The effects of plasma exchange and glucocorticoids on early kidney function among patients with ANCA-associated vasculitis in the PEXIVAS trial.

Therapeutic plasma exchange (PLEX) is an adjunctive treatment for patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and kidney involvement. Little is known about the effect of PLEX on early changes in kidney function. This post-hoc analysis of the PEXIVAS trial investigated the effects of PLEX on changes in kidney function within 12 months.

Rapidly Progressive Glomerulonephritis.

Rapidly progressive glomerulonephritis (RPGN) is a syndrome characterized by a swift decline in kidney function, often over a few months, accompanied by features of nephritic syndrome. It can result in decreased urine output and commonly involves the presence of extensive crescents in kidney biopsies. RPGN is classified into 3 main types based on immune deposit distribution and visualization through immunofluorescence and electron microscopy: antiglomerular basement membrane disease, immune complex glomerulonephritis, and pauci-immune glomerulonephritis.

Clinical Presentation and Treatment Outcomes of Renal Medullary Angiitis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Single-Center Case Series.

Introduction: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with renal involvement primarily affects the renal cortex and presents with key histopathologic findings of a pauci-immune necrotizing and crescentic glomerulonephritis. Infrequently reported and poorly characterized is renal medullary angiitis (RMA), a pathologic variant of AAV primarily involving the renal medulla. This study seeks to describe the presentation and treatment outcomes of RMA.

Structural and theoretical exploration of a multi-methoxy chalcone: Synthesis, quantum theory, electrostatics, molecular packing, DFT analysis, and in-silico anti-cancer evaluation.

This study explores the pharmacological potential of chalcones through a multidisciplinary approach, including synthesis, quantum theory, molecular electrostatics, and density functional theory (DFT) calculations. The synthesized compound, analyzed via single crystal X-ray diffraction, crystallized in the triclinic system (space group P-1) with C-H⋯O interactions stabilizing its structure. Hirshfeld surface analysis confirms these interactions, with H-H contacts dominating (45.

Outcome of pregnancy in sickle cell anemia patients with COVID-19 infection.

Sickle cell anemia (SCA) is a multisystem disease, associated with increased risk for infection and thromboembolic disease, and pregnancy is a stressor for patients with SCA. In general, coronavirus disease 2019 (COVID-19) infection in SCA is associated with a favorable outcome. Literature of pregnancy in SCA with COVID is scarce.

Real-World Experience With Avacopan in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis.

Introduction: Postmarketing data on outcomes of avacopan use in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) are lacking.

Methods: We performed a multicenter retrospective analysis of 92 patients with newly diagnosed or relapsing AAV who received therapy with avacopan. The coprimary outcome measures were clinical remission at 26 and 52 weeks.

Kidney Failure in Pauci-immune Crescentic Glomerulonephritis: Rationale for Immunosuppression to Improve Kidney Outcome.

Purpose Of Review: Pauci-immune crescentic glomerulonephritis is the hallmark finding in ANCA-associated vasculitis (AAV) when the kidneys are affected. The rationale for immunosuppression in AAV is based on the underlying autoimmune nature of the disease. Overall remission rates, kidney outcomes, and the burden of disease have greatly improved since the discovery of various immunosuppressive therapies, but relapses remain common, and a significant proportion of patients continue to progress to end-stage kidney disease.

Histologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis.

Background: Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephritis has not been investigated in large multiethnic cohorts.

Methods: Biopsies from 262 children and 99 adults with IgA vasculitis nephritis ( N =361) from 23 centers in North America, Europe, and Asia were independently scored by three pathologists.

COVID vaccine-induced lupus nephritis: Case report and review of the literature.

Vaccines offer an effective strategy to prevent infectious diseases with minimal adverse effects. On rare occasions, vaccination can disrupt the immune response leading to induction of autoimmune diseases. We describe a case of new-onset lupus nephritis following COVID-19 vaccination with the first dose of the Pfizer vaccine.

The Improved Kidney Risk Score in ANCA-Associated Vasculitis for Clinical Practice and Trials.

Significance Statement: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. More than 1500 patients were collated in an international longitudinal study to revise the ANCA kidney risk score. The score showed satisfactory performance, mimicking the original study (Harrell's C=0.

Efficacy and safety of avacopan in patients with ANCA-associated vasculitis receiving rituximab in a randomised trial.

Objectives: To evaluate the efficacy and safety of avacopan in the subgroup of patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis receiving background induction therapy with rituximab in the phase 3 ADVOCATE trial.

Methods: Key efficacy outcomes were remission at week 26 and sustained remission at week 52. Additional outcomes included the Glucocorticoid Toxicity Index, estimated glomerular filtration rate, urinary albumin to creatinine ratio, health-related quality of life and safety.

Glomerular Diseases Across Lifespan: Key Differences in Diagnostic and Therapeutic Approaches.

Glomerular diseases are common causes of chronic kidney disease in childhood, adolescence, and adulthood. The epidemiology of glomerular diseases differs between different age groups, with minimal change disease being the leading cause of nephrotic syndrome in childhood, while membranous nephropathy and focal segmental glomerulosclerosis are more common in adulthood. IgA vasculitis is also more common in childhood.

COVID-19 outcomes in patients with a history of immune-mediated glomerular diseases.

Introduction: Patients with immune-mediated glomerular diseases are considered at high risk for severe COVID-19 outcomes. However, conclusive evidence for this patient population is scarce.

Methods: We created a global registry and retrospectively collected clinical data of patients with COVID-19 and a previously diagnosed immune-mediated glomerular disease to characterize specific risk factors for severe COVID-19 outcomes.

Crystal structure studies and Hirshfeld surface analysis of 4-(di-methyl-aza-nium-yl)-2-hy-droxy-anilinium dichloride monohydrate at 90 K.

The crystal structure and a Hirshfeld surface analysis of the substituted anilinium salt 4-(di-methyl-aza-nium-yl)-2-hy-droxy-anilinium dichloride monohydrate, CHNO·2Cl·HO, at low temperature (90 K) are presented. The organic cation is essentially planar: the r.m.

Crystal structure and Hirshfeld-surface analysis of a monoclinic polymorph of 2-amino-5-chloro-benzo-phenone oxime at 90 K.

The synthesis and crystal structure of a monoclinic polymorph of 2-amino-5-chloro-benzo-phenone oxime, CHClNO, are presented. The mol-ecular conformation results from twisting of the phenyl and 2-amino-5-chloro benzene rings attached to the oxime group, which subtend a dihedral angle of 80.53 (4)°.

KDOQI US Commentary on the 2021 KDIGO Clinical Practice Guideline for the Management of Glomerular Diseases.

The KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases represents the first update to this set of recommendations since the initial set of KDIGO guideline recommendations was published in 2012. The pace of growth in our molecular understanding of glomerular disease has quickened and a number of newer immunosuppressive and targeted therapies have been introduced since the original set of guideline recommendations, making such an update necessary. Despite these updates, many areas of controversy remain.

Treatment Using Both Voclosporin and Belimumab in Four Patients With Lupus Nephritis.

Nearly 50% of patients with systemic lupus erythematosus (SLE) will develop lupus nephritis (LN). Current treatment regimens for LN are suboptimal as the majority of patients fail to achieve complete renal response after several months of treatment and there are high rates of relapse. We report outcomes in four LN patients who were treated with both voclosporin and belimumab.

Synthesis and crystal structure studies of 5-(tri-fluoro-meth-yl)-1,3,4-thia-diazol-2(3)-one at 180 K.

The synthesis and crystal structure of CHFNOS, systematic name 5-(tri-fluoro-meth-yl)-1,3,4-thia-diazol-2(3)-one (5-TMD-2-one), a compound containing the pharmacologically important heterocycle 1,3,4-thia-diazole, is presented. The asymmetric unit comprises six independent mol-ecules (' = 6), all of which are planar. The r.

Challenges of defining renal response in ANCA-associated vasculitis: call to action?

Avoiding end-stage kidney disease in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has a high therapeutic priority. Although renal response is a crucial measure to capture clinically relevant changes, clinal trials have used various definitions and no well-studied key surrogate markers to predict renal outcome in AAV exist. Differences in clinical features and histopathologic and therapeutic approaches will influence the course of kidney function.