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Churg Strauss Syndrom

E d'Ersu, C Ribi, P Monney, G Vincenti, J Schwitter, S Rotman, R Hullin, J Regamey
This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T2 -weighted imaging sequences for quantification of myocardial scar tissue and quantitative T2 mapping techniques, which allow the detection of myocardial edema.
February 3, 2018: International Journal of Cardiology
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
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...
January 23, 2018: International Archives of Allergy and Immunology
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. This article is protected by copyright. All rights reserved.
February 2, 2018: American Journal of Transplantation
O Syniachenko, O Khaniukov, Ye Yehudina, H Taktashov, K Gashynova
The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1...
December 2017: Georgian Medical News
Woo Cheal Cho, Bharat Ramlal, Mary Fiel-Gan, Xianyuan Song
No abstract text is available yet for this article.
December 18, 2017: Journal of Pathology and Translational Medicine
Tyler Safran, Marina Masckauchan, Jakub Maj, Lawrence Green
Vaccinations have been shown repeatedly to be extremely safe with low incidence of complications. Given the rarity, these adverse events, they must be reported and examined cautiously. This case report illustrates the first case of an adult presenting with Wells syndrome that developed soon after vaccination with the thiomersal, a common preservative, containing influenza vaccine. Wells syndrome, also known as eosinophilic cellulitis, is an uncommon dermatologic condition of unknown etiology. Lesions in patients with Wells syndrome (eosinophilic cellulitis) progress over a few days to become large indurated plaques with associated edema and erythema...
December 14, 2017: Human Vaccines & Immunotherapeutics
Vyacheslav М Zhdan, Yevdokiia М Kitura, Oksana Ye Kitura, Maryna Yu Babanina, Maksym V Tkachenko, Volodymyr G Lebid
A clinical case of Churg-Strauss syndrome has been reported on the 53-year-old female patient Ts. with bronchial asthma and allergic rhinitis. The main clinical signs and syndromes depending on the stage of the disease are presented, as well as therapeutic treatment of patients with this disease.
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Yoon Seob Kim, Yu Mee Song, Hyun-Min Seo, Chul Hwan Bang, Ji Hyun Lee, Jun Young Lee, Young Min Park
No abstract text is available yet for this article.
December 2017: Annals of Dermatology
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
Christopher C Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau, Luke Y C Chen
Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential...
November 2017: American Journal of the Medical Sciences
Ronan Bluett, David McDonnell, Claire O'Dowling, Carl Vaughan
We present the case of a 28-year-old man who presented with chest pain and elevated cardiac biomarkers, with no evidence of acute ischaemia. He had a pronounced eosinophilia, abnormal echocardiographic, cardiac MRI and CT findings. He underwent transbronchial biopsy of carinal lymph nodes and of lung parenchyma. Endomyocardial biopsy yielded an eosinophilic infiltrate. He was treated with high dose glucocorticoids and made a rapid recovery. Testing for FIP1L1-PDGFRA and other BCR-ABL1 mutations was negative...
November 23, 2017: BMJ Case Reports
Stefanie Plenzig, Sara Heinbuch, Hannelore Held, Marcel A Verhoff, Constantin Lux
Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. To rule out the possibility of medical malpractice the prosecuting authority ordered a forensic autopsy. At autopsy macroscopic indicators for perimyocarditis and pneumonia were found...
December 2017: Forensic Science, Medicine, and Pathology
A Jerrentrup, T Müller, J Mariss, S Dylla
This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years...
October 5, 2017: Der Internist
David Aguirre-Valencia, Iván Posso-Osorio, Juan-Carlos Bravo, Fabio Bonilla-Abadía, Gabriel J Tobón, Carlos A Cañas
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms...
September 2017: Clinical Rheumatology
Dayun Park, Ho Jun Lee, Kwang Hoon Lee, Bum Sun Kwon, Jin-Woo Park, Ki Yeun Nam, Kyoung Hwan Lee
Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Resection of a mediastinal mass, which was diagnosed as thymic neuroendocrine carcinoma, was performed five months before his visit...
June 2017: Annals of Rehabilitation Medicine
Xuanyi Jin, Chunyan Ma, Shuang Liu, Zhengyu Guan, Yonghuai Wang, Jun Yang
Hypereosinophilia-associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA...
August 2017: Echocardiography
Nurashikin Mohammad, Wan Syamimee Wan Ghazali
We report a case of 28-year-old Malay woman who initially presented with multiple joints pain with underlying poorly controlled asthma since her childhood. She was treated as seronegative arthritis. However, the involvement of joints, asthma and high-eosinophil counts raised suspicion of Churg-Strauss syndrome. This disease is undoubtedly rare in Malaysians or even in Asian populations. After appropriate therapy was delivered for the correct diagnosis her clinical condition improved. She is currently receiving maintenance treatment...
May 27, 2017: BMJ Case Reports
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
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
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