Department of Pathology, Leiden University Medical Centre, Leiden, The Netherlands.
Department of Nephrology, Leiden University Medical Centre, Leiden, The Netherlands.
Nephrol Dial Transplant. 2017 Apr 1;32(4):654-662. doi: 10.1093/ndt/gfx020.
Full-house immunofluorescence in combination with various histopathologic lesions in the renal biopsies of patients without overt systemic lupus erythematosus (SLE) poses a diagnostic challenge. In this setting, the biopsy findings are sometimes termed non-lupus 'full-house nephropathy' (FHN). It is presently unknown whether idiopathic non-lupus FHN is clinicopathologically and prognostically distinct from lupus FHN.
We included non-lupus FHN patients and lupus FHN controls (four or more American College of Rheumatology or Systemic Lupus International Collaborating Clinics criteria) who were biopsied between 1968 and 2014 at the Leiden University Medical Centre. Non-lupus FHN patients were studied for progression to SLE and/or the presence of other conditions with FHN. The clinicopathologic characteristics and prognosis of idiopathic non-lupus FHN patients were compared with those of lupus FHN patients.
Of 149 included patients, 32 had non-lupus FHN. During the median follow-up of 20 years, no non-lupus FHN patients developed SLE. In all, 20 non-lupus FHN patients had idiopathic non-lupus FHN, and in 12 patients, secondary non-lupus FHN was considered due to membranous nephropathy (anti-PLA2R-positive, n = 1; cancer-associated, n = 3), IgA nephropathy ( n = 4), infection-related glomerulonephritis ( n = 2) or anti-neutrophil cytoplasmic antibody-associated glomerulonephritis ( n = 2). Idiopathic non-lupus FHN patients were more often male (P < 0.001) than lupus FHN patients and their renal biopsies more often showed a mesangial (P = 0.04) or membranous pattern of injury (P = 0.02) and less intense C1q staining (P = 0.002). Clinically, they presented with lower-range erythrocyturia (P = 0.04), more proteinuria (P < 0.01) and less complement consumption in the classical pathway (P < 0.001) than lupus FHN patients. By multivariable Cox regression analysis of patients with a lupus nephritis class III/IV pattern of injury, idiopathic non-lupus FHN compared with lupus FHN was an independent risk factor for end-stage renal disease [hazard ratio 5.31 (95% confidence interval 1.47-19.24)].
Our results show that the clinical recognition of idiopathic non-lupus FHN as a diagnostic category is critical.
在没有明显系统性红斑狼疮（SLE）的患者的肾活检中，全免疫荧光结合各种组织病理学病变带来了诊断上的挑战。在这种情况下，活检结果有时被称为非狼疮“全屋肾病”（FHN）。目前尚不清楚特发性非狼疮 FHN 在临床病理和预后方面是否与狼疮 FHN 不同。
我们纳入了 1968 年至 2014 年间在莱顿大学医学中心接受活检的非狼疮 FHN 患者和狼疮 FHN 对照（四项或更多美国风湿病学会或系统性红斑狼疮国际合作诊所标准）。对非狼疮 FHN 患者进行了进展为 SLE 和/或存在其他 FHN 疾病的研究。比较了特发性非狼疮 FHN 患者与狼疮 FHN 患者的临床病理特征和预后。
在 149 名纳入的患者中，有 32 名患有非狼疮 FHN。在中位数为 20 年的随访期间，没有非狼疮 FHN 患者发展为 SLE。共有 20 名非狼疮 FHN 患者患有特发性非狼疮 FHN，在 12 名患者中，由于膜性肾病（抗 PLA2R 阳性，n = 1；与癌症相关，n = 3）、IgA 肾病（n = 4）、感染相关肾小球肾炎（n = 2）或抗中性粒细胞胞浆抗体相关肾小球肾炎（n = 2），考虑为继发性非狼疮 FHN。特发性非狼疮 FHN 患者更常见于男性（P < 0.001）而非狼疮 FHN 患者，其肾脏活检更常表现为系膜（P = 0.04）或膜性损伤模式（P = 0.02），且 C1q 染色较弱（P = 0.002）。临床上，他们的血尿范围较低（P = 0.04），蛋白尿较多（P < 0.01），经典途径补体消耗较少（P < 0.001），与狼疮 FHN 患者相比。通过多变量 Cox 回归分析损伤模式为狼疮性肾炎 III/IV 级的患者，与狼疮 FHN 相比，特发性非狼疮 FHN 是终末期肾病的独立危险因素[风险比 5.31（95%置信区间 1.47-19.24）]。
我们的结果表明，临床上认识到特发性非狼疮 FHN 是一个诊断类别至关重要。
