Rapidly progressive glomerulonephritis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

RPGN is classified on the basis of the cause of crescent formation resulting from glomerular injury ..

Type I

• Type I RPGN is characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM).
• Type I also known as anti-GBM glomerulonephritis.
• The antibodies formed are known as anticollagen antibodies and react against type IV collagen of GBM.
• The antibodies can be produced by a stimulus such as viral URTI that exposes alveolar collagen membrane or it can be idiopathic.
• The antibodies formed can act against alveolar membrane and lungs get involved in some cases such as in goodpasture syndrome.

Type II

• Type II RPGN is caused by the deposition of immune complexes in the GBM.
• Immune complexes can be formed in certain infections or in connective tissue disorders.
• These immune complexes deposit over the GBM and activate the complement system resulting in crescent formation.
• Examples include:
• Postinfectious (staphylococci/streptococci)
• Connective tissue disorders
• Lupus nephritis
• Henoch-Schönlein purpural)
• Immunoglobulin A nephropathy
• Mixed cryoglobulinemia
• Membranoproliferative glomerulonephritis

Type III

• Type III RPGN is also known as pauci immune RPGN.
• There are no anti GBM antibodies or no immune complexes involved.
• It is further classified into 2 types:
  • Immunogenic - ANCA positive
  • Non immunogenic- ANCA negative/ Idiopathic
• ANCAs cause the release of lytic enzymes from neutrophils that damage the GBM.
• Systemic vasculitis is present in most of the cases but some occur without systemic involvement and only renal findings maybe present.
• Examples include
  • Granulomatosis with polyangiitis (Wegener granulomatosis)
  • Microscopic polyangiitis (MPA)
  • Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
  • Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome)
  • Drugs- hydralazine, allopurinol and rifampin.