Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium sized vessels vasculitis. Vasculitis commonly affects lung, heart, skin and peripheral nerves. The authors report a case of a 47-year-old woman that was admitted with a 1-week history of painful purpuric lesions affecting extensor surfaces of lower extremities. She had a 7 years-long medical history of asthma, paranasal sinusitis and allergic rhinitis. Laboratorial tests revealed leucocytosis, peripheral blood eosinophilia and elevated inflammatory parameters. Antineutrophil cytoplasmic antibodies were negative. Skin biopsy was consistent with necrotizing vasculitis with perivascular eosinophil-rich infiltrate. Electromyogram and nerve conduction studies showed asymmetrical peripheral polyneuropathy. Churg-Strauss syndrome diagnosis was supported by clinical and histopathological findings. High-dose oral steroid therapy led to a dramatic clinical improvement. Skin manifestations can be the presenting feature of Churg-Strauss syndrome. Early recognition is essential for appropriate treatment and prevention of irreversible organ damage.
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Arthritis Res Ther
January 2025
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, the Ministry of Education Key Laboratory, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Objective: Severe gastrointestinal lesions are associated with a poor prognosis in eosinophilic granulomatosis with polyangiitis (EGPA). The goal of this study was to develop an effective predictive model for gastrointestinal lesions and to examine clinical patterns, associated factors, treatment, and outcomes of gastrointestinal lesions in EGPA.
Methods: We retrospectively enrolled 165 EGPA patients.
Pediatr Rheumatol Online J
January 2025
Department of Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Besevler, 06500, Turkey.
Background: Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.
Main Body: Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition.
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.
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