Systemic Lupus Erythematosis (SLE)

on 12.12.08 with 0 comments

Know the pathology, clinical manifestations, classification, and prognosis of lupus nephritis

  1. Class I – normal kidney
  2. Class II –Minimal/Mesangial Lupus Nephritis
    • Earliest and mildest form of renal involvement
    • Characterized by mesangial deposits of Ig and C3
    • Proteinuria and hematuria present
    • Nephrotic syndrome and renal insufficiency are very uncommon
    • EM – IC deposits in mesangium
    • IF – appears similar to IgA
  3. Class IIA – mesangial proliferative change
  4. Class IIB – no proliferative change
  5. Class III – Focal proliferative Lupus Nephritis

    • Proliferative changes present in <50%>
    • ALL glomeruli have immune deposits of IgG, IgA, C3, and often IgM and fibrin-related Ags – seen on IF
    • Mainly mesangial deposits with occasional subendothelial deposits (seen on EM – most reliable predictor of progression)
    • All patients show proteinuria, but nephrotic syndrome and renal insufficiency are rare
    • Hypocomplementemia more severe
    • Long-term prognosis is good, but there is a high incidence of transformation to class IV
  6. Class IV – Diffuse Proliferative Lupus Nephritis

    • Most common and most severe form
    • Proliferation seen in >50% of glomeruli, with crescent formation and necrosis common
    • Extensive mesangial and subendothelial deposits
    • Nephrotic range proteinuria
    • Renal function decreased in 75% of patients at presentation
    • Hypocomplementemia, high levels of anti-DNA Ab, and circulating Igs present in most patients
    • Long-term survival rate is ~75% past five years
    • Best prognosis is with those who show remission of nephrotic syndrome and normalization of serologic parameters achieved w/in 1 year of starting therapy
  7. Class V

    • Occurs in ~15% of SLE patients – hard to distinguish from idiopathic MN on LM
    • IF – all sorts of Ig – contrast to idiopathic MN
    • EM – other deposit sites in addition to those normally associated w/ MN
    • May have undetectable levels of anti-DNA Ab at presentation
    • Long-term prognosis about the same as with II (good)

Category: Pathology Notes



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