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Lupus nephritis

MedGen UID:
6147
Concept ID:
C0024143
Disease or Syndrome
Synonyms: Glomerulonephritides, Lupus; Glomerulonephritis, Lupus; Lupus Glomerulonephritides; Lupus Glomerulonephritis; Lupus Nephritides; Lupus Nephritis; Nephritides, Lupus; Nephritis, Lupus
SNOMED CT: SLE glomerulonephritis syndrome (68815009); Lupus nephritis (68815009); Systemic lupus erythematosus glomerulonephritis syndrome (68815009)
 
HPO: HP:0033726
Monarch Initiative: MONDO:0005556

Definition

Lupus nephritis is a type of glomerulonephritis that constitutes one of the most severe organ manifestations of systemic lupus erythematosus. Lupus nephritis is subclassified in six distinct classes, that represent different manifestations and severities of renal involvement and guide the therapeutic management. [from HPO]

Conditions with this feature

Systemic lupus erythematosus
MedGen UID:
6146
Concept ID:
C0024141
Disease or Syndrome
Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by production of autoantibodies against nuclear, cytoplasmic, and cell surface molecules that transcend organ-specific boundaries. Tissue deposition of antibodies or immune complexes induces inflammation and subsequent injury of multiple organs and finally results in clinical manifestations of SLE, including glomerulonephritis, dermatitis, thrombosis, vasculitis, seizures, and arthritis. Evidence strongly suggests the involvement of genetic components in SLE susceptibility (summary by Oishi et al., 2008). Genetic Heterogeneity of Systemic Lupus Erythematosus An autosomal recessive form of systemic lupus erythematosus (SLEB16; 614420) is caused by mutation in the DNASE1L3 gene (602244) on chromosome 3p14.3. An X-linked dominant form of SLE (SLEB17; 301080) is caused by heterozygous mutation in the TLR7 gene (300365) on chromosome Xp22. See MAPPING and MOLECULAR GENETICS sections for a discussion of genetic heterogeneity of susceptibility to SLE.
Autosomal systemic lupus erythematosus type 16
MedGen UID:
482372
Concept ID:
C3280742
Disease or Syndrome
People with SLE have episodes in which the condition gets worse (exacerbations) and other times when it gets better (remissions). Overall, SLE gradually gets worse over time, and damage to the major organs of the body can be life-threatening.\n\nAbout a third of people with SLE develop kidney disease (nephritis). Heart problems may also occur in SLE, including inflammation of the sac-like membrane around the heart (pericarditis) and abnormalities of the heart valves, which control blood flow in the heart. Heart disease caused by fatty buildup in the blood vessels (atherosclerosis), which is very common in the general population, is even more common in people with SLE. The inflammation characteristic of SLE can also damage the nervous system, and may result in abnormal sensation and weakness in the limbs (peripheral neuropathy); seizures; stroke; and difficulty processing, learning, and remembering information (cognitive impairment). Anxiety and depression are also common in SLE.\n\nSLE may first appear as extreme tiredness (fatigue), a vague feeling of discomfort or illness (malaise), fever, loss of appetite, and weight loss. Most affected individuals also have joint pain, typically affecting the same joints on both sides of the body, and muscle pain and weakness. Skin problems are common in SLE. A characteristic feature is a flat red rash across the cheeks and bridge of the nose, called a "butterfly rash" because of its shape. The rash, which generally does not hurt or itch, often appears or becomes more pronounced when exposed to sunlight. Other skin problems that may occur in SLE include calcium deposits under the skin (calcinosis), damaged blood vessels (vasculitis) in the skin, and tiny red spots called petechiae. Petechiae are caused by a shortage of cells involved in clotting (platelets), which leads to bleeding under the skin. Affected individuals may also have hair loss (alopecia) and open sores (ulcerations) in the moist lining (mucosae) of the mouth, nose, or, less commonly, the genitals.\n\nSystemic lupus erythematosus (SLE) is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body. The signs and symptoms of SLE vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous system, and blood-forming (hematopoietic) system. SLE is one of a large group of conditions called autoimmune disorders that occur when the immune system attacks the body's own tissues and organs.

Professional guidelines

PubMed

Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC, Boletis J, Bruce IN, Cervera R, Doria A, Dörner T, Furie RA, Gladman DD, Houssiau FA, Inês LS, Jayne D, Kouloumas M, Kovács L, Mok CC, Morand EF, Moroni G, Mosca M, Mucke J, Mukhtyar CB, Nagy G, Navarra S, Parodis I, Pego-Reigosa JM, Petri M, Pons-Estel BA, Schneider M, Smolen JS, Svenungsson E, Tanaka Y, Tektonidou MG, Teng YO, Tincani A, Vital EM, van Vollenhoven RF, Wincup C, Bertsias G, Boumpas DT
Ann Rheum Dis 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. PMID: 37827694
Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219
Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN, Cervera R, Doria A, Gordon C, Govoni M, Houssiau F, Jayne D, Kouloumas M, Kuhn A, Larsen JL, Lerstrøm K, Moroni G, Mosca M, Schneider M, Smolen JS, Svenungsson E, Tesar V, Tincani A, Troldborg A, van Vollenhoven R, Wenzel J, Bertsias G, Boumpas DT
Ann Rheum Dis 2019 Jun;78(6):736-745. Epub 2019 Mar 29 doi: 10.1136/annrheumdis-2019-215089. PMID: 30926722

Recent clinical studies

Etiology

Alforaih N, Whittall-Garcia L, Touma Z
J Appl Lab Med 2022 Oct 29;7(6):1450-1467. doi: 10.1093/jalm/jfac036. PMID: 35932197
Rovin BH, Furie R, Teng YKO, Contreras G, Malvar A, Yu X, Ji B, Green Y, Gonzalez-Rivera T, Bass D, Gilbride J, Tang CH, Roth DA
Kidney Int 2022 Feb;101(2):403-413. Epub 2021 Sep 22 doi: 10.1016/j.kint.2021.08.027. PMID: 34560137
Gasparotto M, Gatto M, Binda V, Doria A, Moroni G
Rheumatology (Oxford) 2020 Dec 5;59(Suppl5):v39-v51. doi: 10.1093/rheumatology/keaa381. PMID: 33280015Free PMC Article
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Almaani S, Meara A, Rovin BH
Clin J Am Soc Nephrol 2017 May 8;12(5):825-835. Epub 2016 Nov 7 doi: 10.2215/CJN.05780616. PMID: 27821390Free PMC Article

Diagnosis

Yu C, Li P, Dang X, Zhang X, Mao Y, Chen X
J Autoimmun 2022 Oct;132:102871. Epub 2022 Aug 20 doi: 10.1016/j.jaut.2022.102871. PMID: 35999111
Mejia-Vilet JM, Malvar A, Arazi A, Rovin BH
Kidney Int 2022 Feb;101(2):242-255. Epub 2021 Oct 4 doi: 10.1016/j.kint.2021.09.012. PMID: 34619230
Gasparotto M, Gatto M, Binda V, Doria A, Moroni G
Rheumatology (Oxford) 2020 Dec 5;59(Suppl5):v39-v51. doi: 10.1093/rheumatology/keaa381. PMID: 33280015Free PMC Article
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Almaani S, Meara A, Rovin BH
Clin J Am Soc Nephrol 2017 May 8;12(5):825-835. Epub 2016 Nov 7 doi: 10.2215/CJN.05780616. PMID: 27821390Free PMC Article

Therapy

Furie RA, Aroca G, Cascino MD, Garg JP, Rovin BH, Alvarez A, Fragoso-Loyo H, Zuta-Santillan E, Schindler T, Brunetta P, Looney CM, Hassan I, Malvar A
Ann Rheum Dis 2022 Jan;81(1):100-107. Epub 2021 Oct 6 doi: 10.1136/annrheumdis-2021-220920. PMID: 34615636Free PMC Article
Rovin BH, Furie R, Teng YKO, Contreras G, Malvar A, Yu X, Ji B, Green Y, Gonzalez-Rivera T, Bass D, Gilbride J, Tang CH, Roth DA
Kidney Int 2022 Feb;101(2):403-413. Epub 2021 Sep 22 doi: 10.1016/j.kint.2021.08.027. PMID: 34560137
Rovin BH, Teng YKO, Ginzler EM, Arriens C, Caster DJ, Romero-Diaz J, Gibson K, Kaplan J, Lisk L, Navarra S, Parikh SV, Randhawa S, Solomons N, Huizinga RB
Lancet 2021 May 29;397(10289):2070-2080. Epub 2021 May 7 doi: 10.1016/S0140-6736(21)00578-X. PMID: 33971155
Furie R, Rovin BH, Houssiau F, Malvar A, Teng YKO, Contreras G, Amoura Z, Yu X, Mok CC, Santiago MB, Saxena A, Green Y, Ji B, Kleoudis C, Burriss SW, Barnett C, Roth DA
N Engl J Med 2020 Sep 17;383(12):1117-1128. doi: 10.1056/NEJMoa2001180. PMID: 32937045
Houssiau FA, Vasconcelos C, D'Cruz D, Sebastiani GD, Garrido Ed Ede R, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R
Arthritis Rheum 2002 Aug;46(8):2121-31. doi: 10.1002/art.10461. PMID: 12209517

Prognosis

Mahajan A, Amelio J, Gairy K, Kaur G, Levy RA, Roth D, Bass D
Lupus 2020 Aug;29(9):1011-1020. Epub 2020 Jun 22 doi: 10.1177/0961203320932219. PMID: 32571142Free PMC Article
Ocampo-Piraquive V, Nieto-Aristizábal I, Cañas CA, Tobón GJ
Expert Rev Clin Immunol 2018 Dec;14(12):1043-1053. Epub 2018 Oct 24 doi: 10.1080/1744666X.2018.1538789. PMID: 30338717
Iwamoto T, Niewold TB
Clin Immunol 2017 Dec;185:32-39. Epub 2016 Sep 28 doi: 10.1016/j.clim.2016.09.012. PMID: 27693588Free PMC Article
Hanly JG, O'Keeffe AG, Su L, Urowitz MB, Romero-Diaz J, Gordon C, Bae SC, Bernatsky S, Clarke AE, Wallace DJ, Merrill JT, Isenberg DA, Rahman A, Ginzler EM, Fortin P, Gladman DD, Sanchez-Guerrero J, Petri M, Bruce IN, Dooley MA, Ramsey-Goldman R, Aranow C, Alarcón GS, Fessler BJ, Steinsson K, Nived O, Sturfelt GK, Manzi S, Khamashta MA, van Vollenhoven RF, Zoma AA, Ramos-Casals M, Ruiz-Irastorza G, Lim SS, Stoll T, Inanc M, Kalunian KC, Kamen DL, Maddison P, Peschken CA, Jacobsen S, Askanase A, Theriault C, Thompson K, Farewell V
Rheumatology (Oxford) 2016 Feb;55(2):252-62. Epub 2015 Sep 5 doi: 10.1093/rheumatology/kev311. PMID: 26342222Free PMC Article
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

Clinical prediction guides

Krassanairawiwong K, Charoenpitakchai M, Supasyndh O, Satirapoj B
Int Urol Nephrol 2021 Jul;53(7):1391-1398. Epub 2021 Mar 8 doi: 10.1007/s11255-020-02732-3. PMID: 33682052
Mahajan A, Amelio J, Gairy K, Kaur G, Levy RA, Roth D, Bass D
Lupus 2020 Aug;29(9):1011-1020. Epub 2020 Jun 22 doi: 10.1177/0961203320932219. PMID: 32571142Free PMC Article
Bajema IM, Wilhelmus S, Alpers CE, Bruijn JA, Colvin RB, Cook HT, D'Agati VD, Ferrario F, Haas M, Jennette JC, Joh K, Nast CC, Noël LH, Rijnink EC, Roberts ISD, Seshan SV, Sethi S, Fogo AB
Kidney Int 2018 Apr;93(4):789-796. Epub 2018 Feb 16 doi: 10.1016/j.kint.2017.11.023. PMID: 29459092
Almaani S, Meara A, Rovin BH
Clin J Am Soc Nephrol 2017 May 8;12(5):825-835. Epub 2016 Nov 7 doi: 10.2215/CJN.05780616. PMID: 27821390Free PMC Article
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

Recent systematic reviews

Steiger S, Ehreiser L, Anders J, Anders HJ
Front Immunol 2022;13:999704. Epub 2022 Sep 23 doi: 10.3389/fimmu.2022.999704. PMID: 36211360Free PMC Article
Kostopoulou M, Fanouriakis A, Cheema K, Boletis J, Bertsias G, Jayne D, Boumpas DT
RMD Open 2020 Jul;6(2) doi: 10.1136/rmdopen-2020-001263. PMID: 32699043Free PMC Article
Tunnicliffe DJ, Palmer SC, Henderson L, Masson P, Craig JC, Tong A, Singh-Grewal D, Flanc RS, Roberts MA, Webster AC, Strippoli GF
Cochrane Database Syst Rev 2018 Jun 29;6(6):CD002922. doi: 10.1002/14651858.CD002922.pub4. PMID: 29957821Free PMC Article
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500
Smyth A, Oliveira GH, Lahr BD, Bailey KR, Norby SM, Garovic VD
Clin J Am Soc Nephrol 2010 Nov;5(11):2060-8. Epub 2010 Aug 5 doi: 10.2215/CJN.00240110. PMID: 20688887Free PMC Article

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