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shared:renal:glomerular_disease

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
shared/renal/glomerular_disease.txt · Last modified: 2020/02/25 17:34 by 127.0.0.1