Membranous nephropathy is typically classified as idiopathic and secondary, but nowadays the number of atypical membranous nephropathy (aMN) is increasing, many of which cannot determine its etiology in China. In this study, we compared the clinical and pathological characteristics of idiopathic membranous nephropathy (iMN) with aMN with unknown etiology from a single center in China.We retrospectively reviewed the clinical data of 577 patients with iMN and aMN at Peking University People's Hospital from January 2006 to December 2015 over a 10-year period, and analyzed their clinical and pathological characteristics. The level of serum phospholipase A2 receptors (PLA2R) antibody was detected in 106 iMN and 162 aMN patients.There were 278 iMN patients and 299 aMN patients who were included into this study in 3210 cases of renal biopsy during a 10-year period in our hospital. The average age of patients with iMN was significantly older than those with aMN (54.77 ± 13.01 vs 47.13 ± 16.16, P < .001). Around 75 patients (27%) were smokers in iMN patients, and 111 patients (37.1%) in aMN patients (P = .009). The mainly clinical manifestation of these 2 groups was nephrotic syndrome (61.5% in iMN group vs 58.4% in aMN group), but there were more patients accompanied with nephritis syndrome in aMN group than iMN group (17.1% vs 6.1%, P < .001). The immunofluorescence of renal biopsy showed “full house” in aMN group; and IgG subclass of the glomeruli demonstrated IgG4 (90.4%) was commonest in iMN group, but IgG1 (94.6%) in aMN group. 51 (48.1%) patients with iMN were detected positive PLA2R antibody in their serum, and 93 (57.4%) in aMN patients (P = .168). The patients with positive PLA2R antibody had higher positive rate of microscopic hematuria and urinary protein, lower albumin.The aMN patients are younger, higher smoking rate, its main clinical manifestation is nephrotic syndrome, but more of them accompanied with nephritis syndrome than those in iMN patients. Serum PLA2R antibody could not distinguish aMN from iMN. aMN could be a special glomerular disease in China, and need a further research on a larger scale.

Background Membranous nephropathy (MN) is mainly classified into idiopathic MN (iMN) and secondary MN in etiology. In recent years, a new kind of membranous nephropathy, atypical membranous nephropathy (aMN) which shows “full house” in immunofluorescence but without definite etiology was paid more attention. In a single center cohort, the renal outcomes of iMN and aMN were compared. Methods iMN and aMN patients were selected from renal pathology databank from January 2006 to December 2015. Patients’ demographics, laboratory values, induction regimens and patients’ responses were recorded. Specially, creatinine, eGFR, albumin and 24 h urinary protein excretion were recorded at 6th month after the induction of immunosuppressive (IS) treatment and at the end of follow up. Complete proteinuria remission was defined as urinary protein < 0.3 g/d, partial proteinuria remission was defined as urinary protein between 0.3 g/d ~ 3.5 g/d and decreased > 50 % from the baseline. The primary outcome was worsening renal function, defined as a 30 % or more decrease in eGFR or end-stage renal disease (eGFR < 15ml/min/1.73m2). COX proportional hazard models were used to test if aMN was a risk factor of worsening renal function compared with iMN. Results There were 298 patients diagnosed with MN and followed in our center for 1 year or more, including 145 iMN patients with an average follow-up time of 4.5 ± 2.6 years, and 153 aMN patients with 4.1 ± 2.0 years (p = 0.109). The average age of iMN patients was older than aMN patients (56.1 ± 12.2 versus 47.2 ± 16.2 years old, p < 0.001). There were 99 iMN patients and 105 aMN patients with nephrotic range proteinuria and without previous immunosuppressive treatment. 93 (93.9 %) and 95 (90.5 %) patients underwent immunosuppressive treatment in iMN and aMN group, and there was no significant difference of the overall proteinuria remission rates at 6th month (59.1 % vs. 52.0 %, p = 0.334) and endpoint (73.7 % vs. 69.5 %, p = 0.505) between the two groups. 25 (25.3 %) patients in iMN group and 21 (20.0 %) patients in aMN group reached primary endpoint (X2 = 0.056, p = 0.812). Multivariate COX regression showed that after demographics, baseline laboratory values and remission status at 6th month were adjusted, aMN group had similar renal outcome compared with iMN group, the HR of primary outcome was 0.735 (95 % CI 0.360 ~ 1.503, p = 0.399). Conclusions The proteinuria remission rates and renal outcomes were similar in iMN and aMN patients after covariables were adjusted.

Objectives Idiopathic membranous nephropathy (iMN) is a major cause of nephrotic syndrome. Atypical membranous nephropathy (aMN) is a new type of nephropathy in China, characterized by a ‘full-house’ on immunofluorescent examination, that is IgG, IgA, IgM, C3, C1q positive, but without clinical evidence of a secondary cause. Phospholipase A2 receptor (PLA2R) was the major target antigens in iMN patients. Activation of the mannose-binding lectin (MBL) pathway plays a vital role in the development of MN. Our objective was to investigate the role of PLA2R and MBL in the pathogenesis of iMN and aMN. Methods We conducted a retrospective observational study using propensity score matching by age, gender, and eGFR. All clinical, laboratory data, and follow-up data of the patients were collected. Serum levels of anti-PLA2R antibodies and MBL were tested. Results Finally, 30 iMN patients and 30 aMN patients were included, and 20 healthy controls were retrospectively collected in this study. The 24 h proteinuria level was higher and serum albumin was lower in anti-PLA2R (+) patients than in anti-PLA2R (−) patients in both iMN and aMN groups. In aMN patients, MBL levels were significantly higher in anti-PLA2R (+) patients than in anti-PLA2R (−) patients ( p = .045). The serum level of anti-PLA2R positively correlated with no-remission in both iMN and aMN groups. Conclusions The complement lectin pathway has an association with the development of MN, especially in patients with positive anti-PLA2R antibodies. Serum MBL cannot differentiate between the two diseases. Serum MBL levels are not associated with clinical manifestations, nor with prognosis.