======Glomerular Disease====== =====Nephrotic Syndrome===== * =====Nephritic Syndrome===== * Low complement: post-infectious GN, SLE, MPGN * Normal complement: IgA, Anti-GM (Goodpasture's) =====Epidemiology===== * Age < 15 years: * Nephrotic: MCD, FSGS * Nephritic: IgA, Hsp, post-infectious GN, MPGN, Alport * Age 15-40 years: * Nephrotic: * Nephritic: * Age > 40 years: * Nephrotic: * Nephritic: =====Common causes===== * Hypocomplementemic * Primary renal: acute post-strep, MPGN * Systemic disease: SLE, IE, cryo, HCV * Normocomplementemics * Other infections (uncomm): M. leprae, P. facipraum, P. malariae, S. mansoni, Filatriasis =====Lab abnormalities===== * UA: proteinuria, hematuria, casts * GFR intitially decreased * Streptococcal antibody profile * C3, C4 and/or CH50 depressed in 50% =====Nephrotic Syndrome===== * Causes * Minimal change glomerulopathy * Membranous glomerulopathy: PLA2R, HLA-DQA1 secondary cause in 30%; often self limiting, treat with steroid + Cytoxans * FSGS: 5-12%, more common in AA; normal C4, C3, C2 * Primary * Secondary * Familial/genetic * Viral (HIV/Parvovirus B19) * Drug-induced (heroin, IFN-alpha, lithium, pamidronate) * Mediated by responses * MPGN (also nephritic) * Primary * Type I: mesangiocapillary (more common) * Type II: dense deposit disease (5%) * Type III: mixed pattern * Secondary * IgA (Berger's) * common in Asia (>30%), Europe (20-25%), US (10%) * overproduction of IgA1 * Fibrillary GN * Diabetic glomerulopathy * Amyloidosis * Light chain deposition disease * Approach * History/physical * Lab: CrCl, SPEP/UPEP, lipids, cholesterol, complements, ANA, cryoglobulin, hepatitis serologies * Renal biopsy: when no clear cause evident, or possible cause with atypical course =====Rapidly Progressive Glomerulonephritis===== * Clinical term * Often associated with crescentic * Immunofluorescence pattern: * Antibody mediated * Goodpasture's (anti-GBM with lung involvement) * anti-GBM without lung involvement * Pauci-immune * Microscopic * Wegner's (ANCA, mostly PR3) * * Immune-complex mediated =====HUS/TTP===== * Features * Thrombocytopenia * MAHA * Acute renal failure * Neurological symptoms/signs * Fever * Adult * Idiopathic/ADAMSTS13 deficiency * Hereditary defector of plasma factor H * Drugs =====SLE===== * Classes * I. No abnormalities: no treatment (guided by extrarenal symptoms) * II. Mesangial lupus nephritis: corticosteroids * III. Focal segmental lupus nephritis (<50%): corticosteroids + immunosupressive agents * IV. Diffuse proliferative lupus nephritis (>50%): cyclophosphamide pulses, methylprednisone pulses, oral prednisone, azathioprine * V. Membranous lupus nephritis: corticosteroids with/without immunosuppresive agents * VI. Advanced sclerosing lupus nephritis: dialysis, transplantation