| 词条 | Lupus erythematosus |
| 释义 |
| name = Lupus erythematosus | image = File:Lupus erythematosus, Atlas der Hautkrankheiten.jpg | caption = | field = Rheumatology | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}{{merge|Lupus|discuss=Talk:Lupus erythematosus#Merger proposal|date=October 2018}} Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues.[1] Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The most common and severe form is systemic lupus erythematosus. Signs and symptomsSymptoms vary from person to person, and may come and go. Almost everyone with lupus has joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees. Other common symptoms include:
PhotosensitivityPhotosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease remain to be defined.[3] Causes of photosensitivity may include:
GeneticsCausesIt is typically believed that Lupus is influenced by multiple genes. Lupus is usually influenced by gene polymorphisms, 30 of which have now been linked with the disorder. Some of these polymorphisms have been linked very tentatively however, as the role that they play or the degree to which they influence the disease is unknown. Other genes that are commonly thought to be associated with Lupus are those in the Human leukocyte antigen (HLA) family, which are largely related to healthy functioning of the immune system. There have been several cases where a single gene influence appears to be present, but this is rare. When a single gene deficiency does cause Lupus, it is usually attributed to the genes C1, C2, or C4. The influence of sex chromosomes and environmental factors are also noteworthy. Usually, these factors contribute to Lupus by compromising the immune system.[4] Several studies also indicate a potential association of Lupus with mutations in DNA repair genes [5] Age differenceLupus can develop in any age but most commonly in ages 15 to 44 with varying results. Typically, the manifestation of the disease tends to be more acute in those affected who are of younger age. Women are more likely to get it than men. Patients with juvenile onset Lupus in particular, are vulnerable to mucocutaneous manifestations of the disease (alopecia, skin rash, and ulceration of the mucus membranes) more so than any other age group. However, patients with late onset Lupus have a much higher mortality rate. Nearly 50% of those with late onset Lupus die of their affliction. Women who are of childbearing age are also particularly at risk.[6] Differences in ethnicitySubstantial data have been found to indicate that certain ethnic populations could be more at risk for Lupus Erythematosus, and have a better or worse prognosis. Asian, African, and Native Americans are more likely to get Lupus than Caucasians. Caucasians seem to generally have a more mild manifestation of the disease. Their survival rates after five years were typically around 94%-96%, while patients of African, and some Asian ethnicities had survival rates closer to 79%-92%. The only documented ethnicity that had a higher survival rate than Caucasians were Koreans, who had survival rates nearer to 98%. Among Caucasians, the most common causes of death were complications involving the cardiovascular system, the respiratory system and problems with malignancies.[7][8] Atherosclerotic cardiovascular disease is more prevalent in African American Lupus patients compared to Caucasians with Lupus.[9] DiagnosisFor the diagnosis of lupus 4 out of 11 signs must be present.[10] Testing may include:
ClassificationLupus erythematosus may manifest as systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. Lupus has four main types:
Of these, systemic lupus erythematosus (also known as SLE) is the most common and serious form. A more thorough categorization of lupus includes the following types:[11][12]
Treatment{{Main article|Systemic lupus erythematosus#Treatment}}Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids). An interesting second line drug is methotrexate in its low-dose schedule.[13] In 2011, the U.S. Food and Drug Administration (FDA) approved the first new drug for lupus in more than 50 years to be used in the US, belimumab.[15] In addition to medicative therapy, due to the psychological and social impacts that Lupus may have on an individual, Cognitive Behavioural Therapy (CBT) has also been demonstrated to be effective in reducing stress, anxiety, and depression in lupus sufferers.[14]Recent studies show promising therapeutic efficacy of N-acetylcysteine[15] and sirolimus which block a pathogenic pathway heralded by the activation of the mechanistic target of rapamycin.[16] People with SLE treated with standard care experience a higher risk of opportunistic infections and death compared with the general population. This risk is higher in men and in African Americans.[17] EpidemiologyWorldwide
United Kingdom
United States
See also
References1. ^{{cite book |author1=Fitzpatrick, Thomas B. |author2=Klauss Wolff |author3=Wolff, Klaus Dieter |author4=Johnson, Richard R. |author5=Suurmond, Dick |author6=Richard Suurmond |title=Fitzpatrick's color atlas and synopsis of clinical dermatology |publisher=McGraw-Hill Medical Pub. Division |location=New York |year=2005 |pages= |isbn=978-0-07-144019-6 |oclc= |doi= }}{{page needed|date=March 2015}} 2. ^{{cite web |url=https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0024510/ |title=Systemic Lupus Erythematosus (Lupus, SLE) |publisher=National Library of Medicine |work=PubMed Health}} 3. ^{{cite journal |doi=10.1111/j.1600-0781.2004.00094.x |title=Photosensitivity in lupus erythematosus |journal=Photodermatology, Photoimmunology and Photomedicine |volume=20 |issue=5 |pages=272–9 |year=2004 |last1=Scheinfeld |first1=Noah |last2=Deleo |first2=Vincent A |pmid=15379880}} 4. ^{{cite journal |doi=10.7326/0003-4819-159-7-201310010-01004 |pmid=24081299 |title=Systemic Lupus Erythematosus |journal=Annals of Internal Medicine |volume=159 |issue=7 |pages=ITC4–1 |year=2013 |last1=Kiriakidou |first1=Marianthi |last2=Cotton |first2=D |last3=Taichman |first3=D |last4=Williams |first4=S |citeseerx=10.1.1.1008.5428 }} 5. ^{{cite journal |vauthors=Meas R, Burak MJ, Sweasy JB |title=DNA repair and systemic lupus erythematosus |journal=DNA Repair (Amst.) |volume=56 |issue= |pages=174–182 |date=August 2017 |pmid=28623091 |pmc=5543809 |doi=10.1016/j.dnarep.2017.06.020 |url=}} 6. ^{{cite journal |doi=10.1177/0961203313513508 |pmid=24297642 |title=Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus |journal=Lupus |volume=23 |issue=3 |pages=327–34 |year=2013 |last1=Feng |first1=X. |last2=Zou |first2=Y. |last3=Pan |first3=W. |last4=Wang |first4=X. |last5=Wu |first5=M. |last6=Zhang |first6=M. |last7=Tao |first7=J. |last8=Zhang |first8=Y. |last9=Tan |first9=K. |last10=Li |first10=J. |last11=Chen |first11=Z. |last12=Ding |first12=X. |last13=Qian |first13=X. |last14=Da |first14=Z. |last15=Wang |first15=M. |last16=Sun |first16=L. }} 7. ^{{cite journal |doi=10.1177/0961203313498796 |pmid=23873432 |title=Survival in systemic lupus erythematosus, 1995–2010. A prospective study in a Danish community |journal=Lupus |volume=22 |issue=11 |pages=1185–91 |year=2013 |last1=Voss |first1=A. |last2=Laustrup |first2=H. |last3=Hjelmborg |first3=J. |last4=Junker |first4=P. }} 8. ^"Lupus Facts, Symptoms, Rash, Effects, Complications, and More." WebMD, n.d. Web. 6 October 2014. http://www.webmd.com/lupus/arthritis-lupus. 9. ^{{cite journal |doi=10.1038/srep20303 |pmid=26842423 |pmc=4740809 |title=The Prevalence of Atherosclerosis in Those with Inflammatory Connective Tissue Disease by Race, Age, and Traditional Risk Factors |journal=Scientific Reports |volume=6 |pages=20303 |year=2016 |last1=Alenghat |first1=Francis J |bibcode=2016NatSR...620303A }} 10. ^1 {{cite web|title=Systemic lupus erythematosus: MedlinePlus Medical Encyclopedia|url=https://medlineplus.gov/ency/article/000435.htm|website=Medline Plus|publisher=US National Library of Medicine|language=en}} 11. ^James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Disease of the Skin: Clinical Dermatology. (10th ed.). Saunders. Chapter 8. {{ISBN|0-7216-2921-0}}.{{page needed|date=March 2015}} 12. ^{{cite book |author1=Rapini, Ronald P. |author2=Bolognia, Jean L. |author3=Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=978-1-4160-2999-1 |oclc= |doi= }}{{page needed|date=March 2015}} 13. ^{{cite journal |vauthors= Böhm I |title=Increased peripheral blood B-cells expressing the CD5 molecules in association tp autoantibodies in patients with lupus erythematosus and evidence to selectively down-modulate them |journal=Biomed Pharmacother |volume=58 |issue=5 |pages=338–43 |year=2004 |pmid=15194170 |doi=10.1016/j.biopha.2004.04.010}} 14. ^{{cite journal |doi=10.1002/art.20533 |pmid=15334437 |title=Effects of a stress-reduction program on psychological function, pain, and physical function of systemic lupus erythematosus patients: A randomized controlled trial |journal=Arthritis Care & Research |volume=51 |issue=4 |pages=625–34 |year=2004 |last1=Greco |first1=Carol M |last2=Rudy |first2=Thomas E |last3=Manzi |first3=Susan }} 15. ^{{cite journal |doi=10.1002/art.34502 |pmid=22549432 |pmc=3411859 |title=N-acetylcysteine reduces disease activity by blocking mammalian target of rapamycin in T cells from systemic lupus erythematosus patients: A randomized, double-blind, placebo-controlled trial |journal=Arthritis & Rheumatism |volume=64 |issue=9 |pages=2937–46 |year=2012 |last1=Lai |first1=Zhi-Wei |last2=Hanczko |first2=Robert |last3=Bonilla |first3=Eduardo |last4=Caza |first4=Tiffany N |last5=Clair |first5=Brandon |last6=Bartos |first6=Adam |last7=Miklossy |first7=Gabriella |last8=Jimah |first8=John |last9=Doherty |first9=Edward |last10=Tily |first10=Hajra |last11=Francis |first11=Lisa |last12=Garcia |first12=Ricardo |last13=Dawood |first13=Maha |last14=Yu |first14=Jianghong |last15=Ramos |first15=Irene |last16=Coman |first16=Ioana |last17=Faraone |first17=Stephen V |last18=Phillips |first18=Paul E |last19=Perl |first19=Andras }} 16. ^{{cite journal |doi=10.1016/S0140-6736(18)30485-9 |pmid=29551338 |pmc=5891154 |title=Sirolimus in patients with clinically active systemic lupus erythematosus resistant to, or intolerant of, conventional medications: A single-arm, open-label, phase 1/2 trial |journal=The Lancet |volume=391 |issue=10126 |pages=1186–1196 |year=2018 |last1=Lai |first1=Zhi-Wei |last2=Kelly |first2=Ryan |last3=Winans |first3=Thomas |last4=Marchena |first4=Ivan |last5=Shadakshari |first5=Ashwini |last6=Yu |first6=Julie |last7=Dawood |first7=Maha |last8=Garcia |first8=Ricardo |last9=Tily |first9=Hajra |last10=Francis |first10=Lisa |last11=Faraone |first11=Stephen V |last12=Phillips |first12=Paul E |last13=Perl |first13=Andras }} 17. ^{{Cite journal|last=Dospinescu|first=Paula|last2=Shamliyan|first2=Tatyana A.|date=2017-07-01|title=Additional Improvements in Clinical Response From Adjuvant Biologic Response Modifiers in Adults With Moderate to Severe Systemic Lupus Erythematosus Despite Immunosuppressive Agents: A Systematic Review and Meta-analysis|url=https://www.clinicaltherapeutics.com/article/S0149-2918(17)30707-5/abstract|journal=Clinical Therapeutics|language=English|volume=39|issue=7|pages=1479–1506.e45|doi=10.1016/j.clinthera.2017.05.359|issn=0149-2918|pmid=28673504}} 18. ^1 2 3 {{cite web|url=http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroomreporters.aspx?articleid%3D247%26zoneid%3D60 |accessdate=August 24, 2013 |deadurl=yes |archiveurl=https://web.archive.org/web/20130629233204/http://www.lupus.org/webmodules/webarticlesnet/templates/new_newsroomreporters.aspx?articleid=247&zoneid=60 |archivedate=June 29, 2013 |title=Media Relations }} 19. ^1 2 {{cite web|title= Lupus | publisher= Arthritis Research UK |url=http://www.arthritisresearchuk.org/arthritis-information/data-and-statistics/lupus.aspx |accessdate=August 24, 2013 |deadurl=yes |archiveurl=https://web.archive.org/web/20140201214415/http://www.arthritisresearchuk.org/arthritis-information/data-and-statistics/lupus.aspx |archivedate=February 1, 2014 }} 20. ^1 2 3 {{cite web|url=https://www.cdc.gov/arthritis/basics/lupus.htm|title=Lupus - Arthritis|publisher=CDC|date=2018-10-18}} External links{{Medical resources| DiseasesDB = | ICD10 = {{ICD10|L|93||l|80}} (ILDS L93.010) | ICD9 = {{ICD9|695.4}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = }}{{Localized connective tissue diseases}}{{Urticaria and erythema}}Lupus erythematodes 2 : Autoimmune diseases|Connective tissue diseases |
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