Kidney nontumor / medical renal

Glomerular disease

SLE and related autoantibody mediated glomerulonephritis

Systemic lupus erythematosus

Last author update: 3 March 2014
Last staff update: 7 June 2022

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PubMed Search: Systemic lupus erythematosis [title]

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Systemic lupus erythematosus. website. Accessed February 3rd, 2023.
Definition / general
  • Autoimmune disease of women (90%) with various clinical manifestations
  • 40 - 80% have impaired renal function, due to in situ or circulating immune complexes
  • Most cases idiopathic, but drug induced lupus-like disease is associated with chlorpromazine, hydralazine, isoniazid, methyldopa, procainamide, quinidine
Clinical features
  • Adults and children (2/3 present initially at ages 16 - 30 years)
  • In children in US, prevance of 10 per 100K Medicaid-enrolled children (Arthritis Rheum 2012;64:2669)
  • Most common renal symptoms are proteinuria and microscopic hematuria; also acute nephritis, acute and chronic renal failure, isolated abnormalities in urinary sediment, nephrotic syndrome
  • Biopsy is necessary to determine subsequent clinical course and to assist with treatment
  • Non-lupus nephritides occasionally present (Hum Pathol 2001;32:1125), including IgA nephropathy (Mod Pathol 1995;8:5)
WHO classification of lupus nephritis
  • I: normal or minimal abnormality; no abnormalities identified by H&E, immunofluorescence or EM; asymptomatic; may not actually represent renal disease
  • II (10 - 20% of cases): pure mesangial lesions; mesangial expansion but mostly patent capillaries; mesangial immune deposits; mild proteinuria with variable hematuria, but normal renal function
  • IIA: minimal light microscopy changes, but immune deposits present in mesangium by immunofluorescence and EM
  • IB: glomerular mesangial hypercellularity present by light microscopy, involving center of lobules away from vascular pole; immune deposits only in mesangial region; no significant tubular, interstitial or vascular changes; good prognosis (5 year renal survival > 90%)
  • III (30 - 40% of cases): focal proliferative glomerulonephritis; focal and segmental proliferative intra- or extracapillary necrotizing or sclerosing lesions in < 50% of glomeruli; predominantly mesangial and subendothelial immune deposits (Ig, complement); nephritic urinary sediment; variable proteinuria but usually non-nephrotic
  • IV (40 - 60%): diffuse proliferative glomerulonephritis; predominantly global proliferative lesions, necrosis, crescents in > 50% of glomeruli, variable sclerosis, prominent inflammatory interstitial infiltrate, often wire loop lesions in thickened capillary walls; predominantly mesangial and subendothelial immune deposits of IgG, often IgM and IgA (all three is called a “full house”); also C3 and C1q, fibrin and fibrinogen; nephritic and nephrotic syndromes, hypertension, variable renal insufficiency; rapidly progresses to renal failure without treatment
  • V (10 - 15%): membranous glomerulonephritis; diffuse thickening of capillary walls, subepithelial and mesangial immune deposits (“spike and dome” pattern with silver stain); nephrotic syndrome or severe proteinuria
  • VI: advanced sclerosing glomerulonephritis; glomerular obsolescence and segmental glomerulosclerosis, tubular atrophy, interstitial fibrosis; few immune deposits; chronic renal failure that is unlikely to respond to therapy
  • Overlap common in classes III and V, IV and V


International Society of Nephrology (ISN) / Renal Pathology Society (RPS) classification of Lupus Nephritis (2004)
    Class I: Minimal Mesangial Lupus Nephritis:
  • Glomeruli normal by light microscopy (LM), mesangial deposits by immunofluorescence (IF) / electron microscopy (EM)

    Class II: Mesangial Proliferative Lupus Nephritis:
  • Mesangial hypercelullularity and/or mesangial expansion by LM, immune deposits by IF / EM

    Class III: Focal Lupus Nephritis:
  • Active or inactive focal, segmental or global endo or extracapillary glomerulonephritis involving < 50% of glomeruli, immune deposits are subendothelial
  • Subclassified as A (active lesions), A/C (active and chronic lesions), C (chronic lesions)

    Class IV: Diffuse Lupus Nephritis:
  • > 50% glomeruli with active / inactive diffuse, segmental, or global endo and/or extracapillary glomerulonephritis
  • Diffuse subendothelial deposits can cause wire-loop appearance
  • A (active lesions), A/C (active and chronic lesions), C (chronic lesions)
  • Diffuse segmental if > 50% glomeruli show segmental lesions, diffuse global if > 50% show global lesions

    Class V: Membranous Lupus Nephritis:
  • Global or segmental involvement, subepithelial immune deposits
  • May occur in combination with class III or IV

    Class VI: Advanced Sclerosing Lupus Nephritis:
  • > 90% globally sclerosed glomeruli

  • References: J Am Soc Nephrol 2004;15:241


  • Tubulointerstitial disease: observed in all classes, severe in classes III and IV; usually composed of lymphocytes, plasma cells and macrophages; severe cases have casts
  • Vascular lesions: common, associated with poor prognosis; includes vascular immune deposits, non-inflammatory necrotizing vasculopathy, true vasculitis, thrombotic microangiopathy and non-specific arteriolosclerosis
  • Hematoxylin bodies: focal areas of necrosis containing fragmented nuclei; specific for SLE, but only 1 - 2% sensitive
  • Alveolar hemorrhage (bland alveolar wall changes) is similar to lupus microangiopathy of kidney; both are caused by immune complex deposition and induction of apoptosis (Arch Pathol Lab Med 2001;125:475)
  • Activity index: based on scoring (0 - 3) of (a) glomerular endocapillary proliferation, (b) glomerular neutrophilic infiltration, (c) wire-loop deposits and hyaline thrombi, (d) glomerular fibrinoid necrosis and karyorrhexis, (e) cellular crescents and (f) interstitial inflammation; double the fibrinoid necrosis / karyorrhexis (part d) and cellular crescent (part e) scores; add up (maximum 24)
  • Chronicity index: based on scoring (0 - 3) of (a) glomerular sclerosis, (b) fibrous crescents, (c) tubular atrophy and (d) interstitial fibrosis; add up (maximum 12)
Case reports
Gross images

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Diffuse proliferative lupus nephritis

Microscopic (histologic) images

Contributed by Abdullah Al-Fiaar, M.D.
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Membranous glomerulonephritis

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Membranous lupus nephritis, class II

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Membranous lupus nephritis, class IV

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Membranous lupus nephritis, classes II to IV

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Membranous lupus nephritiss, class IV to VI

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Platelet microthrombi,
macrophagic infiltration,
activated complement
Electron microscopy description
  • Granular glomerular electron dense deposits; may be organized into curvilinear microtubular or fibrillar structures composed of bands 10 - 15 nm in diameter "fingerprinting"
Differential diagnosis
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