Lupus nephritis

Lupus nephritis
Other names	SLE nephritis PAS stain.
Specialty	Nephrology
Symptoms	Joint pain or swelling[2]
Causes	Complication of systemic lupus erythematosus.[3]
Diagnostic method	Complement levels, Urinalysis[3]
Treatment	Corticosteroids may be used[3]

Lupus nephritis is an

General symptoms of lupus nephritis include[2]^[5]

Cause

The cause of lupus nephritis, a

The immune system protects the human body from infection, and with immune system problems it cannot distinguish between harmful and healthy substances. Lupus nephritis affects approximately 3 out of 10,000 people.^[3]

Pathophysiology

The

A tubuloreticular inclusion within capillary endothelial cells is also characteristic of lupus nephritis and can be seen under an electron microscope in all stages. It is not diagnostic however, as it exists in other conditions such as HIV infection.^[8]

Classification

The World Health Organization has divided lupus nephritis into five stages based on the biopsy. This classification was defined in 1982 and revised in 1995.^[9][10]

Class IV disease (Diffuse proliferative nephritis) is both the most severe, and the most common subtype. Class VI (advanced sclerosing lupus nephritis) is a final class which is included by most practitioners. It is thought to be due to the chronic interferon exposure.^[11]

Order	Name	Incidence[12]	Light microscopy	Electron microscopy	Clinical findings and other tests	Treatment
Class I	Minimal mesangial glomerulonephritis	5%	Normal appearance	Mesangial deposits are visible under an electron microscope	Kidney failure is very rare in this form.[12] Normal urinalysis.[13]
Class II	Mesangial proliferative glomerulonephritis	20%	Mesangial hypercellularity and matrix expansion.		Microscopic haematuria with or without proteinuria may be seen. Hypertension, nephrotic syndrome, and acute kidney injury are very rare at this stage.[13]	Responds to high doses of corticosteroids
Class III	Focal glomerulonephritis	25%	Sclerotic lesions involving less than 50% of the glomeruli, which can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions.	Subendothelial deposits are noted, and some mesangial changes may be present	C1q. Clinically, haematuria and proteinuria are present, with or without nephrotic syndrome, hypertension, and elevated serum creatinine.[13]	Often successfully responds to high doses of corticosteroids
Class IV	Diffuse proliferative nephritis	40%	More than 50% of glomeruli are involved. Lesions can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions.	Under electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present.	Clinically, haematuria and proteinuria are present, frequently with nephrotic syndrome, hypertension, hypocomplementemia, elevated anti-dsDNA titres and elevated serum creatinine.[13] Kidney failure is common.[12]	Corticosteroids and immunosuppressant drugs
Class V	Membranous glomerulonephritis	10%	Diffuse thickening of the glomerular capillary wall (segmentally or globally), with diffuse membrane thickening, and subepithelial deposits seen under the electron microscope.		Signs of emboli.[13] Kidney failure is uncommon.[12]
Class VI	Advanced sclerosing lupus nephritis.[6]		Global sclerosis involving more than 90% of glomeruli, and represents healing of prior inflammatory injury.		Active glomerulonephritis is not usually present. This stage is characterised by slowly progressive kidney dysfunction, with relatively bland urine sediment.	Response to immunotherapy is usually poor.

Treatment

Drug regimens prescribed for lupus nephritis include

In those who have SLE, concomitant lupus nephritis is associated with a worse overall prognosis.[20] 10-30% of people with lupus nephritis progress to kidney failure requiring dialysis, with the 5 year mortality rate of lupus nephritis being 5-25%.^[20] The proliferative forms of lupus nephritis are associated with a higher risk of progression to end stage kidney disease.^[20] Black and Hispanic people with lupus nephritis are more likely to present with severe disease at initial presentation (with more proteinuria and more extensive histopathologic changes) and progress to end stage kidney disease. This is thought to be due to socioeconomic factors but auto-antibodies strongly associated with lupus nephritis such as anti-Sm, anti-Ro and anti-ribonucleoprotein are also more commonly seen in Black and Hispanic people.^[20] Men with SLE tend to have more aggressive forms of lupus nephritis as well with a higher risk of progression to end stage kidney disease and higher risk of concurrent cardiovascular disease.^[20]

See also

• Glomerulus (kidney)

References

Further reading

• ISBN 9780080474540
  .
• Greenberg, Arthur; Cheung, Alfred K. (2005-01-01). Primer on Kidney Diseases. Elsevier Health Sciences.
  ISBN 978-1416023128
  .
• Castro-Santana, Lesliane E.; Colón, Marilú; Molina, María J.; Rodríguez, Vanessa E.; Mayor, Angel M.; Vilá, Luis M. (2010-01-01). "Efficacy of two cyclophosphamide regimens for the treatment of lupus nephritis in Puerto Ricans: low versus standard dose". Ethnicity & Disease. 20 (1): S1–116–21.
  PMID 20521398
  .
• Appel, Gerald B.; Contreras, Gabriel; Dooley, Mary Anne; Ginzler, Ellen M.; Isenberg, David; Jayne, David; Li, Lei-Shi; Mysler, Eduardo; Sánchez-Guerrero, Jorge (2009-05-01). "Mycophenolate Mofetil versus Cyclophosphamide for Induction Treatment of Lupus Nephritis". Journal of the American Society of Nephrology. 20 (5): 1103–1112.
  PMID 19369404
  .

External links

Scholia has a topic profile for Lupus nephritis.