Aim This study aimed to investigate the incidence of relapse and FR/SDNS in Chinese children with SSNS and to develop clinical prediction models for relapse and FR/SDNS. Methods This retrospective cohort study involved 339 newly onset SSNS patients between 2006 and 2016. The incidence of relapse and FR/SDNS were estimated using the Kaplan–Meier method. Prediction models were constructed based on Cox proportional‐hazards regression. Results The median follow‐up time was 8.7 years. The cumulative incidence of relapse at 1‐, 2‐, and 5‐year was 51.0%, 62.5%, and 66.6%. The cumulative incidence of FR/SDNS at 1‐, 2‐, and 5‐year was 18.4%, 29.0%, and 32.9%. The final prediction model for first relapse included four variables (serum albumin, triglycerides, IgM, and time to first remission). The model's discriminative ability was low (Harrell's C index = 0.62). The final prediction model for FR/SDNS included four variables (serum albumin, lipoprotein(a), time to first remission, and time to first relapse). The discrimination and calibration of the prediction model for FR/SDNS were acceptable (Harrell's C index = 0.73, Brier score at 1‐ and 2‐year were 0.11 and 0.17). Conclusion The first relapse and FR/SDNS mainly occurred in the first 2 years after initial SSNS onset. The prediction model for relapse developed using common clinical parameters performed poorly, while the prediction model for FR/SDNS might be useful.

Objective The aim of this study was to investigate serum levels of soluble B-cell maturation antigen (sBCMA) in childhood-onset systemic lupus erythematous (cSLE) patients with renal involvement, and to elucidate their association with clinical characteristics. Methods 116 cases of cSLE patients with renal involvement (84 females and 32 males; median age 11.6 (10.1, 12.9) years) hospitalized in Department of Pediatric Nephrology and Rheumatology, the First Affiliated Hospital, Sun Yat-sen University and 31 healthy controls (HCs) were enrolled. Serum concentrations of sBCMA were determined using enzyme-linked immunosorbent assay (ELISA). Clinical and laboratory information of cSLE patients were retrospectively analyzed. Results Serum sBCMA levels were significantly increased in primary cSLE when compared with treated cSLE patients and HCs, whereas there was no significant difference between treated cSLE patients and HCs. Patients with high disease activity displayed higher serum sBCMA levels compared with those with no or mild to moderate disease activity. Positive correlation was observed between serum sBCMA levels and systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), antinuclear antibody titers, anti-double-stranded DNA titers, erythrocyte sedimentation rate, and immunoglobulin G levels, while sBCMA levels were negatively correlated with blood white blood cell count, hemoglobin, platelet count, complement C3 and C4 levels. Serum sBCMA levels decreased as disease ameliorated after treatments among 11 cases with follow-up examinations. Conclusions In cSLE patients with renal involvement, serum sBCMA levels correlated significantly with disease activity, immunological, and hematological parameters, but not with renal parameters. Our results suggest the potential and significance of serum sBCMA as a biomarker in cSLE patients.

BackgroundMutation in the COQ8B gene can cause COQ8B glomerular nephropathy (COQ8B-GN), which is rare and associated with steroid-resistant nephrotic syndrome (SRNS) as well as rapid progression to end-stage renal disease (ESRD). The aim of this study was to analyze the prognosis and recurrence risk of COQ8B-GN in patients after kidney transplantation (KTx) and summarize the characteristics of the Chinese population.MethodsA retrospective study included four cases treated in our hospital with a diagnosis of COQ8B-GN. Chinese and foreign studies were searched from database inception to February 2022.ResultsA total of four cases were included, with the age of onset ranging from 4 to 9 years. The initial presentations were SRNS and asymptomatic proteinuria. Only one had an extrarenal manifestation (thyroid cyst). All patients progressed to ESRD at a mean time of 42 months after onset. With a total follow-up time ranging from 12 to 87 months, three of them had received transplantation. While one case needed a second KTx due to graft failure caused by chronic rejection, two recipients had excellent graft function. No recurrence in allograft was observed. There have been 18 cases of KTx recipients reported globally with follow-up information. Except for two cases of graft failure caused by hyperacute rejection and chronic rejection, respectively, the rest all had good graft function without recurrence. In addition, 44 cases of COQ8B-GN in the Chinese population were identified. At the onset, 75% of the patients were aged ≤10 years with initial symptoms of asymptomatic proteinuria, nephrotic syndrome (NS), or SRNS. By the time of literature publication, 59% of patients had progressed to ESRD (mean age of 10.3 ± 3.6 years). The median time from onset to ESRD was 21 months. Renal pathology mainly showed focal segmental glomerulosclerosis (FSGS), accounting for 61.8% of all biopsies, followed by mesangial proliferative glomerulonephritis (20.6%). The first three prevalent mutations in the COQ8B gene among the Chinese population were c. 748G>C, c. 737G>A, and c. 532C>T.ConclusionCOQ8B-GN in the Chinese population may present with asymptomatic proteinuria, NS, or SRNS initially, with most onsets before the age of 10 years. A lot of patients progress to ESRD in early adolescence. FSGS on biopsy and c. 748G>C in the genetic test are the most frequently seen in Chinese COQ8B-GN patients. KTx is feasible for patients with ESRD due to the low risk of recurrence, but we should pay attention to graft rejection.