Kapsia Eleni, Marinaki Smaragdi, Michelakis Ioannis, Liapis George, Sfikakis Petros P, Tektonidou Maria G, Boletis John
Department of Nephrology and Renal Transplantation, Medical School, National and Kapodistrian University of Athens, Laiko Hospital, Athens, Greece.
Department of Hygiene, Epidemiology and Medical Statistics, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Front Med (Lausanne). 2022 Apr 14;9:809533. doi: 10.3389/fmed.2022.809533. eCollection 2022.
Pure membranous lupus nephritis (MLN) accounts for 10-20% of total cases of lupus nephritis and is generally associated with a better patient and renal survival compared to proliferative classes. Studies of MLN are limited by small sample size and heterogeneity of included populations since patients with pure MLN and those with mixed classes are usually examined together.
To describe clinical and laboratory characteristics of patients with pure MLN, therapeutic regimens, response to treatment, renal relapses, and their long-term renal survival and to define prognostic factors of remission and relapse.
We retrospectively studied an inception cohort of 27 patients with histologically proven pure MLN. Clinical, laboratory and therapeutical parameters were recorded at diagnosis, at different time points (3-6-9-12-18-24-36-72 months) during the course of the disease, at time of renal flare, and at last follow up visit.
48.1% (13/27) of patients were treated with mycophenolic acid (MPA), 29.6% (8/27) with cyclophosphamide (CYC), and 3.7% (1/27) with cyclosporine (all in combination with corticosteroids). Five patients (18.5%) did not receive any immunosuppressive treatment. Mean duration of treatment was 4.7 ± 2.3 years. Median time to complete remission was 9 months (IQR = 7) and median time to partial remission was 4 months (IQR = 4). No clinical or laboratory parameter was found to be significantly associated with time to remission. Time to remission was not significantly affected by either of the two treatment regimens (CYC and MPA) ( = 0.43). Renal flare was observed in 6 (22%) of the 27 patients in a median time of 51 months (IQR = 63). Proteinuria >1 g/24 h at 1 year significantly correlated with risk of flare (OR 20, = 0.02). After a median follow up period of 77 months, all patients had an eGFR > 60 ml/min/1.73 m (mean eGFR 100 ± 32 ml/min/1.73 m).
In a small cohort of patients with pure MLN, long-term renal survival was very good. With the limitation of the small sample size, we could not find any baseline clinical, biochemical or therapeutic factor that could predict time to remission. Proteinuria > 1 g/24 h at 1 year should be further examined in larger cohorts as a possible predictor of flare.
单纯膜性狼疮性肾炎（MLN）占狼疮性肾炎总病例的10%-20%，与增殖性狼疮性肾炎相比，患者及肾脏的生存率通常更高。由于单纯MLN患者和混合类型患者通常一起进行检查，MLN的研究受到样本量小和纳入人群异质性的限制。
描述单纯MLN患者的临床和实验室特征、治疗方案、治疗反应、肾脏复发情况及其长期肾脏生存率，并确定缓解和复发的预后因素。
我们回顾性研究了27例经组织学证实为单纯MLN的起始队列患者。在诊断时、疾病过程中的不同时间点（3-6-9-12-18-24-36-72个月）、肾脏复发时以及最后一次随访时记录临床、实验室和治疗参数。
48.1%（13/27）的患者接受霉酚酸（MPA）治疗，29.6%（8/27）接受环磷酰胺（CYC）治疗，3.7%（1/27）接受环孢素治疗（均联合糖皮质激素）。5例患者（18.5%）未接受任何免疫抑制治疗。平均治疗时间为4.7±2.3年。完全缓解的中位时间为9个月（四分位间距=7），部分缓解的中位时间为4个月（四分位间距=4）。未发现任何临床或实验室参数与缓解时间显著相关。两种治疗方案（CYC和MPA）均未对缓解时间产生显著影响（P=0.43）。27例患者中有6例（22%）出现肾脏复发中位时间为51个月（四分位间距=63）。1年时蛋白尿>1g/24h与复发风险显著相关（比值比20，P=0.02）。中位随访77个月后，所有患者的估算肾小球滤过率（eGFR）>60ml/min/1.73m²（平均eGFR为100±32ml/min/1.73m²）。
在一小群单纯MLN患者中，长期肾脏生存率非常好。由于样本量小，我们未能发现任何可预测缓解时间的基线临床、生化或治疗因素。1年时蛋白尿>1g/24h作为复发的可能预测因素，应在更大队列中进一步研究。
