Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

被引:1
作者
Fouka, Evangelia [1 ]
Drakopanagiotakis, Fotios [2 ]
Steiropoulos, Paschalis [2 ]
机构
[1] Aristotle Univ Thessaloniki, Gen Hosp G Papanikolaou, Med Sch, Dept Resp Med, Thessaloniki 57010, Greece
[2] Democritus Univ Thrace, Med Sch, Dept Resp Med, Alexandroupolis 68100, Greece
关键词
pathogenesis; ANCA-associated vasculitis; lung; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; DIFFUSE ALVEOLAR HEMORRHAGE; EOSINOPHILIC GRANULOMATOSIS; WEGENERS-GRANULOMATOSIS; CLINICAL-ASPECTS; DNA METHYLATION; PLASMA-EXCHANGE; DISEASE; CELLS; POLYANGIITIS;
D O I
10.3390/ijms25105278
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
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页数:18
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