A patient with a two-year history of worsening asthma presented with chest pain and shortness of breath. She developed cardiogenic shock. Analysis of blood chemistry detected increased troponin I concentration. Her electrocardiographic changes were consistent with a diagnosis of anteroseptal myocardial infarction. However, angiography showed normal coronary arteries. Left ventriculography showed severe mitral regurgitation and global hypokinesis. Peripheral eosinophilia was detected. Subsequent endomyocardial biopsy showed myocarditis with prominent eosinophil and plasma cell components. Churg-Strauss syndrome was diagnosed based on her history of asthma, evidence of peripheral eosinophilia and results of endomycardial biopsy. Treatment with a high dose of corticosteroids was initiated. As symptoms of heart failure improved - without recurrence of cardiac and respiratory symptoms - the dose of corticosteroids was gradually reduced. Eight months after her original presentation, she developed urticarial lesions on her abdomen and legs, with muscle soreness but no other associated symptoms. She was treated with a combination of prednisone and dapsone. After the diagnosis of Churg-Strauss syndrome, the patient remained symptom free with a normal ejection fraction for 15 months while taking prednisone.
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Vasc Health Risk Manag
December 2024
Inner Mongolia Medical University Ordos School of Clinical Medicine, Ordos, 017000, People's Republic of China.
Objective: To explore the clinical characteristics and treatment outcomes of intracerebral hemorrhage in eosinophilic granulomatosis with polyangiitis (EGPA).
Methods And Patient Presentation: We report an 18-year-old student of EGPA complicated with intracerebral hemorrhage. The laboratory tests showed a continuous increase in eosinophils.
Arerugi
December 2024
Department of Otorhinolaryngology-Head and Neck Surgery, Graduate School of Medicine, The University of Tokyo.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease with marked elevation of blood eosinophil associated with various symptoms in the respiratory tract, gastrointestinal tract, peripheral nerves, and skin due to systemic vasculitis. Here, we report a case of EGPA in which rhinosinusitis was treated with endoscopic sinus surgery (ESS) with good results. A 49-year-old man who developed bronchial asthma at age 48 was diagnosed with EGPA in our hospital.
View Article and Find Full Text PDFLaryngoscope
November 2024
Department of Otolaryngology Head and Neck Surgery, University Hospital of Modena, Modena, Italy.
Eosinophilic granulomatosis with polyangiitis (EGPA), among various organs and systems, can affect the upper respiratory tract. The otolaryngologist must be able to suspect the pathology with the appearance of the first signs and recognize its late complications. Laryngeal involvement is rare and difficult to diagnose.
View Article and Find Full Text PDFANCA-associated vasculitides (AAV) are small-vessel necrotizing vasculitides, with no or few immune deposits. They are usually associated with the presence of ANCA antibodies (AntiNeutrophil Cytoplasmic Antibody), targeted either against proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). ANCA-associated vasculitides include granulomatosis with polyangiitis (formerly Wegener's), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome).
View Article and Find Full Text PDFJ Autoimmun
December 2024
Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, F-75014 Paris, France; Paris Cité University, F-75006, Paris, France. Electronic address:
Objectives: Current guidelines suggest treating poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) with a combination of glucocorticoids (GCs) plus cyclophosphamide (CYC). However, there is little data to support the need for the addition of CYC. The objective of this study was to compare GCs plus CYC to GCs alone as induction therapy in poor-prognosis EGPA.
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