Trends of preemptive kidney transplantation in lupus compared to other primary diseases leading to end stage kidney disease in the United States of America.

PurposeThe proportion of lupus patients with end-stage kidney disease (ESKD) undergoing preemptive kidney transplantation (PKT) is <5%, due to concern for poor outcomes. In this study, we estimate trends and the likelihood of PKT in lupus patients compared to non-lupus patients approaching ESKD. Additionally, we compare kidney allograft failure (KAF) and all-cause mortality in lupus and non-lupus recipients of PKT.

2024 American College of Rheumatology (ACR) Guideline for the Screening, Treatment, and Management of Lupus Nephritis.

Objective: The objective is to provide evidence-based and expert guidance for the screening, treatment, and management of lupus nephritis.

Methods: The Core Team developed clinical questions for screening, treatment, and management of lupus nephritis using the PICO format (population, intervention, comparator, and outcome). Systematic literature reviews were completed for each PICO question, and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology was used to assess the quality of evidence and to formulate recommendations.

2024 American College of Rheumatology (ACR) Guideline for the Screening, Treatment, and Management of Lupus Nephritis.

Objective: The objective is to provide evidence-based and expert guidance for the screening, treatment, and management of lupus nephritis.

Methods: The Core Team developed clinical questions for screening, treatment, and management of lupus nephritis using the PICO format (population, intervention, comparator, and outcome). Systematic literature reviews were completed for each PICO question, and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology was used to assess the quality of evidence and to formulate recommendations.

Unveiling renal pathology's potential: exploring a rare subtype of amyloid - apolipoprotein CII amyloidosis in the youngest patient: a case report and literature review.

In this clinical case report, we present a rare subtype of amyloidosis, apolipoprotein CII (apo CII), which was diagnosed through a renal biopsy and subsequently confirmed by identifying the p.K41T mutation via germline DNA sequencing. Upon reviewing the literature, five patients exhibiting identical mutation were identified via renal biopsy, while an additional patient was diagnosed through biopsies of the fat pad and bone marrow.

Mycophenolate mofetil withdrawal in patients with systemic lupus erythematosus: a multicentre, open-label, randomised controlled trial.

Background: Mycophenolate mofetil is an immunosuppressant commonly used to treat systemic lupus erythematosus (SLE) and lupus nephritis. It is a known teratogen associated with significant toxicities, including an increased risk of infections and malignancies. Mycophenolate mofetil withdrawal is desirable once disease quiescence is reached, but the timing of when to do so and whether it provides a benefit has not been well-studied.

Associations Between Albuminuria and Mortality Among US Adults by Demographic and Comorbidity Factors.

Background Albuminuria is a known marker of mortality risk. Whether the association between albuminuria and mortality differs by demographic and comorbidity factors remains unclear. Therefore, we sought to determine whether albuminuria is differentially associated with mortality.

Blood Pressure and Cardiovascular Disease Mortality Among US Adults: A Sex-Stratified Analysis, 1999-2019.

Background: Most research examining the association between blood pressure (BP) and cardiovascular disease (CVD) is sex-agnostic. Our goal was to assess sex-specific associations between BP and CVD mortality.

Methods: We combined ten cycles of the National Health and Nutrition Examination Survey (1999-2018), N=53 289.

Identification of Glomerular and Plasma Apolipoprotein M as Novel Biomarkers in Glomerular Disease.

Introduction: Dysregulation of sphingolipid and cholesterol metabolism contributes to the pathogenesis of glomerular diseases (GDs). Apolipoprotein M (ApoM) promotes cholesterol efflux and modulates the bioactive sphingolipid sphingosine-1-phosphate (S1P). Glomerular ApoM expression is decreased in patients with focal segmental glomerulosclerosis (FSGS).

One-year all-cause mortality in hospitalized patients with COVID-19 and end-stage renal disease undergoing hemodialysis.

Introduction: Patients with end-stage renal disease (ESRD) on dialysis and COVID-19 infection have an increased risk of in-hospital mortality, but whether these patients have a higher long-term mortality risk is unknown.

Materials And Methods: Retrospective chart review of 958 patients admitted with COVID-19 infection or those with ESRD admitted for any other reason between February 2020 and August 2020. We collected data on demographics, comorbidities, laboratory tests, and mortality.

Food protein-induced enterocolitis syndrome with pneumatosis intestinalis in an exclusively breastfed infant: A case report and literature review.

A 1-month-old male, exclusively breastfed, presented with 24 h of bloody stools, vomiting, metabolic acidosis, and pneumatosis intestinalis. The patient was initially treated for necrotizing enterocolitis (NEC). However, after suspecting food protein-induced enterocolitis syndrome (FPIES), oral feeding was resumed using an exclusive elemental formula, and the biochemical and radiological findings were resolved.

Safety and Efficacy of Belimumab in Patients with Lupus Nephritis: Open-Label Extension of BLISS-LN Study.

Background And Objectives: In the BLISS-LN study, belimumab improved kidney outcomes in adult patients with active lupus nephritis. This 28-week open-label extension of BLISS-LN assessed belimumab's safety and efficacy.

Design, Setting, Participants, & Measurements: Eligible patients completing BLISS-LN received monthly intravenous belimumab 10 mg/kg plus standard therapy.

A secondary analysis of the Belimumab International Study in Lupus Nephritis trial examined effects of belimumab on kidney outcomes and preservation of kidney function in patients with lupus nephritis.

We performed a post hoc analysis of the Belimumab International Study in Lupus Nephritis (BLISS-LN), a Phase 3, multinational, double-blind, 104-week trial, in which 448 patients with lupus nephritis were randomized to receive intravenous belimumab 10 mg/kg or placebo with standard therapy (cyclophosphamide/azathioprine or mycophenolate mofetil). Add-on belimumab was found to be most effective in improving the primary efficacy kidney response and complete kidney response in patients with proliferative lupus nephritis and a baseline urine protein/creatinine ratio under 3 g/g. However, there was no observed improvement in the kidney response with belimumab treatment in patients with lupus nephritis and sub-epithelial deposits or with a baseline protein/creatinine ratio of 3 g/g or more.

Discoidin domain receptor 1 activation links extracellular matrix to podocyte lipotoxicity in Alport syndrome.

Background: Discoidin domain receptor 1 (DDR1) is a receptor tyrosine kinase that is activated by collagens that is involved in the pathogenesis of fibrotic disorders. Interestingly, de novo production of the collagen type I (Col I) has been observed in Col4a3 knockout mice, a mouse model of Alport Syndrome (AS mice). Deletion of the DDR1 in AS mice was shown to improve survival and renal function.

A Multicenter Randomized Clinical Trial of Hemodialysis Access Blood Flow Surveillance Compared to Standard of Care: The Hemodialysis Access Surveillance Evaluation (HASE) Study.

Introduction: Arteriovenous (AV) access thrombosis remains 1 of the most troubling AV access-related complications affecting hemodialysis patients. It necessitates an urgent and occasionally complicated thrombectomy procedure and increases the risk of AV access loss. AV access stenosis is found in the majority of thrombosed AV accesses.

Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis.

Background: In adults with active lupus nephritis, the efficacy and safety of intravenous belimumab as compared with placebo, when added to standard therapy (mycophenolate mofetil or cyclophosphamide-azathioprine), are unknown.

Methods: In a phase 3, multinational, multicenter, randomized, double-blind, placebo-controlled, 104-week trial conducted at 107 sites in 21 countries, we assigned adults with biopsy-proven, active lupus nephritis in a 1:1 ratio to receive intravenous belimumab (at a dose of 10 mg per kilogram of body weight) or matching placebo, in addition to standard therapy. The primary end point at week 104 was a primary efficacy renal response (a ratio of urinary protein to creatinine of ≤0.

Sterol-O-acyltransferase-1 has a role in kidney disease associated with diabetes and Alport syndrome.

Defective cholesterol metabolism primarily linked to reduced ATP-binding cassette transporter A1 (ABCA1) expression is closely associated with the pathogenesis and progression of kidney diseases, including diabetic kidney disease and Alport Syndrome. However, whether the accumulation of free or esterified cholesterol contributes to progression in kidney disease remains unclear. Here, we demonstrate that inhibition of sterol-O-acyltransferase-1 (SOAT1), the enzyme at the endoplasmic reticulum that converts free cholesterol to cholesterol esters, which are then stored in lipid droplets, effectively reduced cholesterol ester and lipid droplet formation in human podocytes.

Outcomes in adults with systolic blood pressure between 130 and 139 mmHg in Action to Control Cardiovascular Risk in Diabetes Blood Pressure trial and Systolic Blood Pressure Intervention Trial.

Background: Patients with stage 1 systolic hypertension have increased risk of cardiovascular disease (CVD) events.

Methods: Using Cox models, we assess the effect of targeting an intensive SBP goal of less than 120 mmHg compared with standard SBP goal of less than 140 mmHg on the risk of CVD events in adults with stage 1 systolic hypertension with diabetes mellitus enrolled in Action to Control Cardiovascular Risk in Diabetes Blood Pressure trial (ACCORD BP) (n = 1901) and without diabetes mellitus enrolled in Systolic Blood Pressure Intervention Trial (SPRINT) (n = 3484) that used identical SBP goal interventions.

Outcomes: In ACCORD BP, the primary composite CVD outcome was the first occurrence of myocardial infarction, stroke, or CVD mortality.

Effect of blood pressure control on sudden death risk score in the SPRINT trial.

Background: Recent data suggest that population screening for risk of sudden cardiac death (SCD) may be feasible with risk scores that can be implemented in the electronic health record. But, there are no established therapeutic strategies to target lowering risk for SCD in the general population. Our aim was to evaluate the effect of the Systolic Blood Pressure Intervention Trial (SPRINT) intensive blood pressure intervention on SCD risk and cardiovascular (CV) outcomes.

The impact of central IRB's on informed consent readability and trial adherence in SPRINT.

Background: Federal agencies have encouraged the use of central institutional review boards (CIRBs) for multi-site clinical trials. There is limited evidence supporting the use of CIRBs. Our aim is to evaluate how SPRINT sites regulated by CIRBs performed regarding informed consent readability and participant trial adherence compared to those regulated by local IRBs.

Implications of Early Decline in eGFR due to Intensive BP Control for Cardiovascular Outcomes in SPRINT.

Background: The Systolic BP Intervention Trial (SPRINT) found that intensive versus standard systolic BP control (targeting <120 or <140 mm Hg, respectively) reduced the risks of death and major cardiovascular events in persons with elevated cardiovascular disease risk. However, the intensive intervention was associated with an early decline in eGFR, and the clinical implications of this early decline are unclear.

Methods: In a analysis of SPRINT, we defined change in eGFR as the percentage change in eGFR at 6 months compared with baseline.

Modifying Effect of Statins on Fatal Outcomes in Chronic Kidney Disease Patients in the Systolic Blood Pressure Intervention Trial: A Post Hoc Analysis.

Background: Management of chronic kidney disease (CKD) patients includes efforts directed toward modifying traditional cardiovascular risk factors. Such efforts include optimal management of hypertension together with the initiation of statin therapy.

Methods: In this observational study, we determine the modifying effect of statins on the relationship of systolic blood pressure (SBP) goal with mortality and other outcomes in patients with CKD participating in a clinical trial.

Baseline blood pressure control in Hispanics: characteristics of Hispanics in the Systolic Blood Pressure Intervention Trial.

The Systolic Blood Pressure Intervention Trial (SPRINT) tested whether a systolic blood pressure (SBP) value <120 mm Hg reduces adverse clinical outcomes compared with the goal of <140 mm Hg. Here the authors describe the baseline characteristics of Hispanic participants in SPRINT. Nondiabetic hypertensive patients 50 years and older with SBP 130-180 mm Hg taking zero to four blood pressure (BP) medications were enrolled from the mainland United States and Puerto Rico.

Non-GFR Determinants of Low-Molecular-Weight Serum Protein Filtration Markers in CKD.

Background: Unlike the case with creatinine, conditions affecting the non-glomerular filtration rate (GFR) determinants of low-molecular-weight serum proteins, β-trace protein (BTP), β-microglobulin (B2M), and cystatin C, are not well characterized.

Study Design: Pooled cross-sectional analysis of 3 studies.

Setting & Participants: 3,156 persons with chronic kidney disease from the MDRD (Modification of Diet in Renal Disease) Study, AASK (African American Study of Kidney Disease and Hypertension), and CRIC (Chronic Renal Insufficiency Cohort) Study.

ESKD, Transplantation, and Dialysis in Lupus Nephritis.

Kidney disease resulting from systemic lupus erythematosus accounts for 1.9% of the end-stage kidney disease (ESKD) population in the United States. Systemic lupus erythematosus patients with lupus nephritis (LN) who progress to ESKD in the United States are mostly female (81%) and of African ancestry (49%), with a mean age of 41 years at initiation of renal replacement therapy (RRT).