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Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome, which may present as a sub-nephrotic or nephrotic range proteinuria with hypoalbuminemia, hyperlipidemia, and edema. It is an immune-mediated glomerular disease that is pathologically characterized by glomerular intra-membranous and sub-epithelial immune complex deposits (immunoglobulin G4 [IgG4] and complement 3 [C3]) causing membrane thickening.
The pathophysiology of MN was first described by the Heymann nephritis rat model in 1959 (
Podocytopathies are a group of kidney diseases caused by direct or indirect injury to the glomerular podocytes, resulting in proteinuria. Examples of podocytopathies are minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and collapsing glomerulopathy (CG) (
Alport syndrome is an inherited kidney disease characterized by abnormalities in the glomerular basement membrane and is associated with hearing loss, ocular anomalies, and risk for progressive loss of kidney function. Alport syndrome accounts for 3% of children with chronic kidney disease (CKD) and 0.2% of adults with kidney failure in the United States (
In 1914, Volhard and Fahr (
Lupus nephritis is a serious end organ manifestation of systemic lupus erythematosus (SLE). Regardless of the remarkable advances in the knowledge and understanding of lupus nephritis pathophysiology, it remains a weighty source of morbidity and mortality, and 10% to 30% of affected patients progress to end-stage kidney disease within 10 years of being diagnosed with SLE (
Therapy for lupus nephritis has continued to evolve, from the use of cyclophosphamide, azathioprine, and steroids developed in the 1970s–1980s to the use of mycophenolate, tacrolimus, cyclosporine, and rituximab in the 2000s (
During the last decade, several therapeutics targeting the complement cascade have begun to enter the nephrology scene ( Atypical hemolytic uremic syndrome (aHUS) C3 glomerulopathy Lupus nephritis Anti-neutrophil cytoplasmic antibody (ANCA) vasculitis Immunoglobulin A (IgA) nephropathy
Summary of the drugs that target the complement cascade in glomerular disease
In November 2020, the US Food and Drug Administration (FDA) approved lumasiran (brand name “Oxlumo”), the first medical therapy specifically for the management of primary hyperoxaluria type 1 (PH1), a rare and life-threatening disease that often progresses to kidney failure. This announcement may represent a breakthrough, not only in the treatment of PH1 but also in drug development for a host of rare kidney diseases.
PH1 is caused by a congenital defect in the hepatic enzyme alanine glyoxylate aminotransferase, resulting in a failure to metabolize glyoxylate to glycine and the toxic accumulation of oxalate. The buildup of unmetabolized oxalate leads
Tolvaptan, an oral selective vasopressin V2 receptor antagonist, was approved by the US Food and Drug Administration (FDA) for the treatment of clinically significant hypervolemic or euvolemic hyponatremia and rapidly progressing autosomal dominant polycystic kidney disease (ADPKD). It antagonizes the effect of an arginine vasopressin (antidiuretic hormone), which has a key role in water and circulatory homeostasis in the collecting duct of the kidney. Tolvaptan leads to an increase in urine water excretion (aquaresis) that results in enhanced free-water clearance in states of relative vasopressin excess, increasing serum sodium concentrations. Additionally, tolvaptan induces a reduction in cyclic adenosine monophosphate (cAMP),
For more than 30 years, erythropoiesis-stimulating agents (ESAs) have reigned supreme as the treatment for chronic kidney disease (CKD)–related anemia. Can hypoxia-inducible factor-prolyl hydroxylase inhibitors (HIF-PHIs) topple ESAs? HIF-PHIs are oral medications taken three times a week or daily and have been shown in trials to achieve and maintain goal hemoglobin to the same degree as ESAs. HIF-PHIs are small molecules that inhibit the prolyl hydroxylase enzyme that continually marks the HIF for degradation. Each dose transiently increases intracellular HIF2a, a transcription factor, leading to activation of a series of genes, including erythropoietin (EPO) and several iron transport genes. Consequently,
Uremic pruritus is a common, distressing condition that affects 60% of patients on hemodialysis (
Given that uremic toxins likely contribute to the symptoms of uremic pruritus, increasing dialysis dose