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Background Lupus nephritis is considered one of the most common complications of systemic lupus erythematosus (SLE) that greatly affects the overall prognosis. Retinopathy, as one of the disease complications, is an indicator of disease severity, frequent renal impairment, and poor prognosis. Therefore, screening of subclinical retinopathy is of great importance as retinopathy and nephropathy are somehow associated in SLE patients. Aim The aim of this study was to assess the changes in retinal and optic nerve microcirculation in nephritic and nonnephritic SLE patients without clinical evidence of retinopathy or optic neuropathy. Patients and methods This is a prospective observational study that included 66 SLE patients. They were divided into two groups: group A that included 34 patients with nephritis and group B that included 32 patients without nephritis. In addition, 33 healthy participants (group C) were included as a control group. The participants were assessed with optical coherence tomography angiography of the macula and optic nerve. Assessment of vessel densities (VD), foveal avascular zone, retinal nerve fiber layer thickness, and optic nerve head and peripapillary VD were done. Results Group B patients were found to have significantly lower VDs in whole superficial VD (P=0.014) and all sectors of perifovea (P=0.005, 0.006, and 0.013 for whole, superior hemiretina, and inferior hemiretina, respectively). In addition, there was a significantly lower VD of the deep capillary plexus (DCP) in the parafovea (P=0.003, 0.004, and 0.01 for whole, superior, and inferior hemiretina, respectively) and in the perifovea (P=0.007, 0.002, and 0.014 for whole, superior hemiretina, and inferior hemiretina, respectively). Group A showed specific reduction of VD of the DCP of whole and perifoveal superior hemiretina (P=0.04) and choriocapillaris flow parameters (P=0.003). They also showed increase of inferior retinal nerve fiber layer thickness and whole image capillary density (P=0.036 and 0.001, respectively). Concerning the peripapillary capillary density, the whole image peripapillary capillary density and inferior peripapillary VD of group A was increased in comparison to group B (P=0.023 and 0.009, respectively) with no statistically significant difference with group C. Conclusion Nephritic SLE patients showed reduction in VD of the DCP of the perifoveal superior hemiretina and choriocapillaris, while nonnephritic SLE patients showed reduction of VD of the SCP of the perifovea and DCP of parafovea and perifovea.

Background: A clinical syndrome manifested by features of glomerular disease in the urinalysis and by progressive loss of kidney function over a comparatively short period is called rapidly progressive glomerulonephritis (RPGN). RPGN is also called Crescentic glomerulonephritis(CrGN). Crescentic glomerulonephritis (CrGN) is defined as active crescents involving >50% of the total glomeruli. We have done this study to find out the major cause of RPGN in Nepal. Materials and Methods: This is a one-year prospective study done in the department of renal pathology at Pratham Pathology Private Limited over one year (1st November 2021-30th October 2022). Data of all patients were evaluated for the histopathological diagnosis. All cases of RPGN were included in the study. Evaluation of serological and demographic data, along with the number of active crescents was done. Results: A total of 182 cases of kidney biopsies were received for evaluation during one year (1st November 2021-30th October 2022) at Pratham Pathology Laboratory Private Limited, Kathmandu, Nepal. Out of it 18 cases (9.89%) were those of Crescentic glomerulonephritis. The most common cause of Crescentic glomerulonephritis encountered was Lupus Nephritis (7/18 cases). All cases of lupus presented with Crescentic glomerulonephritis were at stage IV. % of glomeruli with crescents ranged from 50-81.25% with mean involved glomeruli 60.61%. Conclusions: A total of 9.89% of the renal biopsies were presented with CrGN. Lupus nephritis was the most common cause of CrGN in Nepal.

Renal hypoxia has recently been implicated as a key contributor and indicator of various glomerular diseases. As such, monitoring changes in renal oxygenation in these disorders may provide an early diagnostic advantage that could prevent potential adverse outcomes. Blood oxygen level-dependent magnetic resonance imaging (BOLD MRI) is an emerging noninvasive technique for assessing renal oxygenation in glomerular disease. Although BOLD MRI has produced promising initial results for the use in certain renal pathologies, the use of BOLD imaging in glomerular diseases, including primary and secondary nephrotic and nephritic syndromes, is relatively unexplored. Early BOLD studies on primary nephrotic syndrome, nephrotic syndrome secondary to diabetes mellitus, and nephritic syndrome secondary to systemic lupus erythematosus have shown promising results to support its future clinical utility. In this review, we outline the advancements made in understanding the use of BOLD MRI for the assessment, diagnosis, and screening of these pathologies.

Objective: To characterize the incidence, type, and risk factors of severe infections (SI) in patients with autoimmune kidney diseases treated with rituximab (RTX). Methods: We conducted a multicenter retrospective cohort study of adult patients with immune-related kidney diseases treated with at least one course of RTX between 2015 and 2019. As a part of the ABCDE Registry, detailed data on RTX application and SI were collected. SI were defined by Common Terminology Criteria for Adverse Events v5.0 as infectious complications grade 3 and above. Patients were dichotomized between "nephrotic" and "nephritic" indications. The primary outcome was the incidence of SI within 12 months after the first RTX application. Results: A total of 144 patients were included. Twenty-five patients (17.4%) presented with SI, mostly within the first 3 months after RTX administration. Most patients in the nephritic group had ANCA-associated vasculitis, while membranous nephropathy was the leading entity in the nephrotic group. Respiratory infections were the leading SI (n= 10, 40%), followed by urinary tract (n=3, 12%) and gastrointestinal infections (n=2, 8%). On multivariable analysis, body mass index (BMI, 24.6 kg/m2 versus 26.9 kg/m2, HR: 0.88; 95%CI: 0.79-0.99; p=0.039) and baseline creatinine (HR: 1.25; 95%CI: 1.04-1.49; p=0.017) were significantly associated with SI. All patients in the nephritic group (n=19; 100%) who experienced a SI received oral glucocorticoid (GC) treatment at the time of infection. Hypogammaglobulinemia was frequent (58.5%) but not associated with SI. Conclusions: After RTX administration, impaired kidney function and lower BMI are independent risk factors for SI. Patients with nephritic glomerular diseases having concomitant GC treatment might be at higher risk of developing SI. [ABSTRACT FROM AUTHOR]

Background: C3 glomerulopathy (C3GP) is characterized by deposition of complement C3 with absence/traces of immunoglobulins in the glomeruli and categorized into dense deposit disease (DDD), C3 glomerulonephritis (C3GN), complement factor H related protein 5(CFHR5) nephropathy etc. Collaborative efforts of pathologists, complement biologists and nephrologists worldwide are expanding the histomorphological pattern and laboratory findings related to C3GP. Hence, we studied point prevalence and morphological spectrum of C3GP in Indian patients to correlate morphological patterns with standard therapies and outcome of the patients. Methods: Retrospective analysis of renal biopsies (2007-2012,n-4565), which on immunofluorescence (IF) had C3 dominant deposits with absence or trace amount of immunoglobulin was carried out. Histopathology and electronmicroscopy (EM) were reviewed; cases were re-classified as DDD and C3GN. Histomorphological patterns of both groups were compared and correlated with treatment. Clinical details and follow up of patients were retrieved from the department of nephrology. Results: There were 31 cases (0.7%) of C3GP sub-classified as DDD (n-13) and C3GN (n-14). It was difficult to sub-classify 4 cases since EM showed overlapping features. C3GN and DDD had distinct clinical characteristics and disease outcome, though pathological features were overlapping. Majority of C3GP patients were males and were in 2nd to 4th decade of life. Nephrotic syndrome in DDD and nephritic-nephrotic presentation in C3GN patients was more common. Hypertension and oliguria were more often observed in C3GN than DDD. Membranoproliferative pattern (MPGN) was commonest pattern in DDD; other patterns seen were mesangial proliferative, mesangial expansive/nodular, exudative and crescentic. C3GN also had all the above patterns, the predominant ones being MPGN and mesangial proliferative. Limited follow-up revealed response to therapy only in C3GN (33%). Progression to ESRD was 33% in DDD and 10% cases in C3GN. Conclusion: C3GP comprise 0.7% of all renal biopsies. MPGN pattern was the commonest morphological pattern in DDD whereas MPGN and mesangial proliferative pattern were equally dominant patterns in C3GN. EM of 4 cases (13%) showed intermediate features. Evaluation of alternate complement pathway must be done in all cases to identify the point of dysregulated alternate complement pathway and to confirm the diagnosis in ambiguous cases. [ABSTRACT FROM AUTHOR]