| 词条 | Dermatomyositis | ||||||||||
| 释义 |
| name = Dermatomyositis | image = Dermatomyositis.jpg | caption = Discrete red areas overlying the knuckles in a person with juvenile dermatomyositis. These are known as Gottron's papules. | field = Rheumatology | symptoms = Rash, muscle weakness, weight loss, fever[1] | complications = Calcinosis, lung inflammation, heart disease[1][3] | onset = 40s to 50s[4] | duration = Long term[1] | types = | causes = Unknown[1] | risks = | diagnosis = Based on symptoms, blood tests, electromyography, muscle biopsies[4] | differential = Polymyositis, inclusion body myositis, scleroderma[4] | prevention = | treatment = Medication, physical therapy, exercise, heat therapy, orthotics, assistive devices, rest[1] | medication = Corticosteroids, methotrexate, azathioprine[1] | prognosis = | frequency = ~ 1 per 100,000 people per year[4] | deaths = }}Dermatomyositis (DM) is a long-term inflammatory disorder which affects muscles.[1] Its symptoms are generally a skin rash and worsening muscle weakness over time.[1] These may occur suddenly or develop over months.[1] Other symptoms may include weight loss, fever, lung inflammation, or light sensitivity.[1] Complications may include calcium deposits in muscles or skin.[1] The cause is unknown.[1] Theories include that it is an autoimmune disease or a result of a viral infection.[1] It is a type of inflammatory myopathy.[1] Diagnosis is typically based on some combination of symptoms, blood tests, electromyography, and muscle biopsies.[2] While there is no cure for the condition, treatments generally improve symptoms.[1] Treatments may include medication, physical therapy, exercise, heat therapy, orthotics, and assistive devices, and rest.[1] Medications in the corticosteroids family are typically used with other agents like methotrexate or azathioprine recommended if steroids are not working well.[1] Intravenous immunoglobulin may also improve outcomes.[1] Most people improve with treatment and in some the condition resolves completely.[1] About 1 per 100,000 people per year are newly affected.[2] The condition usually occurs in those in their 40s and 50s with women being affected more often than men.[2] People of any age, however, may be affected.[2] The condition was first described in the 1800s.[3] Signs and symptomsThe main symptoms include several kinds of skin rash along with muscle weakness in both upper arms or thighs.[4] SkinOne form the rashes take is called "heliotrope" (a purplish color) or lilac, but may also be red. It can occur around the eyes along with swelling, but also occurs on the upper chest or back what is called the "shawl" (around the neck) or "V-sign" above the breasts and may also occur on the face, upper arms, thighs, or hands.[5] Another form the rash takes is called Gottron's sign which are red or violet, sometimes scaly, slightly raised papules that erupt on any of the finger joints (the metacarpophalangeal joints or the interphalangeal joints).[5][33] Gottron's papules may also be found over other bony prominences including the elbows, knees, or feet. All these rashes are made worse by exposure to sunlight, and are often very itchy, painful, and may bleed.[33] If a person exhibits only skin findings characteristic of DM, without weakness or abnormal muscle enzymes, then he or she may be experiencing amyopathic dermatomyositis (ADM), formerly known as "dermatomyositis sine myositis".[35] MusclesPeople with DM experience progressively worsening muscle weakness in the proximal muscles (for example, the shoulders and thighs).[6] Tasks that use these muscles: standing from sitting, lifting, and climbing stairs, can become increasingly difficult for people with dermatomyositis.[6] OtherAround 30% of people have swollen, painful joints, but this is generally mild.[38] In some people the condition affects the lungs, and they may have a cough or difficulty breathing. If the condition affects the heart, there may be arrhythmias. If it affects the blood vessels in the stomach or intestines, which is more common in juvenile DM, the person might vomit blood, have black tarry bowel movements, or may develop a hole somewhere in their GI tract.[38] Causes{{See also|List of human leukocyte antigen alleles associated with cutaneous conditions}}The cause is unknown, but it may result from an initial viral infection or cancer, either of which could raise an autoimmune response.[38] Between 7 and 30% of dermatomyositis arise from cancer, probably as an autoimmune response.[41] The most common associated cancers are ovarian cancer, breast cancer, and lung cancer.[7] 18 to 25% of people with amyopathic DM also have cancer.[33] Malignancy in association with dermatomyositis is more prevalent after age 60. Some cases are inherited, and HLA subtypes HLA-DR3, HLA-DR52, and HLA-DR6 seem to create a disposition to autoimmune dermatomyositis.[38] DiagnosisThe diagnosis of dermatomyositis is based on five criteria which are also used to differentially diagnose with respect to polymyositis:[35]
The fifth criterion is what differentiates dermatomyositis from polymyositis; the diagnosis is considered definite for dermatomyositis if three of items 1 through 4 are present in addition to 5, probable with any two in addition to 5, and possible if just one is present in addition to 5.[35] Dermatomyositis is associated with autoantibodies, especially antinuclear antibodies (ANA).[38] Around 80% of people with DM test positive for ANA and around 30% of people have myositis-specific autoantibodies which include antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including antibodies against histidine—tRNA ligase (also called Jo-1); antibodies to signal recognition particle (SRP); and anti-Mi-2 antibodies.[38] Magnetic resonance imaging may be useful to guide muscle biopsy and to investigate involvement of internal organs;[8] X-ray may be used to investigate joint involvement and calcifications.[9]A given case of dermatomyositis may be classified as amyopathic dermatomyositis if only skin is affected and there is no muscle weakness for longer than 6 months according to one 2016 review,[10] or two years according to another.[33] ClassificationDermatomyositis is a form of systemic connective tissue disorder, a class of diseases that often involve autoimmune dysfunction.[11][12] It has also been classified as an idiopathic inflammatory myopathy along with polymyositis, necrotizing autoimmune myositis, cancer-associated myositis, and sporadic inclusion body myositis.[13] There is a form of this disorder that strikes children, known as juvenile dermatomyositis (JDM).[14] TreatmentThere is no cure for dermatomyositis, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously. Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone. Periodic treatment using intravenous immunoglobulin can also improve recovery. Other immunosuppressive agents used to treat the inflammation associated with dermatomyositis include cyclosporine A, cyclophosphamide, and tacrolimus. Physical therapy is usually recommended to prevent muscle atrophy and to regain muscle strength and range of motion. Many individuals with dermatomyositis may need a topical ointment, such as topical corticosteroids, for their skin disorder. They should wear a high-protection sunscreen and protective clothing. Surgery may be required to remove calcium deposits that cause nerve pain and recurrent infections.[57] Antimalarial medications, especially hydroxychloroquine and chloroquine, are used to treat the rashes, as they are in similar conditions.[33]Rituximab is used when people don't respond to other treatments.[15][16]As of 2016, treatments for amyopathic dermatomyositis in adults did not have a strong evidence base; published treatments included antimalarial medications, steroids, taken or orally or applied to the skin, calcineurin inhibitors applied to the skin, dapsone, Intravenous immunoglobulin (IVIG), methotrexate, azathioprine, and mycophenolate mofetil. None appear to be very effective but among them, IVIG has had the best outcomes.[10] PrognosisBefore the advent of modern treatments such as prednisone, intravenous immunoglobulin, plasmapheresis, chemotherapies, and other drugs, the prognosis was poor.[17] The cutaneous manifestations of dermatomyositis may or may not improve with therapy in parallel with the improvement of the myositis. In some people, the weakness and rash resolve together. In others, the two are not linked, with one or the other being more challenging to control. Often, cutaneous disease persists after adequate control of the muscle disease.{{mcn|date=December 2016}} The risk of death from the condition is much higher if the heart or lungs are affected.[13][18] EpidemiologyIncidence of DM peaks at ages 40–50, but the disease can affect people of all ages.[19][2] It tends to affect more women than men.[2] The prevalence of DM ranges from 1 to 22 per 100,000 people.[20][21][22] HistoryThe diagnostic criteria were proposed in 1975 and became widely adopted.[23][24] Amyopathic DM, also called DM sine myositis, was named in 2002.[23] Society and culture
ResearchAs of 2016, research was ongoing into causes for DM, as well as biomarkers;[30] clinical trials were ongoing for use of the following drugs in DM: ajulemic acid (Phase II), adrenocorticotropic hormone gel (Phase IV, open label), IMO-8400, an antagonist of Toll-like receptor 7,8 and 9 (Ph II), abatacept (Phase IV, open label), and sodium thiosulfate (Phase II).[23] References1. ^1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 {{cite web|title=Dermatomyositis|url=https://rarediseases.info.nih.gov/diseases/6263/dermatomyositis|website=GARD|accessdate=13 July 2017|language=en|date=2017|deadurl=no|archiveurl=https://web.archive.org/web/20170705003213/https://rarediseases.info.nih.gov/diseases/6263/dermatomyositis|archivedate=5 July 2017|df=dmy-all}} {{HHS content|url=http://www.ninds.nih.gov/disorders/dermatomyositis/dermatomyositis.htm|title = NINDS Dermatomyositis Information Page|accessdate=2016-12-12}}2. ^1 2 3 4 5 6 7 8 9 {{cite web|title=Dermatomyositis|url=https://rarediseases.org/rare-diseases/dermatomyositis/|website=NORD (National Organization for Rare Disorders)|accessdate=13 July 2017|date=2015|deadurl=no|archiveurl=https://web.archive.org/web/20170219083233/https://rarediseases.org/rare-diseases/dermatomyositis/|archivedate=19 February 2017|df=dmy-all}} 3. ^{{cite book|last1=Dourmishev|first1=Lyubomir A.|last2=Dourmishev|first2=Assen Lyubenov|title=Dermatomyositis: Advances in Recognition, Understanding and Management|date=2009|publisher=Springer Science & Business Media|isbn=9783540793137|page=5|url=https://books.google.com/books?id=8Wz4PoRqPhgC&pg=PA5|language=en|deadurl=no|archiveurl=https://web.archive.org/web/20170908192411/https://books.google.com/books?id=8Wz4PoRqPhgC&pg=PA5|archivedate=8 September 2017|df=dmy-all}} 4. ^1 {{Cite journal|last=Callen|first=Jeffrey P.|last2=Wortmann|first2=Robert L.|date=2006-09-01|title=Dermatomyositis|journal=Clinics in Dermatology|volume=24|issue=5|pages=363–373|doi=10.1016/j.clindermatol.2006.07.001|issn=0738-081X|pmid=16966018}} 5. ^1 {{Cite journal|last=Callen|first=Jeffrey P.|date=2010-06-01|title=Cutaneous manifestations of dermatomyositis and their management|journal=Current Rheumatology Reports|volume=12|issue=3|pages=192–197|doi=10.1007/s11926-010-0100-7|issn=1534-6307|pmid=20425525}} 6. ^1 {{Cite journal|last=Dalakas|first=Marinos C.|date=2015-04-30|title=Inflammatory Muscle Diseases|journal=New England Journal of Medicine|volume=372|issue=18|pages=1734–1747|doi=10.1056/NEJMra1402225|issn=0028-4793|pmid=25923553|url=http://jdc.jefferson.edu/neurologyfp/89}} 7. ^1 {{cite journal|last1=Di Rollo|first1=D|last2=Abeni|first2=D|last3=Tracanna|first3=M|last4=Capo|first4=A|last5=Amerio|first5=P|title=Cancer risk in dermatomyositis: a systematic review of the literature.|journal=Giornale Italiano di Dermatologia e Venereologia : Organo Ufficiale, Societa Italiana di Dermatologia e Sifilografia|date=October 2014|volume=149|issue=5|pages=525–37|pmid=24975953}} 8. ^{{cite journal|last1=Simon|first1=JP|last2=Marie|first2=I|last3=Jouen|first3=F|last4=Boyer|first4=O|last5=Martinet|first5=J|title=Autoimmune Myopathies: Where Do We Stand?|journal=Frontiers in Immunology|date=14 June 2016|volume=7|pages=234|pmid=27379096|pmc=4905946|doi=10.3389/fimmu.2016.00234}} 9. ^{{cite journal|last1=Ramos-E-Silva|first1=M|last2=Lima Pinto|first2=AP|last3=Pirmez|first3=R|last4=Cuzzi|first4=T|last5=Carneiro|first5=S|title=Dermatomyositis-Part 2: Diagnosis, Association With Malignancy, and Treatment.|journal=Skinmed|date=1 October 2016|volume=14|issue=5|pages=354–358|pmid=27871347}} 10. ^1 2 3 4 {{cite journal|last1=Callander|first1=J|last2=Robson|first2=Y|last3=Ingram|first3=J|last4=Piguet|first4=V|title=Treatment of clinically amyopathic dermatomyositis in adults: a systematic review.|journal=The British Journal of Dermatology|date=11 May 2016|doi=10.1111/bjd.14726|pmid=27167896}} 11. ^1 2 3 4 5 6 {{cite web|last1=Hajj-ali|first1=Rula A.|title=Polymyositis and Dermatomyositis - Musculoskeletal and Connective Tissue Disorders|url=http://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/autoimmune-rheumatic-disorders/polymyositis-and-dermatomyositis|publisher=Merck Manuals Professional Edition|date=June 2013|deadurl=no|archiveurl=https://web.archive.org/web/20151012015400/http://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/autoimmune-rheumatic-disorders/polymyositis-and-dermatomyositis|archivedate=12 October 2015|df=dmy-all}} 12. ^{{cite web|title=ICD-10 Systemic connective tissue disorders (M30-M36)|url=http://apps.who.int/classifications/icd10/browse/2016/en#/M30-M36|publisher=WHO|accessdate=9 December 2016|deadurl=no|archiveurl=https://web.archive.org/web/20170208033519/http://apps.who.int/classifications/icd10/browse/2016/en#/M30-M36|archivedate=8 February 2017|df=dmy-all}} 13. ^1 {{cite journal|last1=Danieli|first1=MG|last2=Gelardi|first2=C|last3=Guerra|first3=F|last4=Cardinaletti|first4=P|last5=Pedini|first5=V|last6=Gabrielli|first6=A|title=Cardiac involvement in polymyositis and dermatomyositis.|journal=Autoimmunity Reviews|date=May 2016|volume=15|issue=5|pages=462–5|doi=10.1016/j.autrev.2016.01.015|pmid=26826433}} 14. ^{{cite journal |vauthors=Feldman BM, Rider LG, Reed AM, Pachman LM |title=Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood |journal=Lancet |volume=371 |issue=9631 |pages=2201–2212 |date=June 2008 |pmid=18586175 |doi=10.1016/S0140-6736(08)60955-1 |url=http://linkinghub.elsevier.com/retrieve/pii/S0140-6736(08)60955-1}} 15. ^{{cite journal|last1=Malik|first1=A|last2=Hayat|first2=G|last3=Kalia|first3=JS|last4=Guzman|first4=MA|title=Idiopathic Inflammatory Myopathies: Clinical Approach and Management.|journal=Frontiers in Neurology|date=20 May 2016|volume=7|pages=64|pmid=27242652|pmc=4873503|doi=10.3389/fneur.2016.00064}} 16. ^{{cite journal|last1=Wright|first1=NA|last2=Vleugels|first2=RA|last3=Callen|first3=JP|title=Cutaneous dermatomyositis in the era of biologicals.|journal=Seminars in Immunopathology|date=January 2016|volume=38|issue=1|pages=113–21|doi=10.1007/s00281-015-0543-z|pmid=26563285}} 17. ^Page 285 in: Thomson and Cotton Lecture Notes in Pathology, Blackwell Scientific. Third Edition 18. ^1 {{cite web|title=Dermatomyositis Information|url=http://www.ninds.nih.gov/disorders/dermatomyositis/dermatomyositis.htm|publisher=National Institute of Neurological Disorders and Stroke|date=July 27, 2015|deadurl=no|archiveurl=https://web.archive.org/web/20161202230955/http://www.ninds.nih.gov/disorders/dermatomyositis/dermatomyositis.htm|archivedate=2 December 2016|df=dmy-all}} 19. ^{{Cite journal|last=Tymms|first=K. E.|last2=Webb|first2=J.|date=1985-12-01|title=Dermatopolymyositis and other connective tissue diseases: a review of 105 cases|journal=The Journal of Rheumatology|volume=12|issue=6|pages=1140–1148|issn=0315-162X|pmid=4093921}} 20. ^{{Cite journal|last=Cooper|first=Glinda S.|last2=Stroehla|first2=Berrit C.|date=2003-05-01|title=The epidemiology of autoimmune diseases|journal=Autoimmunity Reviews|volume=2|issue=3|pages=119–125|issn=1568-9972|pmid=12848952|doi=10.1016/s1568-9972(03)00006-5}} 21. ^{{Cite journal|last=Bernatsky|first=S.|last2=Joseph|first2=L.|last3=Pineau|first3=C. A.|last4=Bélisle|first4=P.|last5=Boivin|first5=J. F.|last6=Banerjee|first6=D.|last7=Clarke|first7=A. E.|date=2009-07-01|title=Estimating the prevalence of polymyositis and dermatomyositis from administrative data: age, sex and regional differences|journal=Annals of the Rheumatic Diseases|volume=68|issue=7|pages=1192–1196|doi=10.1136/ard.2008.093161|issn=1468-2060|pmid=18713785}} 22. ^{{Cite journal|last=Bendewald|first=Margo J.|last2=Wetter|first2=David A.|last3=Li|first3=Xujian|last4=Davis|first4=Mark D. P.|date=2010-01-01|title=Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota|journal=Archives of Dermatology|volume=146|issue=1|pages=26–30|doi=10.1001/archdermatol.2009.328|issn=1538-3652|pmc=2886726|pmid=20083689}} 23. ^1 2 3 4 5 6 7 {{cite journal|last1=Kuhn|first1=A|last2=Landmann|first2=A|last3=Bonsmann|first3=G|title=The skin in autoimmune diseases-Unmet needs.|journal=Autoimmunity Reviews|date=October 2016|volume=15|issue=10|pages=948–54|doi=10.1016/j.autrev.2016.07.013|pmid=27481041}} 24. ^{{cite journal|last1=Bohan|first1=A|last2=Peter|first2=JB|title=Polymyositis and dermatomyositis (first of two parts).|journal=The New England Journal of Medicine|date=13 February 1975|volume=292|issue=7|pages=344–7|pmid=1090839|doi=10.1056/nejm197502132920706|url=http://www.medarhive.ru:80/jour/article/download/723/622}} and {{cite journal|last1=Bohan|first1=A|last2=Peter|first2=JB|title=Polymyositis and dermatomyositis (second of two parts).|journal=The New England Journal of Medicine|date=20 February 1975|volume=292|issue=8|pages=403–7|pmid=1089199|doi=10.1056/nejm197502202920807|url=http://www.medarhive.ru:80/jour/article/view/723|type=Submitted manuscript}} 25. ^{{cite web|url=http://www.grreporter.info/en/maria_callas_did_not_kill_herself_grief_onassis_rare_disease_cost_her_career_and_life/3863|title=Greek Reporter: 'Maria Callas did not kill herself from grief for Onassis, a rare disease cost her career and life'|date=28 December 2010|publisher=GR Reporter|accessdate=1 January 2015|deadurl=no|archiveurl=https://web.archive.org/web/20150104202125/http://www.grreporter.info/en/maria_callas_did_not_kill_herself_grief_onassis_rare_disease_cost_her_career_and_life/3863|archivedate=4 January 2015|df=dmy-all}} 26. ^{{cite web|url=http://www.people.com/people/archive/article/0,,20120826,00.html|title=Laurence Olivier Dies: 'The Rest Is Silence'|date=24 July 1989|publisher=People Magazine|accessdate=16 July 2012|deadurl=no|archiveurl=https://web.archive.org/web/20110310165616/http://www.people.com/people/archive/article/0,,20120826,00.html|archivedate=10 March 2011|df=dmy-all}} 27. ^{{cite web|url=http://www.profootballweekly.com/2010/01/08/forgotten-ricky-bell|title=Forgotten: Ricky Bell|date=8 January 2010|publisher=Pro Football Weekly|accessdate=26 January 2010|deadurl=no|archiveurl=https://web.archive.org/web/20100111093212/http://www.profootballweekly.com/2010/01/08/forgotten-ricky-bell|archivedate=11 January 2010|df=dmy-all}} 28. ^{{Cite newspaper|url=https://www.theguardian.com/books/2011/oct/12/rob-buckman|title=Rob Buckman obituary|journal=The Guardian|date=12 October 2011|accessdate=16 July 2012|deadurl=no|archiveurl=https://web.archive.org/web/20131001025516/http://www.theguardian.com/books/2011/oct/12/rob-buckman|archivedate=1 October 2013|df=dmy-all|last1=Jones|first1=Terry}} 29. ^{{cite web|url=https://www.thestar.com/news/gta/article/1067533--dr-robert-buckman-renowned-oncologist-comedian-and-star-columnist-dead-at-63|title=Dr. Robert Buckman, renowned oncologist, comedian and Star columnist, dead at 63|date=10 October 2011|publisher=The Toronto Star Newspaper|accessdate=16 July 2012|deadurl=no|archiveurl=https://web.archive.org/web/20130120131415/http://www.thestar.com/news/gta/article/1067533--dr-robert-buckman-renowned-oncologist-comedian-and-star-columnist-dead-at-63|archivedate=20 January 2013|df=dmy-all}} 30. ^{{cite journal|last1=Ramos-E-Silva|first1=M|last2=Pinto|first2=AP|last3=Pirmez|first3=R|last4=Cuzzi|first4=T|last5=Carneiro|first5=SC|title=Dermatomyositis--Part 1: Definition, Epidemiology, Etiology and Pathogenesis, and Clinics.|journal=Skinmed|date=1 August 2016|volume=14|issue=4|pages=273–279|pmid=27784516}} External links{{Portal|Medicine}}{{Medical resources| DiseasesDB = 10343 | ICD10 = {{ICD10|M|33|0|m|30}}-{{ICD10|M|33|1|m|30}} (ILDS M33.910) | ICD9 = {{ICD9|710.3}} | ICDO = | OMIM = | MedlinePlus = 000839 | eMedicineSubj = med | eMedicineTopic = 2608 | eMedicine_mult = {{eMedicine2|derm|98}} | MeshID = D003882 | Scholia = Q681160 }}{{Systemic connective tissue disorders}}{{Inflammation}}{{Paraneoplastic syndromes}}{{Authority control}} 8 : Autoimmune diseases|Connective tissue diseases|Disorders of fascia|Steroid-responsive inflammatory conditions|Systemic connective tissue disorders|RTT|RTTNEURO|Idiopathic diseases |
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