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Article Abstract
Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.
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The woman with a swollen tongue : A rare case of an ANCA-negative localized eosinophil-rich granulomatous inflammation affecting solely the tongue.
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September 2025
Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital and University of Zurich, Zurich, Switzerland.
Eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often presenting with chronic rhinosinusitis, pulmonary infiltrates, gastrointestinal and cardiac disorders, or renal lesions. Sinonasal and inner ear manifestations are common, but other affections of the head and neck area are rarely reported. Here we report a case of a young woman with a histopathological diagnosis of eosinophil-rich granulomatous inflammation affecting solely the tongue without other local or systemic lesions.
View Article and Find Full Text PDFClinical value of biomarkers in relation to artery size in eosinophilic granulomatosis with polyangiitis: findings from an inception cohort at a Japanese City Hospital.
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Department of Rheumatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.
Introduction/objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that frequently affects small arteries in the skin and the nerve but can also involve the medium-sized arteries, resembling polyarteritis nodosa (PAN). This study aimed to evaluate the clinical value of biomarkers in EGPA in relation to the size of the affected arteries.
Method: In an inception cohort of EGPA, we examined the relationships between eosinophil counts, myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, levels of rheumatoid factor (RF) and Immunoglobulin E (IgE) and pre-defined outcomes based on artery size.
Unique case of mononeuritis multiplex in a patient of Eosinophilic granulomatosis with polyangiitis.
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Department of Pulmonary Medicine, Narendra Modi Medical College L.G. Hospital, Gujarat, India.
Introduction: Eosinophilic granulomatosis with polyangiitis (formerly known as Churg Strauss syndrome) is a rare multisystem autoimmune disease. It is known to affect 1-3 adults/million population worldwide.
Case: A 50-year-old woman presented to the emergency room with chief complaints of Cough with expectoration since last 7 days and right Lower limb pain and weakness since last 2 days followed by left upper limb pain and weakness since last 1 day.
Immunological Markers Associated with Skin Manifestations of EGPA.
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Department of Clinical and Experimental Medicine, School and Unit of Allergy and Clinical Immunology, "G. Martino" Hospital, University of Messina, 98124 Messina, Italy.
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic vasculitis with eosinophilic inflammation and variable clinical presentations. Although skin manifestations are frequent, current classification criteria do not include them, which may underestimate their diagnostic value. This prospective observational study aimed to assess systemic and skin involvement as well as eosinophilia, anti-neutrophil cytoplasmic antibody (ANCA), and Anti-nuclear antibodies (ANA) serum levels in 20 EGPA patients followed for one year at the University Hospital of Messina, Italy, before starting Mepolizumab, 300 mg.
View Article and Find Full Text PDFAnti-IL5/IL-5 receptor therapies for eosinophilic granulomatosis with polyangiitis: an updated Systematic Review.
Front Immunol
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Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.
Introduction: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare necrotizing vasculitis characterized by eosinophilic inflammation that was traditionally treated with corticosteroids associated with other immunosuppressants. Over the last years different biological therapies targeting IL-5/IL-5 receptor have become available and have been employed to tackle this challenging condition. Aim of the present study is to synthesis the evidence on the clinical presentation of this disease and on the efficacy of the newly available therapeutic strategies.
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