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Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome.
- Source :
-
International journal of molecular sciences [Int J Mol Sci] 2024 May 12; Vol. 25 (10). Date of Electronic Publication: 2024 May 12. - Publication Year :
- 2024
-
Abstract
- Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.
- Subjects :
- Humans
Extracellular Traps metabolism
Extracellular Traps immunology
Antibodies, Antineutrophil Cytoplasmic immunology
Lung Diseases etiology
Lung Diseases immunology
Lung Diseases pathology
Lung pathology
Lung immunology
Autoantibodies immunology
Animals
Microscopic Polyangiitis immunology
Microscopic Polyangiitis complications
Microscopic Polyangiitis pathology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis immunology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis pathology
Anti-Glomerular Basement Membrane Disease immunology
Anti-Glomerular Basement Membrane Disease etiology
Anti-Glomerular Basement Membrane Disease pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1422-0067
- Volume :
- 25
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- International journal of molecular sciences
- Publication Type :
- Academic Journal
- Accession number :
- 38791316
- Full Text :
- https://doi.org/10.3390/ijms25105278