Lupus , kidney transplant , glomerulonephritis

PURPOSE OF REVIEW: Rituximab is increasingly prescribed for glomerular diseases. However, the recently published Kidney Disease Improving Global Outcomes (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases lacks details on recommended dosing regimens for most individual glomerular diseases. We performed this scoping review summarizing the evidence for rituximab dosing in glomerular disease. SOURCES OF INFORMATION: PubMed database. METHODS: The PubMed search methodology was developed with a medical librarian and performed by the first, with review by a second, author...

Antiglomerular basement membrane disease (anti-GBM) is a rare life-threatening autoimmune vasculitis that involves small vessels and it is characterized by circulating autoantibodies directed against type IV collagen antigens expressed in glomerular and alveolar basement membrane. The typical clinical manifestations are the rapidly progressive glomerulonephritis and the alveolar hemorrhage. The diagnosis is usually confirmed by the detection of anti-GBM circulating antibodies. If not rapidly recognized, anti-GBM disease can lead to end stage kidney disease (ESKD)...

Angiotensin II type 1 receptor (AT1R) antibodies are considered non-HLA (human leukocyte antigen) antibodies connected with humoral rejection after kidney transplantation. The role of AT1R antibodies in the pathogenesis of glomerular diseases and systemic vasculitis is unknown. We assessed the level of AT1R antibodies in 136 patients with different types of glomerulonephritis and systemic vasculitis and we observed kidney function and proteinuria, serum albumin and total protein levels for 2 years. The mean levels of AT1R antibodies were the following: 6...

Pediatric renal biopsy is an uncommon event, and the spectrum of the disease is evaluated and managed mostly on the clinical grounds. Compared to adults, the indications for renal biopsy in pediatric population are very few. We reviewed the pediatric renal biopsies received at our tertiary center in Mumbai, India, over a period of six years to study the incidence of various medical renal diseases, their spectrum on histology and its correlation with electron microscopy (EM). A total of 65 pediatric renal biopsies in the age group of 0-12 years were evaluated over a period of six years...

Fibronectin glomerulopathy (FNG) is a rare inherited kidney disease characterized by extensive deposition of fibronectin in the glomeruli, especially in the mesangial and subendothelial regions. The disease progresses slowly and eventually leads to kidney failure in 15-20 years. Here, we report an interesting case. The patient presented with proteinuria and was diagnosed with immune complex-mediated glomerulonephritis, and lupus nephritis was suspected. This patient progressed to end-stage renal disease after 18 years and received an allogeneic kidney transplant...

Retinal drusen are characteristic of macular degeneration and complement activation, but also occur in C3, lupus and IgA nephropathy. This cross-sectional observational study compared drusen counts in different forms of glomerulonephritis. Consecutive individuals with glomerulonephritis attending a general renal or transplant clinic underwent retinal imaging with a non-mydriatic camera. Drusen were counted in deidentified images by trained graders, compared with matched hospital patients, and correlated with clinical features...

Rationale: Q fever is a zoonotic infection that may lead to acute or long-term renal injury. Given its rare incidence, Q fever is not often considered on the initial differential diagnosis for glomerular disease which can lead to delays in treatment. This case highlights the importance of avoiding early diagnostic closure and revisiting the differential diagnosis in the setting of an atypical clinical presentation or response to treatment. Presenting Concerns: A 52-year-old female was referred for assessment of possible glomerulonephritis...

The pattern of glomerular disease differs in incidence among the different geographical areas because of ethnicity, genetic variability, environmental factors, and socioeconomic conditions. The prevalence of pattern glomerular diseases varies from different parts of the world and from within the same country, the current study was performed to show the frequency of occurrence of primary and secondary glomerular disease (SGD) observed in a tertiary care hospital catering to patients from central India. In a retrospective study, we analyzed the clinical and pathological data of 176 kidney biopsies that were performed from 2016 to 2019 at the Department of Nephrology Super Speciality Hospital in Nagpur...

Full-house staining of glomeruli in renal pathology is highly suggestive of lupus nephritis. Other nonlupus entities can also present with a similar pattern on immune fluorescence. Different authors have used different names for this new entity with full house staining on immunofluorescence (IF) with negative serology for lupus. Some authors used the term full-house nephropathy for this new entity. The aim of our study is to define the clinicopathological spectrum and treatment outcomes of nonlupus "full-house" patterns...

Results from biopsy registries are important to know about the prevalence of renal diseases. In large studies done over several years, significant interobserver variability could have existed. Single-year biopsy registry data are analyzed in this study. The study included 481 renal biopsy specimens including 65 from allografts. Primary glomerulonephritis constituted 37.74% and secondary glomerular diseases constituted 32.21% of native kidney biopsies. Minimal change disease was the most common primary glomerular disease, followed by membranous nephropathy (MN)...

This study was conducted to retrospectively investigate the indications for renal biopsy in the native kidneys of children and to analyze the pathological findings in a single tertiary care hospital in North-East India for the past 12 years. All children (≤18 years) who underwent renal biopsy at our hospital from March 2007 to April 2018 were included. Renal tissue specimens were studied under light and immunofluorescence microscopy. The study group included 254 patients (female 57%). The median age was 15 years (range 6-18 years)...

In 2019 and 2021, the European League for Rheumatism (EULAR) jointly with the European Renal Association (ERA) and the Kidney Disease: Improving Global Outcomes (KDIGO), respectively, released updated guidelines on the management of lupus nephritis (LN). The Immunology Working Group of the ERA reviewed and compared both updates. Recommendations were either consistent or differences were of negligible clinical relevance for: indication for kidney biopsy, kidney biopsy interpretation, treatment targets, hydroxychloroquine dosing, first-line initial immunosuppressive therapy for active class III, IV (±V) LN, pregnancy in LN, LN in paediatric patients and LN patients with kidney failure...

OBJECTIVES: Pure membranous (class V) LN is considered a less aggressive phenotype, but tissue fibrosis and chronic kidney disease may still develop. This study aimed to elucidate the prognostic value of a history of class switch in pure membranous LN. METHODS: We included LN patients with at least two clinically indicated kidney biopsies. New onset of end stage kidney disease (ESKD) was defined as estimated glomerular filtration rate <15 ml/min/1.73 m2, initiation of dialysis or kidney transplantation...

The actions of immune cells within the kidney are of fundamental importance in kidney homeostasis and disease. In disease settings such as acute kidney injury, anti-neutrophil cytoplasmic antibody-associated vasculitis, lupus nephritis and renal transplant rejection, immune cells resident within the kidney and those recruited from the circulation propagate inflammatory responses with deleterious effects on the kidney. As in most forms of inflammation, intravital imaging - particularly two-photon microscopy - has been critical to our understanding of immune cell responses in the renal microvasculature and interstitium, enabling visualization of immune cell dynamics over time rather than statically...

OBJECTIVES: Kidney transplant (KTx) recipients with IgAN as primary disease, were compared with recipients with other causes of renal failure, in terms of long-term outcomes. METHODS: Ninety-nine KTx recipients with end-stage kidney disease (ESKD) due to IgAN, were retrospectively compared to; i/ a matched case-control group of patients with non-glomerular causes of ESKD, and ii/ four control groups with ESKD due to glomerular diseases; 44 patients with primary focal segmental glomerulosclerosis (FSGS), 19 with idiopathic membranous nephropathy (IMN), 22 with lupus nephritis (LN) and 21 with pauci-immune glomerulonephritis (PIGN)...

INTRODUCTION: Recurrence of anti-glomerular basement membrane (anti-GBM) glomerulonephritis in the kidney graft is a rare event, described in limited reports. The aim of this study was to evaluate, in a large cohort of patients with long follow-up, the risk of recurrence of anti-GBM disease, the risk factors associated with clinical recurrence, and the long-term patient and graft survival. METHODS: This was a multicenter retrospective study. Inclusion criteria were patients with anti-GBM glomerulonephritis who underwent transplantation of a kidney between 1977 and 2015...

BACKGROUND: We propose a novel clinically significant finding, de novo lupus-like glomerulonephritis (DNLLGN), in patients with autoantibodies and kidney abnormalities in pediatric liver transplant (LT) and intestinal inclusive transplants (ITx). METHODS: We describe the clinical, serologic, and histopathologic presentation and kidney outcomes in eight patients from our center found to have DNLLGN on kidney biopsy. RESULTS: Pediatric recipients of non-kidney solid organ transplants developed an unusual de novo immune complex glomerulonephritis with morphologic similarity to lupus nephritis...

BACKGROUND: Although high-affinity IgG auto- and alloantibodies are important drivers of kidney inflammation that can result in ESKD, therapeutic approaches that effectively reduce such pathogenic antibodies remain elusive. Erythropoietin (EPO) has immunomodulatory functions, but its effects on antibody production are unknown. METHODS: We assessed the effect and underlying mechanisms of EPO/EPO receptor (EPOR) signaling on primary and secondary, T cell-dependent and T-independent antibody formation using in vitro culture systems, murine models of organ transplantation and lupus nephritis, and mice conditionally deficient for the EPOR expressed on T cells or B cells...

BACKGROUND: While there are many cross-sectional studies of glomerulonephritis (GN) incidence, changes in incidence over time, particularly in the 21st century have received less attention. Similarly, little is known about temporal changes in GN prognosis. The presence in Denmark of comprehensive registries for renal biopsy results, end-stage renal disease (ESRD), comorbidity and mortality permit these questions to be addressed. METHODS: Data for all renal biopsies in Denmark between 1985 and 2014 were extracted from the Danish Renal Biopsy Registry and Patobank registries...

Attraction, retention, and differentiation of leukocytes to and within the kidney are governed by chemokines. The chemokine CX3CL1 (fractalkine) and its receptor CX3CR1 are exemplary in this regard as they are highly expressed and further upregulated in a range of kidney diseases. CX3CL1 is chiefly produced by renal endothelium and tubular epithelium, where it promotes leukocyte attraction. Recent data suggest that in addition to established soluble mediators, cellular interactions may enhance CX3CL1 expression...