Clin pharm - GN

Presentations of GN Nephrotic syndrome Cardinal features proteinuria >3.5 g oedema hypoalbuminuria 2ndary thrombosis, hypercholesterolaemia, infections (if recurrent, offer prophylaxis) Not usually associated with renal failure (if failing consider overhydration, renal vein thrombosis image with USS) Nephritic syndrome Cardinal features Renal failure Haematuria (with abnormal red cells on microscopy acanthocytes, red cell casts) Hypertension

Syndromes of GN Minimal change disease Only pathology to cause pure nephrotic syndrome Light microscopy: normal Immunofluorescence: normal EM: podocyte effacement Rx: steroids Membranous Basically nephrotic, but may have 1+ blood Light microscopy: nothing other than BM thickening Immunofluorescence: EM: BM thickening with subendothelial/subepithelial dense deposits and spikes Associations: HBV, EBV, haematological malignancies, lung and colon ca., drugs (gold, penicillamine main differential for nephrotic in RA is AA amyloidosis causing FSGS) Rx: steroids (although often resistant, and commonly becomes chronic) FSGS Basically nephrotic, but may have 1+ blood Not a Dx consider DM and amyloidosis (AL/AA) as causes Kimmelstiel-Wilson nodules (amorphous blob of proteinaceous material) = DM Apple green fluorescence on Congo red stain = amyloidosis Membranoproliferative Nephritic syndrome with high protein leak (approaching nephrotic levels) Causes acute severe renal failure Associations: HCV, cryoglobulinaemia, partial lipodystrophy IE: nephritic[footnoteRef:1]; look for peripheral stigmata; signs occur at height of infection [1: Commonly associated with crescentic GN, deposition visible on immunofluorescence (if IgA only = IgAN); granular staining is present on immunofluorescence in type III conditions (due to distal antibody formation, e.g. IgAN, post-infectious) cf. Goodpastures, where there is linear deposition of IgG directly targeting the BM]

Post-infectious: nephritic; do an ASOT; occurs a few weeks after infection IgA: nephritic; commonest worldwide, often incidental finding a few days after infection; if renal function deteriorates, a renal biopsy is indicated (often triggered by infection) Goodpastures nephritic; pulmonary-renal syndrome Vasculitis: nephritic GPA: pulmonary-renal syndrome with nasal Sx; +ve cANCA vs proteinase-3 MPA: vasculitic rash and renal impairment; +ve pANCA Churg-Strauss (asthma, eosinophilia, vasculitis; +ve pANCA vs. myeloperoxidase; can be triggered by LTRAs) SLE: can do anything

Pathological patterns Focal segmental: only some of some glomeruli are affected Diffuse segmental: only some of all glomeruli are affected Focal global: all of some glomeruli are affected Diffuse global: all of all glomeruli are affected Sclerosis: scarring without inflammation (thickening within the glomeruli without influx of inflammatory cells) Glomerulonephritis: inflammation present