RAPIDLY PROGRESSIVE GN

Defined as a subacute reduction in GFR of >50%, with evidence of a proliferative GN; causes overlap with those of acute GN (Table 145-2). Broadly classified into three major subtypes on the basis of renal biopsy findings and pathophysiology: (1) immune complex–associated, e.g., in systemic lupus erythematosus (SLE); (2) “pauci-immune,” associated with antineutrophil cytoplasmic antibodies (ANCA); and (3) associated with anti–glomerular basement (anti-GBM) antibodies, e.g., in Goodpasture’s syndrome. All three forms will typically have a proliferative, crescentic GN by light microscopy but differ in the results of the immunofluorescence and electron microscopy components of the renal biopsy.
TABLE 145-2: Causes of Rapidly Progressive Glomerulonephritis
  1. Infectious diseases
    1. Poststreptococcal glomerulonephritisa
    2. Infective endocarditis
    3. Occult visceral sepsis
    4. Hepatitis B infection (with vasculitis and/or cryoglobulinemia)
    5. HIV infection
    6. Hepatitis C infection (with cryoglobulinemia, membranoproliferative glomerulonephritis)
  2. Multisystem diseases
    1. Systemic lupus erythematosus
    2. Henoch-Schönlein purpura
    3. Systemic necrotizing vasculitis (including granulomatosis with polyangiitis [Wegener’s])
    4. Goodpasture’s syndrome
    5. Essential mixed (IgG/IgM) cryoglobulinemia
    6. Malignancy
    7. Relapsing polychondritis
    8. Rheumatoid arthritis (with vasculitis)
  3. Drugs
    1. Penicillamine
    2. Hydralazine
    3. Allopurinol (with vasculitis)
    4. Rifampin
    5. Cocaine adulterated with levamisole (ANCA-associated)
  4. Idiopathic or primary glomerular disease
    1. A. Idiopathic crescentic glomerulonephritis
      1. Type I—with linear deposits of Ig (anti-GBM antibody–mediated)
      2. Type II—with granular deposits of Ig (immune complex–mediated)
      3. Type III—with few or no immune deposits of Ig (“pauci-immune”)
      4. Antineutrophil cytoplasmic antibody–induced, forme fruste of vasculitis
      5. Immunotactoid glomerulonephritis
      6. Fibrillary glomerulonephritis
    2. Superimposed on another primary glomerular disease
      1. Mesangiocapillary (membranoproliferative) glomerulonephritis (especially type II)
      2. Membranous glomerulonephritis
      3. Berger’s disease (IgA nephropathy)
aMost common cause.
Abbreviation: GBM, glomerular basement membrane.
Source: Glassock RJ, Brenner BM: Harrison’s Principles of Internal Medicine, 13th ed, 1995.

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