Gietzen Rachelle, Caza Tiffany N, Best-Rocha Alejandro, Larsen Christopher P
Arkana Laboratories, Little Rock, Arkansas, USA.
Kidney Int Rep. 2025 Mar 11;10(5):1460-1467. doi: 10.1016/j.ekir.2025.03.001. eCollection 2025 May.
Pauci-immune crescentic glomerulonephritis (GN) is nearly synonymous with antineutrophil cytoplasmic antibody (ANCA)-associated disease. Cases with immune complex deposition create a diagnostic conundrum leading to suspicion for concurrent infection or autoimmune disease. Small case series have demonstrated myeloperoxidase (MPO) in the immune deposits in patients with membranous nephropathy (MN) and ANCA-associated disease. However, the specificity of MPO staining to characterize immune deposits in crescentic GN has not been thoroughly evaluated.
We performed MPO immunostaining of 143 kidney biopsies, including pauci-immune crescentic GN ( = 15), ANCA with immune-complex crescentic GN ( = 20), MN without crescents ( = 24), endocarditis-associated crescentic GN ( = 25), hydralazine-associated crescentic GN ( = 11), and concurrent crescentic GN and MN without phospholipase A2 receptor (PLA2R) ( = 38) and with PLA2R ( = 10). MPO immunohistochemistry (IHC) was evaluated for positivity, character, and location of MPO immune deposits by 4 blinded pathologists.
In patients with dual crescentic GN and MN without PLA2R, 84.2% were MPO-IHC positive. Crescentic GN with mesangial IgG was MPO-IHC positive in 40%. Crescentic GN related to hydralazine exposure was MPO-IHC positive in 72.7%. All cases with pauci-immune crescentic GN, endocarditis-associated cases, and MN cases with known antigens were negative for MPO.
Our study demonstrated that glomerular immune deposits in patients with crescentic GN with positive MPO serology demonstrated MPO positivity in the pattern of immune deposits in the majority of cases. Glomerular immune complexes in patients with MPO-positive crescentic GN therefore represent MPO-IgG immune complexes and should be thought of as one disease rather than a second disease process.
寡免疫性新月体性肾小球肾炎(GN)几乎等同于抗中性粒细胞胞浆抗体(ANCA)相关疾病。伴有免疫复合物沉积的病例会引发诊断难题,导致怀疑并发感染或自身免疫性疾病。小型病例系列研究已证实在膜性肾病(MN)和ANCA相关疾病患者的免疫沉积物中存在髓过氧化物酶(MPO)。然而,MPO染色在鉴别新月体性GN免疫沉积物方面的特异性尚未得到充分评估。
我们对143例肾活检组织进行了MPO免疫染色,包括寡免疫性新月体性GN(n = 15)、伴有免疫复合物的新月体性GN的ANCA(n = 20)、无新月体的MN(n = 24)、心内膜炎相关的新月体性GN(n = 25)、肼屈嗪相关的新月体性GN(n = 11),以及并发无磷脂酶A2受体(PLA2R)的新月体性GN和MN(n = 38)和有PLA2R的新月体性GN和MN(n = 10)。4名 blinded病理学家对MPO免疫组化(IHC)的MPO免疫沉积物的阳性、特征和位置进行了评估。
在无PLA2R的新月体性GN和MN并发患者中,84.2%为MPO-IHC阳性。伴有系膜IgG的新月体性GN中40%为MPO-IHC阳性。与肼屈嗪暴露相关的新月体性GN中72.7%为MPO-IHC阳性。所有寡免疫性新月体性GN病例、心内膜炎相关病例以及已知抗原的MN病例MPO均为阴性。
我们的研究表明,MPO血清学阳性的新月体性GN患者的肾小球免疫沉积物在大多数病例中呈现出MPO免疫沉积物模式的MPO阳性。因此,MPO阳性的新月体性GN患者的肾小球免疫复合物代表MPO-IgG免疫复合物,应被视为一种疾病而非第二种疾病过程。