Lupus nephritis

Lupus nephritis
Synonyms	SLE nephritis<ref>Ponticelli, C., Renal transplantation in lupus nephritis, Lupus, Vol. 14(Issue: 1), pp. 95–98, DOI: 10.1191/0961203305lu2067oa, PMID: 15732296,</ref>
Pronounce
Field
Symptoms
Complications
Onset
Duration
Types
Causes	Complication of systemic lupus erythematosus.<ref name=NIH/>
Risks
Diagnosis	Complement levels, Urinalysis<ref name=NIH/>
Differential diagnosis
Prevention
Treatment	Corticosteroids may be used<ref name=NIH/>
Medication
Prognosis
Frequency
Deaths

Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE), an autoimmune disease.<ref name="NIH">

Lupus nephritis: MedlinePlus Medical Encyclopedia(link). www.nlm.nih.gov.

</ref> It is a type of glomerulonephritis in which the glomeruli become inflamed. As the result of SLE, the cause of glomerulonephritis is said to be secondary and has a different pattern and outcome from conditions with a primary cause originating in the kidney.<ref>Saxena, Ramesh,

 Lupus nephritis: current update, 
 Arthritis Research & Therapy, 
 
 Vol. 13(Issue: 5),
 pp. 240,
 DOI: 10.1186/ar3378,
 PMID: 22078716,
 PMC: 3308062,</ref><ref name=niddk/>

Classification

Class I disease (minimal mesangial glomerulonephritis) in its histology has a normal appearance under a light microscope, but mesangial deposits are visible under an electron microscope. At this stage urinalysis is normal.<ref name=lewis/>

Class II disease (mesangial proliferative glomerulonephritis) is noted by 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.<ref name="lewis">Edmund J.,

 Lupus Nephritis. online version, 
  
 OUP Oxford, 
  
  
  
 ISBN 9780199568055, 
  
  
  
 Pages: 174–177,</ref>

Class III disease (focal glomerulonephritis) is indicated by 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. Under the electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present. Immunofluorescence reveals positively for IgG, IgA, IgM, C3, and C1q. Clinically, haematuria and proteinuria are present, with or without nephrotic syndrome, hypertension, and elevated serum creatinine.<ref name=lewis/>

Diffuse proliferative lupus nephritis as seen in a pathology specimen

Class IV disease (diffuse proliferative nephritis) is both the most severe, and the most common subtype. 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.<ref name=lewis/>

Class V disease (membranous glomerulonephritis) is characterized by diffuse thickening of the glomerular capillary wall (segmentally or globally), with diffuse membrane thickening, and subepithelial deposits seen under the electron microscope. Clinically, stage V presents with signs of nephrotic syndrome. Microscopic haematuria and hypertension may also been seen. Stage V can also lead to thrombotic complications such as renal vein thromboses or pulmonary emboli.<ref name=lewis/>

Class VI, or advanced sclerosing lupus nephritis.<ref name="emedicine">,

 Lupus Nephritis: Practice Essentials, Background, Pathophysiology, 
 , 
 
 
 
 
 
 
 
 Full text,</ref> a final class which is included by most practitioners. It is represented by 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. 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.<ref name="pmid24821149">, 
 Tubulo-reticular inclusions in lupus nephritis: are they relevant?, 
 Saudi Journal of Kidney Diseases and Transplantation, 
 2014,
 Vol. 25(Issue: 3),
 pp. 539–43,
 DOI: 10.4103/1319-2442.132169,
 PMID: 24821149,</ref> It is thought to be due to the chronic interferon exposure.<ref name="pmid22162633">, 
 Activation of type I interferon pathway in systemic lupus erythematosus: association with distinct clinical phenotypes, 
 Journal of Biomedicine & Biotechnology, 
 2011,
 Vol. 2011,
 pp. 1–13,
 DOI: 10.1155/2011/273907,
 PMID: 22162633,
 PMC: 3227532,</ref>

Signs and symptoms

General symptoms of lupus nephritis include<ref name="niddk">

Lupus Nephritis(link). www.niddk.nih.gov.

</ref><ref> Information, National Center for Biotechnology. Lupus Nephritis - National Library of Medicine(link). PubMed Health.

</ref>

Cause

The cause of lupus nephritis, a genetic predisposition, plays role in lupus nephritis. Multiple genes, many of which are not yet identified, mediate this genetic predisposition.<ref name="emedicine"/><ref>Salgado, Alberto,

 Lupus Nephritis: An Overview of Recent Findings, 
 Autoimmune Diseases, 
 
 Vol. 2012,
 pp. 849684,
 DOI: 10.1155/2012/849684,
 PMID: 22536486,
 PMC: 3318208,</ref>

The immune system protects the human body from infection, with immune system problems it cannot distinguish between harmful and healthy substances. Lupus nephritis affects approximately 3 out of 10,000 people.<ref name=NIH/>

Pathophysiology

The pathophysiology of lupus nephritis has autoimmunity contributing significantly. Autoantibodies direct themselves against nuclear elements. The characteristics of nephritogenic autoantibodies (lupus nephritis) are antigen specificity directed at nucleosome, high affinity autoantibodies form intravascular immune complexes, and autoantibodies of certain isotypes activate complement.<ref name=emedicine/>

Diagnosis

Membranous nephropathy

The diagnosis of lupus nephritis depends on blood tests, urinalysis, X-rays, ultrasound scans of the kidneys, and a kidney biopsy. On urinalysis, a nephritic picture is found and red blood cell casts, red blood cells and proteinuria is found. 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.<ref name="pmid14747370">,

 The classification of glomerulonephritis in systemic lupus erythematosus revisited, 
 J. Am. Soc. Nephrol., 
 
 Vol. 15(Issue: 2),
 pp. 241–50,
 DOI: 10.1097/01.ASN.0000108969.21691.5D,
 PMID: 14747370,
 
 
 Full text,</ref><ref>

National Guideline Clearinghouse | American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis.(link). www.guideline.gov.

</ref>

• Class I is minimal mesangial glomerulonephritis which is histologically normal on light microscopy but with mesangial deposits on electron microscopy. It constitutes about 5% of cases of lupus nephritis.<ref name=agabegi2nd-table6-4>Table 6-4 in: ,

 Step-Up to Medicine (Step-Up Series), 
  
 Hagerstwon, MD:Lippincott Williams & Wilkins, 
 2008, 
  
  
 ISBN 978-0-7817-7153-5,</ref> Kidney failure is very rare in this form.<ref name=agabegi2nd-table6-4/>

• Class II is based on a finding of mesangial proliferative lupus nephritis. This form typically responds completely to treatment with corticosteroids. It constitutes about 20% of cases.<ref name=agabegi2nd-table6-4/> Kidney failure is rare in this form.<ref name=agabegi2nd-table6-4/>
• Class III is focal proliferative nephritis and often successfully responds to treatment with high doses of corticosteroids. It constitutes about 25% of cases.<ref name=agabegi2nd-table6-4/> Kidney failure is uncommon in this form.<ref name=agabegi2nd-table6-4/>
• Class IV is diffuse proliferative nephritis. This form is mainly treated with corticosteroids and immunosuppressant drugs. It constitutes about 40% of cases.<ref name=agabegi2nd-table6-4/> Kidney failure is common in this form.<ref name=agabegi2nd-table6-4/>
• Class V is membranous nephritis and is characterized by extreme edema and protein loss. It constitutes about 10% of cases.<ref name=agabegi2nd-table6-4/> Kidney failure is uncommon in this form.<ref name=agabegi2nd-table6-4/>

Treatment

Cyclophosphamide

Drug regimens prescribed for lupus nephritis include mycophenolate mofetil (MMF), intravenous cyclophosphamide with corticosteroids, and the immune suppressant azathioprine with corticosteroids. MMF and cyclophosphamide with corticosteroids are equally effective in achieving remission of the disease. MMF is safer than cyclophosphamide with corticosteroids, with less chance of causing ovarian failure, immune problems or hair loss. It also works better than azathioprine with corticosteroids for maintenance therapy.<ref>,

 Treatment for lupus nephritis., 
 Cochrane Database Syst Rev, 
 2012,
 Vol. 12,
 pp. CD002922,
 DOI: 10.1002/14651858.CD002922.pub3,
 PMID: 23235592,</ref><ref>Masson, Philip, 
 Induction and maintenance treatment of proliferative lupus nephritis, 
 Cochrane Review/Commentary, 
 
 Vol. 18,
 pp. 71–72,
 DOI: 10.1111/nep.12011,
 
 
 
 Full text,
 Accessed on: 4 November 2015.</ref>  A 2016 network meta-analysis, which included 32 RCTs of lupus nephritis, demonstrated that tacrolimus and MMF followed by azathioprine maintenance were associated with a lower risk of serious infection when compared to other immunosuppressants or glucocorticoids.<ref>Singh, Jasvinder A., 
 Risk of serious infections with immunosuppressive drugs and glucocorticoids for lupus nephritis: a systematic review and network meta-analysis, 
 BMC Medicine, 
 
 Vol. 14(Issue: 1),
 pp. 137,
 DOI: 10.1186/s12916-016-0673-8,
 PMID: 27623861,
 PMC: 5022202,</ref><ref>Tang, Kuo-Tung, 
 Induction therapy for membranous lupus nephritis: a systematic review and network meta-analysis, 
 International Journal of Rheumatic Diseases, 
 
 Vol. 21(Issue: 6),
 pp. 1163–1172,
 DOI: 10.1111/1756-185X.13321,
 PMID: 29879319,</ref> Individuals with lupus nephritis have a high risk for B-cell lymphoma (which begins in the immune system cells).<ref name=niddk/>

See also

• Glomerulus (kidney)

References

Further reading

• Robert G.,

 Systemic Lupus Erythematosus. online version, 
  
 Academic Press, 
  
  
  
 ISBN 9780080474540,

• Arthur,

 Primer on Kidney Diseases. online version, 
  
 Elsevier Health Sciences, 
  
  
  
 ISBN 978-1416023128,

• Castro-Santana, Lesliane E.,

 Efficacy of two cyclophosphamide regimens for the treatment of lupus nephritis in Puerto Ricans: low versus standard dose, 
 Ethnicity & Disease, 
 
 Vol. 20(Issue: 1),
 pp. S1–116–21,
 
 PMID: 20521398,
 PMC: 3572835,

• Appel, Gerald B.,

 Mycophenolate Mofetil versus Cyclophosphamide for Induction Treatment of Lupus Nephritis, 
 Journal of the American Society of Nephrology, 
 
 Vol. 20(Issue: 5),
 pp. 1103–1112,
 DOI: 10.1681/ASN.2008101028,
 PMID: 19369404,
 PMC: 2678035,

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