The Oxford classification for IgA nephropathy (IgAN) was updated in 2017. We have validated the revised Oxford classification considering treatment with corticosteroids/immunosuppressors. In this retrospective analysis, 871 IgAN patients were enrolled. Patients were divided into two groups, those treated with or without corticosteroids/immunosuppressors. The 20-year renal prognosis up to end-stage renal disease was assessed using the Oxford classification. In all patients, the renal survival rate was 87.5% at 10 years and 72.6% at 20 years. The T score alone was significantly related to renal prognosis in the Kaplan-Meier analysis and multivariate Cox regression analysis. In the non-treatment group (n = 445), E, S, T, and C scores were significantly related to renal survival rates, however, in the treatment group (n = 426), T score alone was significantly related to renal prognosis on Kaplan-Meier analysis, indicating that corticosteroids/immunosuppressors improved renal prognosis in E1, S1, and C1. In patients with E1, S1, or C1, the treatment group showed significantly better renal prognosis than the non-treatment group in univariate and multivariate analysis. The Oxford classification and T score were used to determine renal prognosis in IgAN patients. Corticosteroids/immunosuppressors improved renal prognosis, especially E1, S1, and C1 scores. IgA nephropathy (IgAN) was first reported 50 years ago by Berger 1. IgAN was initially labelled as a benign disease; however, it was later shown to have a poor long-term prognosis 2-5. Although the prognostic risk factors of IgAN have not been clearly defined, hypertension, deterioration of renal function, and increased levels of proteinuria are known prognostic factors 2-5. Histological findings may also inform prognosis 6-9 , although these factors have not achieved worldwide acceptance. In 2009, the Oxford classification was reported by the International IgAN Network and International Renal Pathology Society 10,11. In the Oxford classification, mesangial hypercellularity (M), segmental glomerulosclerosis (S), tubular atrophy/interstitial fibrosis (T), were selected as prognostic factors and endothelial hypercellularity (E) were selected as reactive factors against corticosteroids/immunosuppressors (MEST score). After this report of the Oxford classification, several validation studies were performed, with different results among those studies 12-25. Using a multivariate analysis, it was determined that the T score was the most valuable marker of progression; however, other factors differed according to the clinical background (race, age), inclusion criteria (estimated glomerular filtration rate [eGFR] > 30 mL/min/1.73 m 2 , proteinuria > 0.5 g/day, minimum followup > 1 year), duration of follow-up, treatment, and endpoint of each study (eGFR slope, 50% reduction of eGFR, or end-stage renal disease [ESRD]). Interestingly, several reports validated not only the MEST score but also the crescent formation (as the C score), and multivariate analysis indicated that the C s...

The Oxford classification of IgA nephropathy (IgAN) can evaluate each MEST-C score individually. We analysed a new grading system that utilised the total MEST-C score in predicting renal prognosis. Altogether, 871 IgAN patients were classified into three groups using the new Oxford classification system (O-grade) that utilised the total MEST-C score (O-grade I: 0–1, II: 2–4, and III: 5–7 points), and the 10-year renal prognosis was analysed. The clinical findings became significantly severer with increasing O-grades, and the renal survival rate by the Kaplan–Meier method was 94.1%, 86.9%, and 74.1% for O-grades I, II, and III, respectively. The hazard ratios (HRs) for O-grades II and III with reference to O-grade I were 2.8 (95% confidence interval [CI] 1.3–6.0) and 6.3 (95% CI 2.7–14.5), respectively. In the multivariate analysis, mean arterial pressure and eGFR, proteinuria at the time of biopsy, treatment of corticosteroids/immunosuppressors, and O-grade (HR 1.63; 95% CI 1.11–2.38) were the independent factors predicting renal prognosis. Among the nine groups classified using the O-grade and Japanese clinical-grade, the renal prognosis had an HR of 15.2 (95% CI 3.5–67) in the severest group. The O-grade classified by the total score of the Oxford classification was associated with renal prognosis.

Renal disease is a common complication of rheumatoid arthritis (RA) and can occur secondary to RA or be induced by therapeutic agents. Recently, glomerular deposition of galactose-deficient IgA1 (Gd-IgA1) was identified as a feature of primary IgA vasculitis with nephritis (IgA-VN). We herein report a case of IgA-VN in an RA patient whose disease activity was controlled by treatment with etanercept. To distinguish between primary IgA-VN and secondary IgA-VN caused by RA or etanercept, we performed immunostaining of renal biopsy sections with the Gd-IgA1-specific antibody KM55. Positive KM55 staining confirmed the diagnosis of primary IgA-VN in a patient with RA.

<b><i>Background:</i></b> In patients with systemic lupus erythematosus (SLE), disease activity can persist even after initiating dialysis. However, guidelines for the treatment of patients with SLE after dialysis is initiated have not yet been established. <b><i>Case Presentation:</i></b> We describe the case of a 54-year-old Japanese woman who was diagnosed with SLE at age 12, progressed to end-stage renal disease (ESRD), and initiated hemodialysis for lupus nephritis. However, SLE activity persisted after hemodialysis. Cyclophosphamide and mycophenolate mofetil were administered in addition to prednisolone and immunoadsorption, but this treatment strategy was limited by side effects. The patient was subsequently treated with belimumab, and the activity of SLE decreased rapidly. <b><i>Conclusions:</i></b> ESRD patients with SLE show no significant decrease in transitional B cells and have elevated levels of B-cell activating factor (BAFF). Both transitional B cells and BAFF are important therapeutic targets for belimumab, indicating that patients with ESRD may benefit from belimumab therapy. However, the effects of belimumab may be potentiated in patients with uremia, who may be more susceptible to adverse events such as infections. Patients with SLE who receive belimumab after initiation of hemodialysis therefore require careful follow-up. Here, we report the first case of belimumab administration in a patient with SLE after initiation of hemodialysis.

Systemic lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease that affects multiple organs and tissues. Lupus nephritis (LN) is a serious complication of SLE, which occurs at a high rate. Conventional treatment strategies of LN have been widely accepted by two concepts such as induction therapy and maintenance therapy. In LN induction therapy until recently, cyclophosphamide in combination with prednisone (PSL) has been the standard method of treatment for proliferative forms of LN. In the latest review, the combination of mycophenolate mofetil (MMF) is also considered a standard treatment option. Furthermore, a multi-target therapy with tacrolimus (Tac) added to PSL and MMF, with reference to a regimen after organ transplantation has also been reported. In LN maintenance therapy, although recent reports have demonstrated that MMF, azathioprine, and Tac in combination with PSL may prevent renal flares, there is no definite opinion in the period of use or the method of tapering. On the contrary, there are also concepts of two mechanisms of therapy for LN, such as a treatment based on the immunological mechanism as an autoimmune disease and a treatment based on the non-immunological mechanism as a chronic kidney disease. Nephrologists need to continue searching for the best-mix treatment regimen according to various clinical findings. We review the options available for the treatment of LN, and summarize the results of recently published clinical trials that add new perspectives to the management of kidney disease in SLE.