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Justin C Chia, P Régine Mydlarski
PURPOSE: Omalizumab is a recombinant humanized monoclonal antibody that inhibits the binding of IgE to the high-affinity IgE receptor (FceRI) on the surface of mast cells and basophils. Omalizumab has been approved for use in asthma, and new reports show promise in a variety of dermatologic diseases. Herein, we review the literature on omalizumab in dermatology and discuss the safety, efficacy and mechanisms of action for this emerging therapy. MATERIALS AND METHODS: PubMED, MEDLINE, and Embase databases were searched for the period January 1, 1990, to September 1, 2016...
October 19, 2016: Journal of Dermatological Treatment
Christian Pagnoux
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated...
September 2016: Eur J Rheumatol
A Singh, P Jain, A Sharma, M Ali, V Arya
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Nobuyuki Masaki, Ami Issiki, Masato Kirimura, Tetsuo Kamiyama, Osamu Sasaki, Hiroyuki Ito, Yoshiaki Maruyama, Toshihiko Nishioka
Eosinophilic myocarditis may be accompanied by Churg-Strauss syndrome (CSS). We report a case of CSS that was accompanied by myocardial changes in the early stage. A 71-year-old woman complained of mild chest pain at rest, but routine echocardiography did not reveal any endocardial abnormalities. Four months later, the patient was hospitalized due to congestive heart failure with neuropathy of both upper extremities. A diagnosis of eosinophilic myocarditis was made based on the patient's laboratory results and the presence of mural thrombus...
2016: Internal Medicine
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
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
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
Margarida Pujol-López, Sergio Prieto-González, Susanna Prat-González, José Hernández-Rodríguez
No abstract text is available yet for this article.
August 29, 2016: Medicina Clínica
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
Ani A Bodoutchian, Hitender Jain, Tania Velez
BACKGROUND: Cardiomyopathy in patients with Churg-Strauss syndrome (CSS) carries a poor prognosis, with a high 5-year mortality rate, and requires treatment with immunosuppressive therapy. There is no single pathognomonic test or clinical finding for diagnosing CSS; instead, it is based upon meeting four of six criteria. CASE REPORT: A 47-year-old woman with a 6-month medical history of "asthma" presented to our hospital with a 1-month history or dyspnea on exertion...
September 2016: Journal of Emergency Medicine
Gokhan Sargin, Taskin Senturk
The first International Chapel Hill Consensus Conference (CHHC) was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second CHHC 2012, which were not included in the CHCC 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener"s. New categories such as Behçet"s and Cogan etc. were also added...
June 26, 2016: Revista Brasileira de Reumatologia
Shri Ram Sharma, Hibu Habung, Masaraf Hussain
No abstract text is available yet for this article.
July 2016: Neurology India
Magdalena Pirowska, Aleksander Obtułowicz, Grzegorz Dyduch, Sylwia Lipko-Godlewska, Anna Wojas-Pelc
INTRODUCTION: Palisaded neutrophilic and granulomatous dermatitis (PNGD) are terms which include such diseases as rheumatoid nodules, Churg-Strauss granuloma, and interstitial granulomatous dermatitis with arthritis. This heterogeneous group was first described in 1965. It is associated with immunological diseases. There are reported cases of coexistence with systemic lupus erythematosus, rheumatoid arthritis, Wegener's granuloma, inflammatory bowel disease, generalized vascular inflammation, and lymphoproliferative disorders...
June 2, 2016: Annals of Agricultural and Environmental Medicine: AAEM
T V Beketova, M Yu Volkov, E L Nasonov
The 2015 international guidelines for the diagnosis and treatment of eosinophilic granulomatosis with polyangiitis, formerly known as the Churg-Strauss syndrome, are detailed and an attempt is made to expand an evidence base, by attracting more recent available publications. The new guidelines should not be regarded as final standards; these are primarily to extend the possibilities of choosing a personified management strategy for patients with eosinophilic granulomatosis with polyangiitis and to serve as the starting point for further in-depth investigations...
2016: Terapevticheskiĭ Arkhiv
Paula R David, Amir Dagan, Maartje Colaris, Mintsje de Boer, Jan W Cohen Tervaert, Yehuda Shoenfeld
No abstract text is available yet for this article.
March 2016: Israel Medical Association Journal: IMAJ
Solen Kernéis, Clément Turbelin, Christian Pagnoux, Odile Launay, Alfred D Mahr, Loïc Guillevin, Pierre-Yves Boëlle, Thomas Hanslik
OBJECTIVES: To estimate the impact of vaccinations, infections and traumatic life events on the disease activity of a web-based cohort of systemic necrotising vasculitis (SNV) patients. METHODS: Adults diagnosed with SNV self-reported vaccinations, infectious episodes and traumatic life events every 3 months during follow-up on a secure dedicated website. Participants reported information on disease activity assessed with 3 scores: the French version of the Medical Outcome Study Short Form-36 (SF-36), the visual numerical scale for Patient Global Assessment (PGA) and the modified Disease Extent Index (mDEI)...
May 2016: Clinical and Experimental Rheumatology
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
Rossella Reggia, Chiara Bazzani, Laura Andreoli, Mario Motta, Andrea Lojacono, Sonia Zatti, Francesca Ramazzotto, Monica Nuzzo, Angela Tincani
PROBLEM: Cyclosporin A (CYS A) is an immunosuppressant agent administered in autoimmune diseases, and its use during pregnancy and lactation is a debated topic. METHOD OF STUDY: The demographic characteristics, the activity of the underlying disease, and the onset of fetal-maternal complications have been investigated in 21 consecutive patients (2 RA, 14 SLE, 2 PA, 1 SjS, 1 DM, 1 Churg-Strauss vasculitis), treated with CYS A throughout 29 gestations. A subanalysis of the SLE group was performed...
June 2016: American Journal of Reproductive Immunology: AJRI
G Shojaati, K Chaloupka
BACKGROUND: There are many different aetiologies for acute lid swelling, including infection, inflammation, degeneration, tumours and trauma. We present five uncommon cases of acute lid swelling and give an overview of differential diagnoses for rapidly evolving eyelid swelling. HISTORY AND SIGNS: We reviewed the charts of five patients with initial presentation of acute lid swelling. For the five patients, there were diagnoses of Churg Strauss syndrome, an allergic reaction to hyaluronic acid, lymphangioma, a ruptured dermoid cyst and a co-infected pre-existing orbital lesion that was only evident in the follow-up...
April 2016: Klinische Monatsblätter Für Augenheilkunde
Veronica Seccia, Susanna Fortunato, Lodovica Cristofani-Mencacci, Iacopo Dallan, Augusto P Casani, Manuela Latorre, Pierluigi Paggiaro, Maria Laura Bartoli, Stefano Sellari-Franceschini, Chiara Baldini
OBJECTIVES/HYPOTHESIS: To evaluate the clinical features of audiologic impairment and its relationship with the nasal, vestibular, and rheumatologic profile in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome. STUDY DESIGN: Prospective cross-sectional study. METHODS: Thirty-nine patients with EGPA, considered controlled according to the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index, underwent a complete audiologic evalutaion with otomicroscopy, impedance audiometry, speech audiometry, and auditory brainstem responses; rhinologic evaluation was made by means of fiberoptic endoscopy and nasal cytology; the clinical evaluation was completed with analysis of the facial function and, in patients with referred vertigo, with videonystagmography (VNG)...
April 14, 2016: Laryngoscope
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