Asthmatic granulomatosis (AG) is a variant of severe asthma, first described in 2012, that consists of small airway changes compatible with asthma as well as interstitial nonnecrotizing granulomas. Treatment of AG requires immunosuppression as opposed to the traditional asthma therapy of inhaled steroids. We describe a 5-year course of a patient with AG that has shown no improvement with immunosuppression or other standards of therapy.
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http://dx.doi.org/10.1080/08998280.2018.1484221 | DOI Listing |
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.
SAGE Open Med Case Rep
December 2024
Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis.
View Article and Find Full Text PDFMod Rheumatol Case Rep
December 2024
Department of Rheumatology and Clinical Immunology, Wakayama Medical University, Wakayama, Japan.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis preceded by bronchial asthma or allergic sinusitis and accompanied by peripheral blood eosinophilia. Immunosuppressive drugs, such as cyclophosphamide in addition to high-dose glucocorticoids, are recommended for induction of remission in patients with severe EGPA. Although mepolizumab is widely recognized as remission induction therapy in non-fatal/non-organ disabling or relapsed/refractory EGPA, its efficacy and safety in induction of remission for severe cases have been ambiguous.
View Article and Find Full Text PDFArerugi
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 PDFImmunol Invest
December 2024
Clinic of Otorhinolaryngology and Maxillofacial Surgery, University Clinical Center of Serbia, Belgrade, Serbia.
Background: In this review article, we aimed to discuss the pathogenesis of sensorineural hearing loss (SNHL) in patients with different forms of chronic rhinosinusitis (CRS), with special reference to the connection of the immune response of the nasal and middle ear mucosa and inner ear structures.
Methods: Articles for this review were identified using PubMed and Google© Scholar databases.
Results: Different phenotypes of CRS may be associated with impaired function of the inner and outer cells of the organ of Corti.
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