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Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture.
Diagnostics (Basel)
January 2025
Department of Internal Medicine and Gastroenterology, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.
: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. : We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain.
View Article and Find Full Text PDFA real-world study to evaluate effectiveness of mepolizumab in treating severe asthma in Taiwan (REMIT).
Ther Adv Respir Dis
January 2025
Department of Chest Medicine, Taipei Veterans General Hospital, No. 201, Section 2, Shipai Road, Beitou District, Taipei City 11217, Taiwan.
Background: REMIT is the first real-world study of mepolizumab effectiveness in patients with severe asthma (SA) in Taiwan.
Objectives: The primary objective evaluated changes in clinically significant exacerbations (CSEs; defined as use of oral corticosteroids (OCS) or emergency department (ED) visits and/or hospitalizations) in the 12 months pre- and post-mepolizumab treatment. Secondary objectives assessed changes in the number of CSEs requiring ED visits/hospitalizations and daily maintenance OCS (mOCS) dosage 12 months pre- and post-mepolizumab treatment.
Comparison of Imaging Findings between Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis on Sinus CT: Importance of High-Density Opacification of the Paranasal Sinuses.
AJNR Am J Neuroradiol
January 2025
From the Department of Radiology (I.R., S.P., A.K., O.S.), Boston Medical Center, Boston University Chobanian & Avedisian School of Medicine, Boston, Massachusetts
Background And Purpose: Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA) are the most common causes of chronic sinusitis from systemic granulomatous diseases. While both are small- to medium-sized vasculitis with necrotizing granulomas, they have different clinical courses and prognoses. High-density sinus opacification has been reported in allergic fungal sinusitis with eosinophilic infiltrates.
View Article and Find Full Text PDFSimilarities and differences of clinical manifestations and prognosis between eosinophilic gastroenteritis and eosinophilic granulomatosis with polyangiitis complicating gastrointestinal involvement.
Clin Rheumatol
January 2025
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
Objective: To investigate the similarities and differences of clinical manifestations and long-term prognosis between eosinophilic gastroenteritis (EGE) and eosinophilic granulomatosis with polyangiitis (EGPA) complicating GI involvement (EGPA-GI).
Methods: Sixty-two EGE and 30 EGPA-GI patients were retrospectively enrolled in PUMCH from 2008 to 2023. Baseline clinical records were collected.
An overview of proactive monitoring and management of respiratory issues in ataxia-telangiectasia in a specialist and shared care pediatric clinic.
Front Pediatr
December 2024
Paediatrics and Paediatric Respirology, National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom.
Ataxia-telangiectasia (A-T) is an ultrarare autosomal recessive disorder and occurs in all racial and ethnic backgrounds. Clinically, children and young people with A-T are affected by sinopulmonary infections, neurological deterioration with concomitant bulbar dysfunction, increased sensitivity to ionizing radiation, immunodeficiency, a decline in lung function, chronic liver disease, endocrine abnormalities, cutaneous and deep-organ granulomatosis, and early death. Pulmonary complications become more frequent in the second decade of life and are a leading cause of death in individuals with A-T.
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