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Churg Strauss Syndrome

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
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
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
X F Wang, D Y Xia, X Huang
No abstract text is available yet for this article.
August 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
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
Shri Ram Sharma, Hibu Habung, Masaraf Hussain
No abstract text is available yet for this article.
July 2016: Neurology India
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
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
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
J L Sánchez-Vicente, S Gálvez-Carvajal, A Medina-Tapia, T Rueda, L González-García, M Szewc, A Muñoz-Morales
CASE REPORT: We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. DISCUSSION: Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome...
April 8, 2016: Archivos de la Sociedad Española de Oftalmología
J Muto, N Fujimoto, K Ono, T Kobayashi, K R Chen, S Suzuki, H Wachi, S Tajima
BACKGROUND: Neutrophil elastase plays an important role in skin inflammation induced by neutrophil infiltration. Elafin is an inducible elastase inhibitor expressed by keratinocytes, and is known to be involved in pathogenesis of neutrophilic skin disorders such as psoriasis. METHODS: Immunohistochemical studies of elafin expression in the cases of vasculitis were performed. Induction of elafin expression in cultured vascular cells and its effect on neutrophil migration were studied in vitro...
September 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Yukihisa Ogawa, Hiroshi Nishimaki, Kiyoshi Chiba, Kenji Murakami, Yuka Sakurai, Keishi Fujiwara, Takeshi Miyairi, Yasuo Nakajima
PURPOSE: To describe the candy-plug technique using an Excluder aortic extender for distal occlusion of a large false lumen aneurysm in chronic aortic dissection. TECHNIQUE: A 60-year-old female patient with a history of chronic type B aortic dissection and high-dose steroid use for Churg-Strauss syndrome developed a large 6.2 cm maximum diameter false lumen aneurysm. She underwent thoracic endovascular aortic repair from the left common carotid artery to the descending aorta to cover the proximal entry at the level of distal arch, with coil embolization of the left subclavian artery...
June 2016: Journal of Endovascular Therapy
Haruki Koike
Non-systemic vasculitic neuropathy (NSVN) is a vasculitis confined to the peripheral nervous system. It is one of the most common causes of vasculitic neuropathy, along with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). It remains unclear whether this disease is truly an isolated nosological entity, or part of the spectrum of systemic vasculitides. Anti-neutrophil cytoplasmic antibodies (ANCA) are negative in NSVN even though the size of affected vessels in this disease is similar to that in ANCA-associated vasculitis...
March 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
B Chaigne, J Dion, L Guillevin, L Mouthon, B Terrier
Eosinophilic granulomatosis with polyangitis (EGPA) (formerly Churg-Strauss syndrome) is a rare small-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides. MPO-ANCA is present in only 31 to 38% of patients. In this review, we describe the pathophysiology of EGPA, which is characterized by a genetic predisposition, an environmental association, and a cellular dysfunction of eosinophils, neutrophils, and T and B cells.
May 2016: La Revue de Médecine Interne
Rashmi S Katre, Abhijit Sunnapwar, Carlos S Restrepo, Venkata S Katabathina, Amy Mumbower, Ameya Baxi, Sushilkumar Sonavane
Eosinophil-associated diseases (EADs) are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. Cardiopulmonary and gastrointestinal system involvement can be due to primary EAD with no known cause or can be secondary to known systemic disease. The cardiopulmonary spectrum of EADs comprises simple pulmonary eosinophilia, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis, parasitic infections, and idiopathic hypereosinophilic syndrome...
March 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
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