Atypical Onset of Eosinophilic Granulomatosis with Polyangitis in a Patient with Long-term Well-controlled Bronchial Asthma

Published on Jun 20, 2015in Genetics in Medicine8.904
· DOI :10.14442/GENERAL.16.99
Satoshi Akao (Okayama University), Hideharu Hagiya3
Estimated H-index: 3
(Okayama University)
+ 7 AuthorsFumio Otsuka35
Estimated H-index: 35
(Okayama University)
(Background) Many autoimmune diseases differ in individual of different races, but there has been very scarce information on the clinicopathological features of Churg Strauss syndrome (CSS) among Asians patients. (Objective) To clarify the clinicopathological details of Japanese CSS patients (Methods) The medical records of CSS patients hospitalized in l980-2007 were carefully reviewed. (Results) Seventeen patients fulfilled the Japanese Ministry of Health, Labour and Welfare (MHLW) criteria and...
7 Citations
#1Vincent Cottin (University of Lyon)H-Index: 78
#2Chahéra Khouatra (University of Lyon)H-Index: 19
Last. J.F. Cordier (University of Lyon)H-Index: 17
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Background: Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg–Strauss syndrome (CSS). Methods: We conducted a retrospective study of 24 consecutive patients (aged 41.1 ± 13.5 years) with CSS in a single center. All had asthma (starting 8.1 ± 9.5 years prior to the diagnosis of CSS), blood eosinophilia (6.1 ± 4.4 × 109/l) and systemic manifestations of CSS. Antineutrophil cytoplasmic antibodies were found in 7 of 22 tested patients. Seven patients...
31 CitationsSource
#1William DuMouchel (AT&T Labs)H-Index: 28
#2Eric T. Smith (RTP: Research Triangle Park)H-Index: 1
Last. June S. Almenoff (RTP: Research Triangle Park)H-Index: 7
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Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis (AGA), is a rare vasculitis that occurs in patients with bronchial asthma. The nature of the association of CSS with various asthma therapies is unclear. Objective: This study investigated the associations of different multidrug asthma therapy regimens and the reporting of AGA (the preferred code for CSS in the coding dictionary for the Adverse Event Reporting System [AERS]) by applying an iterative method of...
78 CitationsSource
#1C. BoccagniH-Index: 1
#2F. TesserH-Index: 5
Last. Francesco MonacoH-Index: 43
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Churg-Strauss syndrome (CSS) is a disseminated small vessel vasculitis characterized by late-onset asthma, upper airways disease, eosinophilia and late neurological manifestations such as peripheral neuropathy. Recently, several cases of CSS have been reported in patients treated with leukotriene antagonists after weaning corticosteroids. We describe a case of CSS developed while the patient was receiving montelukast for asthma treatment, after corticosteroids withdrawal. A causal relationship b...
11 CitationsSource
Churg-Strauss syndrome (CSS) is a primary systemic vasculitis occurring primarily in patients with asthma. Unlike other small vessel vasculitides, CSS is characterised by blood and tissue eosinophilia. Corticosteroids are the therapy of first choice for all stages of the disease when active vasculitis needs to be treated rapidly. In patients with severe disease and organ- or life-threatening manifestation, the addition of cyclophosphamide appears to improve the outcome and reduces the incidence ...
73 CitationsSource
#1Ghassan Jamaleddine (AUB: American University of Beirut)H-Index: 13
#2Karim A. Diab (AUB: American University of Beirut)H-Index: 11
Last. Thurayya Arayssi (AUB: American University of Beirut)H-Index: 20
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Abstract Background and Objectives: Leukotriene antagonists (LTAs), or antileukotrienes, are a new group of anti-inflammatory drugs used for the treatment of asthma. They might substitute for or allow tapering of corticosteroids in asthmatic patients. These drugs have been associated with the development of Churg-Strauss syndrome (CSS), a rare form of vasculitic angiitis. It is unclear whether the development of CSS is a direct drug effect or an unmasking of a preexisting condition on withdrawal...
67 CitationsSource
Abstract The pulmonary vasculitides are a group of rare but serious disorders that require early recognition, accurate diagnosis, and effective therapy. Churg-Strauss syndrome (CSS) is classified as small vessel vasculitis. Four different definitions for the diagnosis of CSS have been developed: (1) the pathologic criteria put forth by Churg and Strauss, (2) the criteria based on clinical grounds from Lanham and colleagues, (3) the criteria based on clinical grounds from the American College of ...
91 CitationsSource
Abstract The Churg-Strauss syndrome (CSS) is a distinct form of vasculitis that is notable for its eosinophilia and frequent associations with asthma and sinusitis. Because there has been an increasing recognition that CSS can develop in patients with asthma and that CSS might be associated with specific asthma treatments, the National Heart, Lung, and Blood Institute, the National Institute of Allergy and Infectious Diseases, the Office of Rare Diseases, National Institutes of Health, and the U...
173 CitationsSource
Antileukotriene drugs are new therapeutic agents that have recently been approved for the treatment of asthma. Several cases of eosinophilic conditions including Churg-Strauss syndrome have been reported to be associated with zafirlukast, a cysteinyl leukotriene type 1 receptor antagonist. So far no other leukotriene modifier has been associated with the syndrome. The case history is presented of a man with allergic rhinitis and asthma who had received intermittent pulse therapy with oral cortic...
86 CitationsSource
#1Loïc Guillevin (University of Paris)H-Index: 132
#2Pascal CohenH-Index: 48
Last. Philippe CasassusH-Index: 53
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