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28339364/clinical-and-prognostic-features-of-korean-patients-with-mpo-anca-pr3-anca-and-anca-negative-vasculitis
#4
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/28337408/eosinophilic-granulomatosis-with-polyangiitis-formerly-known-as-churg-strauss-syndrome-as-a-differential-diagnosis-of-hypereosinophilic-syndromes
#5
Yuri Albuquerque Pessoa Santos, Bruno Rangel Antunes Silva, Pollyanna Natividade Zanconato Barros Assis Lira, Luiz Carlos Aguiar Vaz, Thiago Thomaz Mafort, Leonardo Palermo Bruno, Agnaldo José Lopes
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28328160/co-morbidities-in-severe-asthma-clinical-impact-and-management
#6
REVIEW
Celeste Porsbjerg, Andrew Menzies-Gow
Patients with severe asthma represent a minority of the total asthma population, but carry a majority of the morbidity and healthcare costs. Achieving better asthma control in this group of patients is therefore of key importance. Systematic assessment of patients with possible severe asthma to identify treatment barriers and triggers of asthma symptoms, including co-morbidities, improves asthma control and reduces healthcare costs and is recommended by international guidelines on management of severe asthma...
March 22, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28281453/iga-antibodies-to-myeloperoxidase-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss
#7
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
#8
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
#9
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/28255916/hospital-morbidity-database-for-epidemiological-studies-on-churg-strauss-syndrome
#10
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
#11
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
#12
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
#13
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/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
#14
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
#15
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/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
#16
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
#17
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/28087591/clinical-trials-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis-what-we-have-learnt-so-far-and-what-we-still-have-to-learn
#18
Divi Cornec, Emilie Cornec-Le Gall, Ulrich Specks
The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28079316/clinical-characteristics-and-treatment-response-of-peripheral-neuropathy-in-the-presence-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-experience-at-a-single-tertiary-center
#19
Hye Jin Cho, Sehyo Yune, Jin Myoung Seok, Eun Bin Cho, Ju Hong Min, Yeon Lim Seo, Byung Jae Lee, Byoung Joon Kim, Dong Chull Choi
BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014...
January 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28072629/the-key-clues-to-reach-the-diagnosis-of-loeffler-endomyocardial-fibrosis-associated-with-eosinophilic-granulomatosis-with-polyangiitis
#20
Enrico Ammirati, Domenico Sirico, Linda Brevetti, Laura Scudiero, Diana Artioli, Patrizia Pedrotti, Maria Frigerio
Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements...
January 7, 2017: Journal of Cardiovascular Medicine
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