Glomerular diseases

This is a summary of diseases that affect the glomerulus of the kidney, including those that case nephrotic and nephritic syndromes. I created this presentation with Google Slides. Image were created or taken from Wikimedia Commons I created this video with the YouTube Video Editor. ADDITIONAL TAGS: Glomerular diseases Minimal change disease Focal segmental glomerulosclerosis Membranous glomerulonephritis Diabetic nephropathy Amyloid nephropathy Lupus nephritis Membranoproliferative glomerulonephritis IgA nephropathy Acute postinfectious glomerulonephritis Anti-GBM disease ANCA glomerulonephritis Nephrotic Nephritic Systemic Primary glomerular disease with nephrotic syndrome Most common in children, especially young children LM: “minimal change” -- looks normal IF: negative (no immune complex deposition) EM: foot process effacement, see figure secondary causes: cancer, infection, drugs, atopy (hyperallergic) Associated with lymphoma and use of NSAIDs Primary glomerular disease with nephrotic syndrome Focal means some glomeruli, segmental means not all of glomerulus LM: segmental glomerular scarring, see hyaline material (deposits from plasma) IF: positive for Ig and complement, granular appearance EM: segmental effacement Primary FSGS is idiopathic Secondary causes: genetic, infection (HIV, parvovirus), drugs (heroin), sickle cell, obesity Most common in blacks Primary glomerular disease with nephrotic syndrome LM: capillary wall thickening with IgG and C3 immune complexes IF: positive for IgG and C3 in the capillary, granular EM: immune complexes in subEPIthelial space Primary cause: Ab against anti-phospholipase A2 receptor Secondary causes: cancer, lupus, NSAIDs, HBV, Hep B, syphilis… Ag can be Hep B or cancer Secondary nephropathy with nephrotic syndrome; DM is primarily systemic disease Caused by DM types I and II → accumulation of glycosylated plasma protein in GBM and mesangium LM: mesangial expansion and KW nodules IF: linear staining of IgG EM: thickened GBM Treatment is reduce blood sugar, lower blood pressure, avoid nephrotoxins Most common cause for end stage renal disease in United States Secondary nephropathy with nephrotic syndrome; amyloidosis is primarily a systemic disease Caused by accumulation of polypeptides (especially AL and AA amyloid) LM: thickening of mesangium amorphous pale pink stuff in glomerulus, confirm with Congo Red stain IF: monoclonal staining of accumulated amyloid protein EM: randomly arranged fibrils Associated with rheumatoid arthritis and multiple myeloma Ranked by class: I (best) to VI (worst) Antigen antibody complexes deposit in gloms, activate complement which leads to proliferation of mesangium and infiltration of PMNs LM: endocapillary and mesangial proliferation and sometimes crescents IF: granular pattern, everything lights up! “full house pattern” EM: deposits anywhere and everywhere Hypocomplementemia - both C3 and C4 blood levels are low Presents with nephritic and/or nephrotic syndrome; kind of in between Type I is immune complex and C3 deposits Caused by bacterial infection, hep C infection, malignancies Type II is just C3 deposits but no Ig Caused by complement dysregulation LM: capillary wall thickening with hypercellularity IF: immune complexes and/or C3 granular deposits causing hypocomplementemia EM: same deposits (subendo and BM) Deposits of IgA alone or with other Igs, in mesangium Activates complement, which causes proliferation of mesangial cells LM: mesangial hypercellularity IF: IgA positive, granular pattern in the mesangium EM: mesangial deposits Primary IgA nephropathy is idiopathic Can be part of systemic disease IgA vasculitis; related to Henoch– Schönlein purpura Occurs few weeks after infection; most often follows strep or staph LM: endocapillary and mesangial hypercellularity and PMNs IF: pos for C3 in capillary walls, granular EM: subepithelial humps Hypocomplementemia; low C3 levels Treatment is supportive Nephritic Caused by autoAb to glomerular basement membrane Abs recruit complement and lymphocytes → damages capillary → proliferation and accumulation in Bowman’s space → crescent LM: crescent formation IF: pos for IgG in linear pattern EM: normal Disease is called Goodpasture’s syndrome when presents with both kidney and lung involvement LM: crescents, necrosis IF: pauci-immune; not much lights up EM: normal Associated diseases: Granulomatosis with polyangiitis (GPA); Wegener's; PR3 Eosinophilic granulomatosis with polyangiitis (eGPA); Churg–Strauss churg Strauss Microscopic polyangiitis (MPA)