Juanet Cristián, Hassi Isabel, Koirala Abbal
Division of Nephrology, Pontificia Universidad Católica de Chile, Santiago, Chile.
Division of Rheumatology, Universidad de los Andes, Santiago, Chile.
Glomerular Dis. 2024 Oct 11;4(1):189-199. doi: 10.1159/000541792. eCollection 2024 Jan-Dec.
Pauci-immune glomerulonephritis (PIGN) is typically secondary to antineutrophil cytoplasmic antibodies (ANCA) small-vessel vasculitis. However, some cases lack detectable circulating ANCA and are called ANCA-negative PIGN (seronegative PIGN). The reported incidence of this varies greatly. Its relationship to ANCA-associated vasculitis (AAV) is unclear.
This review explores the pathophysiology of seronegative PIGN and summarizes findings from 12 studies focusing on this disease. The role of neutrophils appears to be central, with activation through cellular and humoral mechanisms. Most studies have noted less extrarenal involvement and more chronic changes in the kidney biopsy in seronegative PIGN compared to ANCA-positive cases. Studies have mostly reported using corticosteroids with cyclophosphamide for induction therapy and azathioprine for maintenance. The renal survival was noted to be lower compared to ANCA-positive PIGN.
Whether ANCA-negative PIGN represents a distinct disease or is part of the AAV spectrum remains unclear. Prospective large-scale studies are needed to understand this disease for optimal diagnosis and management.
寡免疫性肾小球肾炎（PIGN）通常继发于抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎。然而，一些病例检测不到循环中的ANCA，被称为ANCA阴性PIGN（血清阴性PIGN）。其报道的发病率差异很大。它与ANCA相关性血管炎（AAV）的关系尚不清楚。
本综述探讨了血清阴性PIGN的病理生理学，并总结了12项针对该疾病的研究结果。中性粒细胞的作用似乎至关重要，通过细胞和体液机制被激活。与ANCA阳性病例相比，大多数研究指出血清阴性PIGN在肾外受累较少，肾活检中慢性改变较多。研究大多报道使用糖皮质激素联合环磷酰胺进行诱导治疗，硫唑嘌呤进行维持治疗。与ANCA阳性PIGN相比，肾存活率较低。
ANCA阴性PIGN是一种独特的疾病还是AAV谱系的一部分仍不清楚。需要进行前瞻性大规模研究以了解该疾病，从而实现最佳诊断和管理。
