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Lupus Nephritis

Lupus Nephritis is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • The renal manifestation of systemic lupus erythematosus (SLE)
  • American College of Rheumatology (ACR) criteria: persistent proteinuria >500 mg/day or ≥3 on dipstick and/or presence of cellular casts; alternatively, spot urine protein-to-creatinine ratio >0.5 and “active urinary sediment” (>5 RBC/hpf, >5 WBC/hpf in absence of infection, or cellular casts—RBC or WBC casts) (1)
  • Clinical manifestations primarily due to immune complex–mediated glomerular disease. Tubulointerstitial and vascular involvement often coexist. Diagnosis is based on clinical findings, urine abnormalities, autoantibodies, and renal biopsy.
  • Treatment and prognosis depend on International Society of Nephrology/Renal Pathology Society (ISN/RPS) histologic class—risk of end-stage renal disease (ESRD) highest in class IV.
  • Delay in diagnosis/treatment increases risk of ESRD.

Epidemiology

  • Peak incidence of SLE is 15 to 45 years of age.
  • Predominant sex: female > male (10:1)
  • Once SLE develops, lupus nephritis (LN) affects both genders equally; it is more severe in children and men and less severe in older adults.

Incidence
  • SLE: 1.4 to 22/100,000
  • Up to 60% of SLE patients develop LN over time; 25–50% of SLE patients have nephritis as the initial presentation.

Pediatric Considerations
LN is more common and more severe in children: 60–80% of children have LN at or soon after SLE onset.

Prevalence
SLE: 7.4 to 159.4/100,000

Etiology and Pathophysiology

  • Immune complex–mediated inflammation injures glomeruli, tubules, interstitium, and vasculature.
  • Glomeruli: Varying degrees of mesangial proliferation, crescent formation, and fibrinoid necrosis cause reduced glomerular filtration rate (GFR).
  • Persistent inflammation (chronicity) leads to sclerosis and glomerular loss.
  • Tubulointerstitial injury (edema, inflammatory cell infiltrate acutely; tubular atrophy in chronic phase) with or without tubular basement membrane immune complex deposition leads to reduced renal function.
  • Vascular lesions: immune complex deposition and noninflammatory necrosis in arterioles
  • SLE is a multifactorial disease, with multigenic inheritance; exact etiology remains unclear.
  • Defective T-cell autoregulation and polyclonal B-cell hyperactivity contribute to dysregulated apoptosis. Impaired clearance of apoptotic cells inhibits self-tolerance to nuclear antigen.
  • Anti-DNA, anti-C1q, anti-α-actin, and other nuclear component autoantibodies develop.
  • Deposition of circulating immune complexes or autoantibodies attaching to local nuclear antigens leads to complement activation, inflammation, and tissue injury.
  • Interaction of genetic, hormonal, and environmental factors leads to great variability in LN severity.

Genetics
Multigenic inheritance; clustering in families, ~25% concordance in identical twins

Risk Factors

Younger age, African American or Hispanic race, more ACR criteria for SLE, longer disease duration, hypertension, lower socioeconomic status, family history of SLE, anti-dsDNA antibodies

Commonly Associated Conditions

Skin, hematologic, cerebral, pulmonary, GI, and cardiopulmonary systems are often involved in SLE.

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Citation

Stephens, Mark B., et al., editors. "Lupus Nephritis." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117507/all/Lupus_Nephritis.
Lupus Nephritis. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117507/all/Lupus_Nephritis. Accessed April 24, 2019.
Lupus Nephritis. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117507/all/Lupus_Nephritis
Lupus Nephritis [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117507/all/Lupus_Nephritis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Lupus Nephritis ID - 117507 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117507/all/Lupus_Nephritis PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -