Lupus Nephritis

Aka: Lupus Nephritis, Lupus Glomerulonephritis, Glomerulonephritis due to Systemic Lupus

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II. Epidemiology

  1. Renal disease or Lupus Nephritis is ultimately present in 50 to 60% of SLE cases

III. Grading

  1. Class 1: Minimal Mesangial (earliest and most mild form)
    1. Minimal Proteinuria
    2. Normal Serum Creatinine
    3. Histology with immune complex deposition on immunofluorescence, but otherwise normal glomeruli
  2. Class 2: Mesangial Proliferative
    1. Microscopic Hematuria with or without Proteinuria
    2. Histology with mesangial matrix expansion and immune complex deposition
  3. Class 3: Focal Lupus Nephritis
    1. Microscopic Hematuria
    2. Proteinuria (Nephrotic Syndrome may be present)
    3. Hypertension
    4. eGFR decreased
    5. Histology with Glomerulonephritis (focal, segmental or global) affecting <50% of glomeruli
  4. Class 4: Diffuse Lupus Nephritis (most common)
    1. Includes Class 3 features
    2. Complement C3 low
    3. Anti-Double Stranded DNA Antibody (dsDNA) elevated
    4. Histology with Glomerulonephritis affecting >50% of glomeruli and subendothelial diffuse immune deposits
      1. Diffuse Segmental Subtype (4S): Segmental lesions affecting >50% of glomeruli
      2. Diffuse Global Subtype (4G): Global lesions affecting >50% of glomeruli
      3. Subtypes may be further characterized: Active, Active and chronic, and Inactive with glomerular scar
  5. Class 5: Membranous Nephropathy
    1. Nephrotic Syndrome
    2. Microscopic Hematuria
    3. Hypertension
    4. Serum Creatinine normal
    5. Membranous Nephropathy may be the only SLE manifestation, and may occur combined with Class 3 ot 4
    6. Histology with subepithelial immune deposits (global or segmental)
  6. Class 6: Advanced Sclerosing Lupus
    1. Slowly progressive Chronic Kidney Disease to end-stage Renal Failure
    2. Proteinuria with otherwise negative Urine Microscopic Exam
    3. Histology with global sclerosis affecting >90% of glomeruli

IV. Labs

  1. Screening in Systemic Lupus Erythematosus patients (without diagnosed renal disease)
    1. Screen Urinalysis and Serum Creatinine every 3-6 months
    2. Reflex abnormal findings to Urine Protein to Creatinine Ratio
  2. Renal biopsy indications
    1. Proteinuria >=1 g per 24 hours OR
    2. Hematuria > 0.5 g per 24 hours OR
    3. Cellular Casts

V. Management

  1. General
    1. See Prevention of Kidney Disease Progression
    2. Consult nephrology and Rheumatology
  2. Class 1 and 2
    1. Renin-Angiotensin System agents (e.g. ACE Inhibitors or Angiotensin Receptor Blockers)
  3. Class 3, 4 and 5
    1. Induction Phase
      1. Pulse IV Corticosteroids AND
      2. Cyclophosphamide IV for 6 doses (over 3-6 months) OR Mycophenolate daily for 6 months
        1. Cyclosporine or Tacrolimus may be used as alternative in Class 5 disease
    2. Maintenance Phase
      1. Wean Corticosteroids to lowest effective dose
      2. Longterm Mycophenolate or Aziothioprine
  4. Class 6
    1. Renin-Angiotensin System agents (e.g. ACE Inhibitors or Angiotensin Receptor Blockers)
    2. Prepare for renal replacment therapy (e.g. Hemodialysis, Renal Transplant)

VI. Resources

  1. Lupus Nephritis (StatPearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK499817/

VII. References

  1. Rojas-Rivera (2023) Clin Kidney J 16(9):1384-402 +PMID: 37664575 [PubMed]
  2. Schwartz (2014) Curr Opin Rheumatol 26(5):502-9 +PMID: 25014039 [PubMed]

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Related Studies

Ontology: Lupus Nephritis (C0024143)

Definition (CSP) glomerulonephritis associated with systemic lupus erythematosus; classified into four histologic types: mesangial, focal, diffuse, and membranous.
Definition (MSH) Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Concepts Disease or Syndrome (T047)
MSH D008181
SnomedCT 68815009, 197608009
English Glomerulonephritides, Lupus, Glomerulonephritis, Lupus, Lupus Glomerulonephritides, Lupus Glomerulonephritis, Lupus Nephritides, Lupus Nephritis, Nephritides, Lupus, Nephritis, Lupus, LUPUS NEPHRITIS, SLE glomerulonephritis syndrome (disorder), Systemic lupus erythematosus glomerulonephritis syndrome (disorder), Systemic lupus erythematosus glomerulonephritis syndrome, nephritis due to systemic lupus erythematosis, nephritis due to systemic lupus erythematosis (diagnosis), nephritis due to sle, Nephritis systemic lupus erythematosus, Nephritis SLE, SLE nephritis, Lupus Nephritis [Disease/Finding], lupus glomerulonephritis, nephritis lupus, sle nephritis, lupus nephritis, Nephrotic syndrome in systemic lupus erythematosus, (Nephrotic syndrome in systemic lupus erythematosus) or (lupus nephritis) (disorder), (Nephrotic syndrome in systemic lupus erythematosus) or (lupus nephritis), Lupus nephritis, SLE glomerulonephritis syndrome, Lupus nephritis, NOS, SLE glomerulonephritis syndrome, NOS
Italian Nefrite lupoide, Nefrite da Lupus Eritematoso Sistemico, Nefrite da lupus eritematoso sistemico, Nefrite da lupus erimatoso sistemico, Glomerulonefrite da lupus, Nefrite da lupus
Dutch nefritis systemische lupus erythematodes, nefritis SLE, SLE nefritis, lupus nefritis, Glomerulonefritis, lupus, Lupus erythematosus disseminatus, Lupus glomerulonefritis, Lupusnefritis, Nefritis, lupus-
French Lupus érythémateux systémique avec atteinte rénale, Néphrite liée au LES, Glomérulonéphrite lupique, Néphropathie lupique, Néphrite lupique
German Nephritis, systemischer Lupus erythematosus, Nephritis SLE, SLE-Nephritis, Glomerulonephritis, Lupus-, Lupus-Glomerulonephritis, Lupusnephritis, Nephritis, Lupus-
Portuguese Nefrite LES, Nefrite de lúpus eritematoso sistémico, Nefrite de LES, Nefrite de Lupus, Glomerulonefrite Lúpica, Nefrite Lúpica
Spanish Nefritis en el LED, Nefritis en el lupus eritematoso diseminado, glomerulonefritis asociada con LES, glomerulonefritis asociada con lupus eritematoso sistémico (trastorno), glomerulonefritis asociada con lupus eritematoso sistémico, nefritis lúpica, síndrome de glomerulonefritis lúpica, Nefritis en el lupus, Glomerulonefritis Lúpica, Nefritis Lúpica
Swedish Lupusnefrit
Japanese ﾙｰﾌﾟｽｼﾞﾝｴﾝ, ＳＬＥ腎炎, 全身性エリテマトーデス腎炎, ｾﾞﾝｼﾝｾｲｴﾘﾃﾏﾄｰﾃﾞｽｼﾞﾝｴﾝ, SLEｼﾞﾝｴﾝ, 腎炎-ループス, Lupus腎炎, ループス糸球体腎炎, ループス腎炎, 狼瘡腎炎, 糸球体腎炎-ループス
Czech nefritida při lupus erythematosus, Lupusová nefritida, Nefritida při systémovém lupus erythematodes, lupusová nefritida
Finnish SLE-nefriitti
Russian GLOMERULONEFRIT VOLCHANOCHNYI, LIUPUS-NEFRIT, NEFRIT VOLCHANOCHNYI, VOLCHANOCHNYI GLOMERULONEFRIT, VOLCHANOCHNYI NEFRIT, ВОЛЧАНОЧНЫЙ ГЛОМЕРУЛОНЕФРИТ, ВОЛЧАНОЧНЫЙ НЕФРИТ, ГЛОМЕРУЛОНЕФРИТ ВОЛЧАНОЧНЫЙ, ЛЮПУС-НЕФРИТ, НЕФРИТ ВОЛЧАНОЧНЫЙ
Croatian LUPUSNI NEFRITIS
Polish Zapalenie nerek toczniowe, Zapalenie nerek lupoidalne
Hungarian Nephritis systemás lupus erythematosusban, lupus nephritis, SLE nephritis, SLE-nephritis
Norwegian Lupusnefritt, Lupusglomerulonefritt
Derived from the NIH UMLS (Unified Medical Language System)

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