Pathology of lupus glomerulonephritis

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Pathology of lupus glomerulonephritis

  1. 1. The primary clinical purposes for pathologic classification systemare to:• Facilitate communication: Between pathologists Between pathologists and clilinicalans Between clinicians In understanding the literature• Facilitate clinical management: Guiding treatment Suggesting prognosis Indicating an etiology or pathogenic mechanism
  2. 2. • Mesangial lupus nephritis• Focal proliferative lupus nephritis• Diffuse proliferative lupus nephritis• Membranous lupus nephritis*Mesangial lupus nephritis was add in a 1977 publication (Am J Med 62:12-30, 1977) to the other three categories that were published in 1970 (Ann Intern Med 73:929, 1970)
  3. 3. “Original WHO Classification” Buffalo, NY, 1974; or Geneva, 1975 “Modified WHO Classification ”ISKDC, Paris, 1980 (Churg and Sobin, 1982) “Modified WHO Classification ” Jacob churg 1995
  4. 4. • Class I. normal normal by LM, IM and IF• Class II. Messangial changes A .minimal alterations (normal by LM, mesangial deposits by IF and EM) B. mesangial glomerulitis (mesangial hypercellularity)• Class III. Focal and segmental proliferative glomerulonephritis (<50% glomeruli involved)• Class IV. Diffuse proliferative glomerulonephritis (<50% glomeruli involved)• Class V. membranous glomerulonephritis Pure Mixed patterns (such as Class III associated with Class V)
  5. 5. • I. normal a. Nil (by all techniques) b. normal by LM but deposits by EM or IF• II. Pure mesangial alterations (mesangiopathy) a. mesangial widening mild hypercellularty b. moderate hypercellularity• III. Focal segmental glomerulonephritis a. active necrotizing lesions b. active and sclerosing lesions c. sclerosing lesions• IV. Diffuse glomerulonephritis a. without segmental lesions b.c. and d. like a.b and c. above• V. diffuse membranous glomerulonephritis a. pure b. associated with category II c. associated with actegory III d. associated with category IV• VI. Advanced sclerosing glomerulonephritis
  6. 6. • I. Normal a. nil( by all techniques) b. normal by LM but deposits by EM or IF• II Pure Mesangial Alteration (Mesangiopathy) a. mesanglal widening mild hypercellularity b. moderate hypercellularity• III Focal Segmental Glomerulonephritis a. active necrotizing lesion b. active and sclerosing lesions c. sclerosing lesions• IV. Diffuse Glomerulonephritis a. without segment lesions b. c. and d, like A, B, and C above• V. Diffuse Membranous Glomerulonephritis a. pure b. associted with category II• VI. Advanced Sclerosing Glomerulonephritis
  7. 7. Majority approach Minority approach• Class I: no lesion by LM, EM, • IA. No lesion by LM, EM or IM IM • IB. no lesion by LM but mesangial deposits by EM and/or IM • IIA. mild mesangial• Class IIA. no lesion by LM, but hypercellularty mesangial deposits by IM • IIB. moderate mesangial and/or EM hypercellularity• IIB. mesangial hypercellularty
  8. 8. Edward R. Murrow
  9. 9. Majority apporach Minority approachClass III :focal GN affecting <50% Segmental GN affecting of glomeruli <50% or >50% of glomeruliClass IV: diffuse GN affeceting > Diffuse non-seegmental GN 50% of glomeruli Affecting <50 % of glomeruliClass V: membranous GN Membranous GN A. no hypercellularity • A. no hypercellularity B. plus class II • B. plus class II • C. plus class III • D. plus class IVMixed class III or IV plus V
  10. 10. Proposal of the international societyof nephrology and renalpathological society working groupon the classification of lupusglomerulonephritis
  11. 11. • Class I. Minimal mesangial lupus glomerulonephritis (LGN)• Class II. Mesangial proliferative LGN• Class III. Focal LGN ( involving < 50 % of glomeruli)• Class IV.Diffuse LGN ( involving 50% or > glomeruli)• Class V. Membranous LGN• Class VI.Advanced sclerotic LGN ( >90 % sclerotic glomeruli )*for classes III and IV, the diagnosis should include one of the following: with active lesions/with active and chronic lesions/Inactive with scars*for classes III and IV, the diagnosis should include the percentage of glomeruli with fibrinoid necrosis and/or cellular crescents when present*for class IV, the diagnosis should include one of the following: predominantly segmental (IV- S)/predominantly global (IV-G)*class V may occur in combination with III or IV in which case both will be diagnosed
  12. 12. Class I. Minimal mesangial lupus glomerulonephritisNormal glomeruli by LM but mnesangeial immune deposits byIF and/ or EM.
  13. 13. Class II. Mesangial proliferative LGNPurely mesangial hyhpercellularity of any degree or mesangial matrex expansion by LM with immune deposits, predominatly mesangial with none or few, isolated subepithelial and/or subendothelial deposits by IF and/or EM not visible by LM.
  14. 14. Class III. Focal lupus glomerulonephritis Active or inactive focal , segmental and /or global endo- and /or extracapiliary GN, typically with focal, subendothelial immune extracapillary GN, with or without focal or diffuse mesangial alterations. III (A)Active focal proliferative LGN III (A/C) Active and sclerotic focal proliferative LGN III (C)Inactive sclerotic focal LGN*Indicate the proportion of glomeruli with active and with sclerotic lesions*Indicate the proportion of glomeruli with fibrinoid necrosis and/or cellular crescents
  15. 15. Class IV. Diffuse segmental (IV-S) or global (IV-G) LGN Active or inactive diffuse (50% or more involved glomeruli), segmental or global endo- or extracapiliary GN with diffuse subendothelial immune deposits, with or without mesangial alterations. This class is divided into diffuse segmental (IV-S) when >50% of the involved glomeruli have segmental lesions, and diffuse global (IV-G) when >50% of the involved glomeruli have ealobal lesions. IV (A) Active diffuse segmental or global proliferative LGN IV (A/C) Diffuse segmental or global proliferative & sclerotic LGN IV (C) Diffuse segmental or global sclerotic LGN*Indicate the proportion of glomeruli with active and with sclerotic lesions*Indicate the proportion of glomeruli with fibrinoid necrosis and/or cresents
  16. 16. Class V. membranous LGNNumerous global or segmental subepithelial immunedeposits or their morphologic sequelae by LM and/or IFand/or EM with or without mesangial alterations.May occur in combination with III or IV in which case bothwill be diagnosed
  17. 17. Class VI. Advanced sclerotic lupus glomerulonephritis90 % or > glomeruli globally sclerosed without residual activity
  18. 18. • Class I Minimal mesangial lupus glomerulonephritis (LGN)• Class II Mesangial proliferative LGN• Class III Focal LGN (Involving < 50% of glomeruli)• Class IV Diffuse LGN (Involving >= 50% gloeruli, IV-S and IV-G)• Class V Membranous LGN• Class VI Advanced sclerotic LGN (> 90% sclerotic glomeruli)

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