杀菌通透性增强蛋白-抗中性粒细胞胞质抗体相关性血管炎合并支气管扩张1例并文献复习

Bactericidal permeability increasing protein-anti-neutrophil cytoplasmic antibody-associated vasculitis complicated with bronchiectasis:a case report and literature review

抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性血管炎是一组以血清中能够检测到ANCA为突出特点的系统性小血管炎,杀菌通透性增强蛋白(bactericidal permeability increasing protein,BPI)是ANCA的靶抗原之一。BPI-ANCA相关性血管炎临床上虽并不多见,但合并支气管扩张并非偶然。该文报道1例BPI-ANCA相关性血管炎肾损害合并支气管扩张,并复习相关文献,探讨BPI-ANCA相关性血管炎的病例特点及支气管扩张与ANCA相关性血管炎的相关性,以提高临床医师对该类疾病的认识。

Anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis is a group of systemic small vasculitis characterized by the detection of ANCA in serum.Bactericidal permeability enhancing protein(BPI)is one of the target antigens of ANCA.BPI-ANCA-associated vasculitis is not common clinically,and the combination of bronchiectasis is not accidental.The paper reported a case of BPI-ANCA-associated vasculitis with renal damage combined with bronchiectasis.We reviewed relevant literature to explore the characteristics of BPI-ANCA-associated vasculitis and the correlation between bronchiectasis and ANCA-associated vasculitis,so as to improve the clinician's understanding on this disease.