Current approaches to overcome recurrent focal segmental glomerulosclerosis after kidney transplantation.

PURPOSE OF REVIEW: Recurrent focal segmental glomerulosclerosis (FSGS) presents with nephrotic syndrome shortly after kidney transplantation. This review will overview the role of circulating permeability factors in disease pathogenesis and treatment options for recurrent FSGS. RECENT FINDINGS: Novel circulating permeability factors have been identified in serum samples. Current research is focused on detection of permeability factors as a marker of treatment response. Furthermore, novel monoclonal antibodies are being utilized to further induce remission. SUMMARY: Posttransplant recurrent FSGS can have a deleterious effect on allograft. Early detection of disease recurrence with prompt treatment is optimal for clinical remission. Plasmapheresis with anti-B cell therapy is considered the mainstay of treatment. Newer B cell therapies and detection of circulating factors in serum may help in providing targeted treatment in a subset of patients.

Antigens, antibodies, and membranous nephropathy: a decade of progress.

Membranous nephropathy (MN) is either primary or associated with various etiologies, each with unique glomerular antigens. The discovery of the phospholipase A2 receptor (PLA2R) antigen in primary MN revolutionized our understanding of MN and led to major clinical progress. Other recently discovered antigens in MN include the THSD7A antigen and exostosin. Sethi et al. have now identified a new antigen, NELL-1, in primary MN, again decreasing the number of patients whose antigen remains unknown.

C3 Glomerulopathy: Pathogenesis and Treatment.

C3 glomerulopathy (C3G) is a rare set of kidney diseases with 2 patterns: C3 glomerulonephritis (C3GN) and dense deposit disease. Pathogenesis of both diseases is due to complement dysregulation in the alternative pathway. Acquired or genetic alterations of the regulatory proteins of the complement pathway result in C3G. Although the disease is characterized by low C3 levels in serum and C3-dominant staining by immunofluorescence on biopsy, other disease entities such as infection-related glomerulonephritis and masked monoclonal deposits can present similarly. Both the C3GN and dense deposit disease variants of C3G are progressive and recur in transplanted kidneys. Although no direct treatment is available, complement blockers are either available or in the clinical trial phase. This review will survey the pathogenesis of C3GN and current treatment options.