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| {{Infobox medical condition (new) | | {{Short description|A kidney disorder associated with systemic lupus erythematosus}} |
| | name = Lupus nephritis | |
| | synonyms = '''SLE nephritis'''<ref>{{Cite journal|title = Renal transplantation in lupus nephritis|journal = Lupus|date = 2005-01-01|issn = 0961-2033|pmid = 15732296|pages = 95–98|volume = 14|issue = 1|first = C.|last = Ponticelli|first2 = G.|last2 = Moroni|doi=10.1191/0961203305lu2067oa}}</ref>
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| | image = Diffuse proliferative lupus nephritis - high mag.jpg
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| | caption = [[Micrograph]] of diffuse proliferative lupus nephritis showing increased mesangial matrix and mesangial hypercellularity. [[Kidney biopsy]]. [[PAS stain]].
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| | causes = Complication of systemic lupus erythematosus.<ref name=NIH/>
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| | diagnosis = Complement levels, Urinalysis<ref name=NIH/>
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| | treatment = Corticosteroids may be used<ref name=NIH/>
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| '''Lupus nephritis''' is an [[inflammation]] of the [[kidney]]s caused by [[systemic lupus erythematosus]] (SLE), an [[autoimmune disease]].<ref name="NIH">{{Cite web|title = Lupus nephritis: MedlinePlus Medical Encyclopedia|url = https://www.nlm.nih.gov/medlineplus/ency/article/000481.htm|website = www.nlm.nih.gov|accessdate = 2015-10-31}}</ref> It is a type of [[glomerulonephritis]] in which the [[glomerulus (kidney)|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>{{Cite journal|title = Lupus nephritis: current update|journal = [[Arthritis Research & Therapy]] |date = 2011-01-01|issn = 1478-6354|pmc = 3308062|pmid = 22078716|pages = 240|volume = 13|issue = 5|doi = 10.1186/ar3378|first = Ramesh|last = Saxena|first2 = Tina|last2 = Mahajan|first3 = Chandra|last3 = Mohan}}</ref><ref name=niddk/>
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| ==Classification==
| | '''Lupus nephritis''' is an inflammation of the kidneys caused by [[systemic lupus erythematosus]] (SLE), an [[autoimmune disease]] in which the body's immune system attacks its own tissues. This condition is a serious complication of SLE and can lead to significant kidney damage. |
| '''Class I''' disease ([[minimal mesangial glomerulonephritis]]) in its [[histology]] has a normal appearance under a [[optical microscope|light microscope]], but [[mesangial]] deposits are visible under an [[electron microscope]]. At this stage [[urinalysis]] is normal.<ref name=lewis/> | |
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| '''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">{{Cite book|title = Lupus Nephritis|url = https://books.google.com/books?id=aSPXO6lFfTYC|publisher = OUP Oxford|date = 2010-11-04|pages=174–177|isbn = 9780199568055|language = en|first = Edmund J.|last = Lewis|first2 = Melvin M.|last2 = Schwartz}}</ref>
| | ==Pathophysiology== |
| | Lupus nephritis occurs when [[autoantibodies]] form immune complexes that deposit in the [[glomeruli]], the filtering units of the kidneys. This deposition leads to inflammation and damage to the kidney tissues. The exact mechanism involves a complex interplay of genetic, environmental, and immunological factors that result in the production of [[antinuclear antibodies]] and other autoantibodies. |
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| '''Class III''' disease ([[focal proliferative nephritis|focal glomerulonephritis]]) is indicated by [[sclerosis (medicine)|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]], [[Complement component 3|C3]], and [[C1q]]. Clinically, haematuria and proteinuria are present, with or without nephrotic syndrome, hypertension, and elevated serum creatinine.<ref name=lewis/>
| | ==Classification== |
| | | Lupus nephritis is classified into six classes based on the [[International Society of Nephrology]]/[[Renal Pathology Society]] (ISN/RPS) classification system: |
| [[File:Diffuse proliferative lupus nephritis.jpg|thumb|Diffuse proliferative lupus nephritis as seen in a pathology specimen]]
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| '''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-[[Anti-dsDNA antibodies|dsDNA]] titres and elevated serum creatinine.<ref name=lewis/>
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| '''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/>
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| '''Class VI''', or '''advanced sclerosing lupus nephritis'''.<ref name="emedicine">{{Cite journal|title = Lupus Nephritis: Practice Essentials, Background, Pathophysiology|url = http://emedicine.medscape.com/article/330369-overview#a5|date = 2018-12-23}}</ref> a final class which is included by most practitioners. It is represented by global [[Sclerosis (medicine)|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">{{cite journal |vauthors=Kfoury H |title=Tubulo-reticular inclusions in lupus nephritis: are they relevant? |journal=Saudi Journal of Kidney Diseases and Transplantation |volume=25 |issue=3 |pages=539–43 |year=2014 |pmid=24821149 |doi= 10.4103/1319-2442.132169|url=}}</ref> It is thought to be due to the chronic [[interferon]] exposure.<ref name="pmid22162633">{{cite journal |vauthors=Karageorgas TP, Tseronis DD, Mavragani CP |title=Activation of type I interferon pathway in systemic lupus erythematosus: association with distinct clinical phenotypes |journal=Journal of Biomedicine & Biotechnology |volume=2011 |issue= |pages=1–13 |year=2011 |pmid=22162633 |pmc=3227532 |doi=10.1155/2011/273907 |url=}}</ref>
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| ==Signs and symptoms==
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| General symptoms of lupus nephritis include<ref name="niddk">{{Cite web|title = Lupus Nephritis|url = http://www.niddk.nih.gov/health-information/health-topics/kidney-disease/lupus-nephritis/Pages/index.aspx|website = www.niddk.nih.gov|accessdate = 2015-10-31}}</ref><ref>{{Cite web|title = Lupus Nephritis - National Library of Medicine|url = https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0024511/|website = PubMed Health|accessdate = 2015-11-03|first = National Center for Biotechnology|last = Information|first2 = U. S. National Library of Medicine 8600 Rockville|last2 = Pike|first3 = Bethesda|last3 = MD|first4 = 20894|last4 = Usa}}</ref>
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| {{columns-list|colwidth=30em|
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| *[[Fever]]
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| *[[Edema]]
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| *[[High blood pressure]]
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| *[[Arthralgia|Joint pain]]
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| *[[Muscle pain]]
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| *[[Malar rash]]
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| *Foamy [[urine]]
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| }}
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| ==Cause==
| | * '''Class I: Minimal mesangial lupus nephritis''' - Normal appearance under light microscopy, but immune deposits are present under electron microscopy. |
| The cause of lupus nephritis, a [[Heredity|genetic]] predisposition, plays role in lupus nephritis. Multiple [[genes]], many of which are not yet identified, mediate this genetic predisposition.<ref name="emedicine"/><ref>{{Cite journal|title = Lupus Nephritis: An Overview of Recent Findings|last = Salgado|first = Alberto|date = 2012|journal = Autoimmune Diseases|volume = 2012|pages = 849684|doi = 10.1155/2012/849684|pmid = 22536486|pmc = 3318208}}</ref>
| | * '''Class II: Mesangial proliferative lupus nephritis''' - Mesangial hypercellularity and immune deposits. |
| | * '''Class III: Focal lupus nephritis''' - Involves less than 50% of all glomeruli. |
| | * '''Class IV: Diffuse lupus nephritis''' - Involves more than 50% of glomeruli and is the most severe form. |
| | * '''Class V: Membranous lupus nephritis''' - Characterized by thickening of the glomerular basement membrane. |
| | * '''Class VI: Advanced sclerosing lupus nephritis''' - Represents end-stage renal disease with more than 90% of glomeruli sclerosed. |
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| 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/>
| | ==Symptoms== |
| | | Symptoms of lupus nephritis can vary widely but often include: |
| ==Pathophysiology== | | * [[Hematuria]] (blood in the urine) |
| 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 system|complement]].<ref name=emedicine/>
| | * [[Proteinuria]] (excess protein in the urine) |
| | * [[Hypertension]] (high blood pressure) |
| | * [[Edema]] (swelling, particularly in the legs, ankles, and feet) |
| | * [[Renal insufficiency]] |
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| ==Diagnosis== | | ==Diagnosis== |
| [[File:Membranous nephropathy - mpas - very high mag.jpg|thumb|Membranous nephropathy]]
| | Diagnosis of lupus nephritis typically involves a combination of laboratory tests and a [[kidney biopsy]]. Laboratory tests may include: |
| The diagnosis of lupus nephritis depends on [[blood test]]s, [[urinalysis]], X-rays, [[ultrasound]] scans of the kidneys, and a [[kidney biopsy]]. On urinalysis, a nephritic picture is found and [[urinary cast#Red blood cell casts|red blood cell casts]], [[red blood cell]]s 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">{{cite journal |vauthors=Weening JJ, D'Agati VD, Schwartz MM, etal |title=The classification of glomerulonephritis in systemic lupus erythematosus revisited |journal=J. Am. Soc. Nephrol. |volume=15 |issue=2 |pages=241–50 |date=February 2004 |pmid=14747370 |doi= 10.1097/01.ASN.0000108969.21691.5D|url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=14747370}}</ref><ref>{{Cite web|title = National Guideline Clearinghouse {{!}} American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis.|url = http://www.guideline.gov/content.aspx?id=36900|website = www.guideline.gov|accessdate = 2015-11-01|archive-url = https://web.archive.org/web/20150918234706/http://www.guideline.gov/content.aspx?id=36900|archive-date = 2015-09-18|dead-url = yes|df = }}</ref>
| | * [[Urinalysis]] to detect proteinuria and hematuria |
| | | * Blood tests to assess kidney function, such as [[serum creatinine]] and [[glomerular filtration rate]] (GFR) |
| *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: {{cite book |author1=Elizabeth D Agabegi |author2=Agabegi, Steven S. |title=Step-Up to Medicine (Step-Up Series) |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2008 |pages= |isbn=978-0-7817-7153-5 |oclc= |doi= |accessdate=}}</ref> [[Kidney failure]] is very rare in this form.<ref name=agabegi2nd-table6-4/> | | * [[Antinuclear antibody test]] (ANA) and other specific autoantibody tests |
| *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/>
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| *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/>
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| *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/> | |
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| == Treatment ==
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| [[File:Cyclophosphamide.svg|thumb|100 px|Cyclophosphamide]]
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| Drug regimens prescribed for lupus nephritis include [[mycophenolate mofetil]] (MMF), intravenous [[cyclophosphamide]] with corticosteroids, and the immune suppressant [[azathioprine]] with [[corticosteroid]]s. 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>{{Cite journal | last1 = Henderson | first1 = L. | last2 = Masson | first2 = P. | last3 = Craig | first3 = JC. | last4 = Flanc | first4 = RS. | last5 = Roberts | first5 = MA. | last6 = Strippoli | first6 = GF. | last7 = Webster | first7 = AC. | title = Treatment for lupus nephritis. | journal = Cochrane Database Syst Rev | volume = 12 | issue = | pages = CD002922 | month = | year = 2012 | doi = 10.1002/14651858.CD002922.pub3 | pmid = 23235592 }}</ref><ref>{{Cite journal|url = http://kidneyandtransplant.cochrane.org/sites/kidneyandtransplant.cochrane.org/files/uploads/news/Nephrology%20Vol%2018%20Iss%201%202013%2071-72-LUPUS%20NEPHRITIS.pdf|title = Induction and maintenance treatment of proliferative lupus nephritis|last = Masson|first = Philip|date = 2011|journal = Cochrane Review/Commentary|volume = 18|pages = 71–72|doi = 10.1111/nep.12011|pmid = |access-date =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>{{Cite journal|last=Singh|first=Jasvinder A.|last2=Hossain|first2=Alomgir|last3=Kotb|first3=Ahmed|last4=Wells|first4=George|date=2016-09-13|title=Risk of serious infections with immunosuppressive drugs and glucocorticoids for lupus nephritis: a systematic review and network meta-analysis|journal=BMC Medicine|volume=14|issue=1|pages=137|doi=10.1186/s12916-016-0673-8|issn=1741-7015|pmc=5022202|pmid=27623861}}</ref><ref>{{Cite journal|last=Tang|first=Kuo-Tung|last2=Tseng|first2=Chien-Hua|last3=Hsieh|first3=Tsu-Yi|last4=Chen|first4=Der-Yuan|date=June 2018|title=Induction therapy for membranous lupus nephritis: a systematic review and network meta-analysis|journal=International Journal of Rheumatic Diseases|volume=21|issue=6|pages=1163–1172|doi=10.1111/1756-185X.13321|issn=1756-185X|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/>
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| ==See also==
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| *[[Glomerulus (kidney)]]
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| ==References==
| | A kidney biopsy is often performed to determine the class of lupus nephritis and guide treatment decisions. |
| {{Reflist}}
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| ==Further reading== | | ==Treatment== |
| *{{Cite book|authorlink1=Robert G. Lahita|title = Systemic Lupus Erythematosus|url = https://books.google.com/books?id=FPj1IT9xy2wC|publisher = Academic Press|date = 2004-06-09|isbn = 9780080474540|first = Robert G.|last = Lahita}}
| | Treatment of lupus nephritis aims to reduce inflammation, prevent kidney damage, and preserve kidney function. Common treatments include: |
| *{{Cite book|title = Primer on Kidney Diseases|url = https://books.google.com/?id=BUE9-mY4FkoC&pg=PA236&dq=lupus+nephritis#v=onepage&q=lupus%2520nephritis&f=false|publisher = Elsevier Health Sciences|date = 2005-01-01|isbn = 978-1416023128|language = en|first = Arthur|last = Greenberg|first2 = Alfred K.|last2 = Cheung}} | | * [[Corticosteroids]] to reduce inflammation |
| *{{Cite journal|title = Efficacy of two cyclophosphamide regimens for the treatment of lupus nephritis in Puerto Ricans: low versus standard dose|journal = Ethnicity & Disease|date = 2010-01-01|issn = 1049-510X|pmc = 3572835|pmid = 20521398|pages = S1–116–21|volume = 20|issue = 1|first = Lesliane E.|last = Castro-Santana|first2 = Marilú|last2 = Colón|first3 = María J.|last3 = Molina|first4 = Vanessa E.|last4 = Rodríguez|first5 = Angel M.|last5 = Mayor|first6 = Luis M.|last6 = Vilá}} | | * [[Immunosuppressive drugs]] such as [[cyclophosphamide]], [[mycophenolate mofetil]], and [[azathioprine]] |
| *{{Cite journal|title = Mycophenolate Mofetil versus Cyclophosphamide for Induction Treatment of Lupus Nephritis|journal = Journal of the American Society of Nephrology|date = 2009-05-01|issn = 1046-6673|pmc = 2678035|pmid = 19369404|pages = 1103–1112|volume = 20|issue = 5|doi = 10.1681/ASN.2008101028|first = Gerald B.|last = Appel|first2 = Gabriel|last2 = Contreras|first3 = Mary Anne|last3 = Dooley|first4 = Ellen M.|last4 = Ginzler|first5 = David|last5 = Isenberg|first6 = David|last6 = Jayne|first7 = Lei-Shi|last7 = Li|first8 = Eduardo|last8 = Mysler|first9 = Jorge|last9 = Sánchez-Guerrero}} | | * [[Biologic agents]] like [[rituximab]] or [[belimumab]] |
| | * [[Antihypertensive medications]] to control blood pressure |
| | * [[Angiotensin-converting enzyme inhibitors]] (ACE inhibitors) or [[angiotensin receptor blockers]] (ARBs) to reduce proteinuria |
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| == External links == | | ==Prognosis== |
| {{Medical resources
| | The prognosis of lupus nephritis varies depending on the class and severity of the disease, as well as the response to treatment. Early diagnosis and aggressive treatment can improve outcomes and reduce the risk of progression to [[end-stage renal disease]]. |
| | DiseasesDB =
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| | ICD10 = {{ICD10|M|32|1|m|32}}+{{ICD10|N|08|5|n|00}}*
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| | ICD9 = {{ICD9|583.81}}
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| | ICDO =
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| | OMIM =
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| | MedlinePlus = 000481
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| | eMedicineSubj = med
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| | eMedicineTopic = 1597
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| | MeshID = D008181
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| }}
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| {{Lupus nephritis}}
| | ==Related pages== |
| {{Nephrology}}
| | * [[Systemic lupus erythematosus]] |
| | * [[Autoimmune disease]] |
| | * [[Kidney disease]] |
| | * [[Glomerulonephritis]] |
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| [[Category:Rheumatology]]
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| [[Category:Kidney diseases]] | | [[Category:Kidney diseases]] |
| {{stub}}
| | [[Category:Autoimmune diseases]] |
| | [[Category:Systemic lupus erythematosus]] |
A kidney disorder associated with systemic lupus erythematosus
Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE), an autoimmune disease in which the body's immune system attacks its own tissues. This condition is a serious complication of SLE and can lead to significant kidney damage.
Pathophysiology
Lupus nephritis occurs when autoantibodies form immune complexes that deposit in the glomeruli, the filtering units of the kidneys. This deposition leads to inflammation and damage to the kidney tissues. The exact mechanism involves a complex interplay of genetic, environmental, and immunological factors that result in the production of antinuclear antibodies and other autoantibodies.
Classification
Lupus nephritis is classified into six classes based on the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification system:
- Class I: Minimal mesangial lupus nephritis - Normal appearance under light microscopy, but immune deposits are present under electron microscopy.
- Class II: Mesangial proliferative lupus nephritis - Mesangial hypercellularity and immune deposits.
- Class III: Focal lupus nephritis - Involves less than 50% of all glomeruli.
- Class IV: Diffuse lupus nephritis - Involves more than 50% of glomeruli and is the most severe form.
- Class V: Membranous lupus nephritis - Characterized by thickening of the glomerular basement membrane.
- Class VI: Advanced sclerosing lupus nephritis - Represents end-stage renal disease with more than 90% of glomeruli sclerosed.
Symptoms
Symptoms of lupus nephritis can vary widely but often include:
Diagnosis
Diagnosis of lupus nephritis typically involves a combination of laboratory tests and a kidney biopsy. Laboratory tests may include:
A kidney biopsy is often performed to determine the class of lupus nephritis and guide treatment decisions.
Treatment
Treatment of lupus nephritis aims to reduce inflammation, prevent kidney damage, and preserve kidney function. Common treatments include:
Prognosis
The prognosis of lupus nephritis varies depending on the class and severity of the disease, as well as the response to treatment. Early diagnosis and aggressive treatment can improve outcomes and reduce the risk of progression to end-stage renal disease.
Related pages
