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Churg Strauss Syndrome

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https://www.readbyqxmd.com/read/28540906/an-overlap-of-granulomatosis-with-polyangiitis-and-eosinophilic-granulomatosis-with-polyangiitis
#1
Sujit Surendran, Chandramohan Gundappa, Arun Gandhi, Anila Abraham Kurien, Edwin Fernando
We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA)...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28511440/portal-venous-thrombosis-eosinophilic-vasculitis
#2
Vasanthi Natarajan, David Jose, Kevin John, Ashok Kumar Das
Portal Vein Thrombosis (PVT) is caused by various thrombophilic states. PVT secondary to underlying vasculitis especially Churg-Strauss disease is among the rarest presentation. Here, we report a case of peripheral eosinophilia, eosinophilic ascitis and venous thrombosis involving portal vein and superior mesenteric vein diagnosed as Churg-Strauss Syndrome (CSS). He was managed with steroids and anticoagulants. Following initiation of steroids, eosinophilia and eosinophilic ascitis improved.
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28509134/two-cases-of-atypical-hemolytic-uremic-syndrome-ahus-and-eosinophilic-granulomatosis-with-polyangiitis-egpa-a-possible-relationship
#3
Mercedes Cao, Tamara Ferreiro, Bruna N Leite, Francisco Pita, Luis Bolaños, Francisco Valdés, Angel Alonso, Eduardo Vázquez, Juan Mosquera, María Trigás, Santiago Rodríguez
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28476172/anca-associated-vasculitis-in-childhood-recent-advances
#4
REVIEW
Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A Ramirez, Enrica P Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Giacomo Emmi, Pasquale Esposito, Mariagrazia Catanoso, Giancarlo Barbano, Alice Bonanni, Augusto Vaglio
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome...
May 5, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28365634/lesson-of-the-month-1-beware-the-atypical-presentation-eosinophilic-granulomatosis-with-polyangiitis-presenting-as-acute-coronary-syndrome
#5
Juergen Schiefermueller, Bashir Alaour, Alison Calver, Nick Curzen
We describe the case of a 45-year-old woman presenting with troponin positive cardiac-sounding chest pain. An initial emergency angiogram demonstrated two vessel coronary disease, including a distal right coronary artery occlusion. No percutaneous coronary intervention was performed and the patient was treated medically. At re-presentation with further pain a few days later, coronary angiography demonstrated no significant coronary lesions. After consideration of other multisystem symptoms and raised eosinophil count, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) presenting with coronary arteritis...
April 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28337408/eosinophilic-granulomatosis-with-polyangiitis-formerly-known-as-churg-strauss-syndrome-as-a-differential-diagnosis-of-hypereosinophilic-syndromes
#6
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/28321782/targeting-interleukin-5-or-interleukin-5r%C3%AE-safety-considerations
#7
REVIEW
Diego Bagnasco, Matteo Ferrando, Marco Caminati, Alice Bragantini, Francesca Puggioni, Gilda Varricchi, Giovanni Passalacqua, Giorgio Walter Canonica
Asthma is a highly prevalent chronic disease of the airways; approximately 10% of patients with asthma will experience a severe form of the disease. New understanding of the pathogenesis of asthma has enabled the development of novel drugs and provided hope for patients with asthma. Interleukin (IL)-5 and IL-5 receptor subunit α (IL-5-Rα) plays a crucial role in the development, maturation, and operation of eosinophils so were the first important therapeutic target of these new drugs. While the results of early clinical trials of these drugs were not promising, results improved once researchers discovered the drugs worked best in patients with high eosinophil levels...
March 20, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
https://www.readbyqxmd.com/read/28255916/hospital-morbidity-database-for-epidemiological-studies-on-churg-strauss-syndrome
#8
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
#9
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/28208913/eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hepatitis-b-infection-a-rare-association
#10
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/28205199/heart-involvement-in-churg-strauss-syndrome
#11
Sigita Glaveckaite, Nomeda Valeviciene, Darius Palionis, Egle Kontrimaviciute, Eugenijus Lesinskas
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28185705/cardiovascular-magnetic-resonance-imaging-pattern-at-the-time-of-diagnosis-of-treatment-na%C3%A3-ve-patients-with-connective-tissue-diseases
#12
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Theodoros Dimitroulas, Konstantinos Bratis, George D Kitas, Petros Sfikakis, Maria Tektonidou, Georgia Karabela, Efthymios Stavropoulos, Gikas Katsifis, Kyriaki A Boki, Anastasia Kitsiou, Vasiliki Filaditaki, Elias Gialafos, Sotiris Plastiras, Vasiliki Vartela, Genovefa Kolovou
BACKGROUND-AIM: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]...
February 1, 2017: International Journal of Cardiology
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
#13
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
#14
REVIEW
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/28119348/a-pragmatic-approach-to-vasculitis-in-the-gastrointestinal-tract
#15
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/28098669/eye-problems-in-a-woman-with-churg-strauss-syndrome
#16
Benjamin D Pierce, Carrlene B Donald, Anthony P Mendez
Churg-Strauss syndrome is a rare, systemic vasculitis of unknown cause. Ocular involvement is a rare but established complication and can lead to vision damage or blindness if not treated promptly. Treatment of ocular manifestations corresponds with systemic treatment of the disease and consists primarily of corticosteroids.
February 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#17
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28094120/omalizumab-associated-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#18
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
https://www.readbyqxmd.com/read/28089164/low-median-nerve-palsy-as-initial-manifestation-of-churg-strauss-syndrome
#19
Young Hak Roh, Young Do Koh, Jung Ho Noh, Hyun Sik Gong, Goo Hyun Baek
Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage...
January 11, 2017: Journal of Hand Surgery
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
#20
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
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