Primary glomerular disease

Abstract

This is a review of the clinical features, pathogenesis, investigation and management of glomerulonephritis (GN). GN can occur as a primary isolated renal disease, as a manifestation of systemic diseases such as vasculitis or lupus, or secondary to drugs, infections or tumours. It is an important cause of morbidity and mortality and a potentially preventable cause of end-stage renal disease; early diagnosis is vital to allow timely referral to specialist units where renal biopsy can be performed. Proteinuria and/or haematuria are typical findings. Pathogenesis involves immune cells and inflammatory mediators, with intrinsic glomerular cells, especially podocytes, also being important in glomerular injury and the responses to it. I present brief outlines of schemes for appropriate investigations when GN is suspected, and guidelines for referral strategies to investigate proteinuria and haematuria, and manage common forms of GN. When nephrotic syndrome (NS) is present, it can lead to major morbidity and potential mortality even if excretory renal function is well preserved. NS should be managed, irrespective of the cause, with diuretics, antiproteinuric agents, cholesterol-lowering agents and sometimes anticoagulants. Treatment with corticosteroids, with or without other immunosuppressive agents, is effective in many forms of GN, but adverse effects are problematic. More selective forms of immunotherapy are increasingly used as understanding of the pathogenesis of GN improves but there is a continuing need for better collaboration on clinical trials so that the evidence base can be improved.