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| __NOTOC__ | | __NOTOC__ |
| {{DiseaseDisorder infobox |
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| Name = Systemic sclerosis |
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| ICD10 = {{ICD10|M|34||m|30}} |
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| ICD9 = {{ICD9|710.1}} |
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| ICDO = |
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| Image = Scleroderma-301.jpg|
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| Caption = MCP, PIP and DIP joints destructions in patient with Scleroderma. <br> [http://www.radswiki.net Image courtesy of RadsWiki]|
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| OMIM = 181750 |
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| MedlinePlus = 000429 |
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| DiseasesDB = 12845 |
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| MeshID = D012595 |
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| }}
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| {{Scleroderma}} | | {{Scleroderma}} |
| | | {{CMG}}; '''Associate Editor-In-Chief:''' {{MKA}} {{CZ}} |
| {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} | |
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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{SK}} Systemic sclerosis | | {{SK}} Systemic sclerosis; CREST syndrome; limited scleroderma; progressive systemic sclerosis; localized scleroderma; mixed connective disease; morphea - linear; sclerosis, systemic; systemic scleroderma |
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| ===Click [[The Heart in Progressive Systemic Sclerosis (Scleroderma)|here]] for The Heart in Scleroderma=== | | ===Click [[The Heart in Progressive Systemic Sclerosis (Scleroderma)|here]] for The Heart in Scleroderma=== |
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| ==Overview== | | ==[[Scleroderma overview|Overview]]== |
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| == Epidemiology == | | ==[[Scleroderma historical perspective|Historical Perspective]]== |
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| ==Pathophysiology== | | ==[[Scleroderma classification|Classification]]== |
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| ==Etiology== | | ==[[Scleroderma pathophysiology|Pathophysiology]]== |
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| ==Types of Scleroderma== | | ==[[Scleroderma causes|Causes]]== |
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| ==Diagnosis== | | ==[[Differentiating Scleroderma from other diseases|Differentiating Scleroderma from other Diseases]]== |
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| Diagnosis is by clinical suspicion, presence of autoantibodies (specifically [[anti-centromere antibodies|anti-centromere]] and anti-scl70/[[anti-topoisomerase antibodies]]) and occasionally by biopsy. Of the antibodies, 90% have a detectable [[anti-nuclear antibody]]. Anti-centromere antibody is more common in the limited form (80-90%) than in the systemic form (10%), and anti-scl70 is more common in the diffuse form (30-40%) and in African-American patients (who are more susceptible to the systemic form).<ref name=JimenezDerk>{{cite journal |author=Jimenez SA, Derk CT |title=Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis |journal=Ann. Intern. Med. |volume=140 |issue=1 |pages=37-50 |year=2004 |pmid=14706971 |doi=}}</ref>
| | ==[[Scleroderma epidemiology and demographics|Epidemiology and Demographics]]== |
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| In 1980 the American College of Rheumatology agreed upon diagnostic criteria for scleroderma.<ref>{{cite journal |author= |title=Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee |journal=Arthritis Rheum. |volume=23 |issue=5 |pages=581-90 |year:1980 |pmid=7378088 |doi=}} Available online at {{cite web | title=Criteria for the Classification of Systemic Sclerosis 1980| work= | url=http://www.rheumatology.org/publications/classification/systsclr.asp | accessdate:5 August 2007}}</ref>
| | ==[[Scleroderma risk factors|Risk Factors]]== |
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| Diffuse scleroderma can cause [[musculoskeletal system|musculoskeletal]], [[lung|pulmonary]], [[gastrointestinal tract|gastrointestinal]], [[kidney|renal]] and other complications.<ref name=Primer/>Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs.
| | ==[[Scleroderma screening|Screening]]== |
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| ===[[The heart in progressive systemic sclerosis (scleroderma)|Cardiovascular Symptoms]]===
| | ==[[Scleroderma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ===Skin Symptoms=== | | ==Diagnosis== |
| | | [[Scleroderma diagnostic study of choice|Diagnostic Study of Choice]] | [[Scleroderma history and symptoms|History and Symptoms]] | [[Scleroderma physical examination|Physical Examination]] | [[Scleroderma laboratory findings|Laboratory Findings]] | [[Scleroderma electrocardiogram|Electrocardiogram]] | [[Scleroderma x ray|X Ray]] | [[Scleroderma CT|CT]] | [[Scleroderma MRI|MRI]] | [[Scleroderma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Scleroderma other imaging findings|Other Imaging Findings]] | [[Scleroderma other diagnostic studies|Other Diagnostic Studies]] |
| ===Musculoskeletal System Related Symptoms===
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| ===Respiratory System Symptoms===
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| ===Gastrointestinal System Related Symptoms===
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| ===Renal Symptoms===
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| == Therapy ==
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| There is no cure for every patient with scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.<ref>{{cite journal |author=Oliver GF, Winkelmann RK |title=The current treatment of scleroderma |journal=Drugs |volume=37 |issue=1 |pages=87-96 |year=1989 |pmid=2651089 |doi=}}</ref>
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| Digital ulcerations and pulmonary hypertension can be helped by [[prostacyclin]] (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.<ref>{{cite journal |author=Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y |title=New therapeutic strategies for systemic sclerosis--a critical analysis of the literature |journal=Clin. Dev. Immunol. |volume=12 |issue=3 |pages=165-73 |year=2005 |pmid=16295521 |doi=}}</ref>
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| ===Topical/symptomatic=== | | ==Treatment== |
| | | [[Scleroderma medical therapy|Medical Therapy]] | [[Scleroderma surgery|Surgery]] | [[Scleroderma primary prevention|Primary Prevention]] | [[Scleroderma secondary prevention|Secondary Prevention]] | [[Scleroderma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Scleroderma future or investigational therapies|Future or Investigational Therapies]] |
| Topical treatment for the skin changes of scleroderma do not alter the disease course, but may improve pain and ulceration. A range of [[NSAID]]s (nonsteroidal anti-inflammatory drugs) can be used to ease painful symptoms, such as [[naproxen]]. There is limited benefit from [[glucocorticoid|steroids]] such as prednisone. Episodes of Raynaud's phenomenon sometimes respond to [[nifedipine]] or other calcium channel blockers; severe digital ulceration may respond to [[prostacyclin]] analogue [[iloprost]], and the dual endothelin-receptor antagonist [[bosentan]] may be beneficial for Raynaud's phenomenon.<ref name=Zandberg>{{cite journal |author=Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y |title=New therapeutic strategies for systemic sclerosis--a critical analysis of the literature |journal=Clin. Dev. Immunol. |volume=12 |issue=3 |pages=165–73 |year=2005 |pmid=16295521 |doi=}} {{PMC|2275417}}</ref> The skin tightness may be treated systemically with [[methotrexate]] and [[cyclosporin]].<ref name=Zandberg/> If there is esophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs as they are gastric irritants, and so a [[proton pump inhibitor]] (PPI) such as [[omeprazole]] can be given in conjunction.
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| ===Kidney disease===
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| Scleroderma renal crisis, the occurrence of [[acute renal failure]] and [[malignant hypertension]] (very high blood pressure with evidence of organ damage) in people with scleroderma, is effectively treated with drugs from the class of the [[ACE inhibitor]]s. The benefit of ACE inhibitors extends even to those who have to commence [[hemodialysis|dialysis]] to treat their kidney disease, and may give sufficient benefit to allow the discontinuation of renal replacement therapy.<ref name=Zandberg/> [[ACE inhibitors]] are also used for [[prophylaxis]],<ref name=jimenez/><ref name=steen11033587/> and [[renal transplantation]]. Transplanted kidneys are known to be affected by scleroderma and patients with early onset renal disease (within one year of the scleroderma diagnosis) are thought to have the highest risk for recurrence.<ref>Pham PT, Pham PC, Danovitch GM, Gritsch HA, Singer J, Wallace WD, Hayashi R, Wilkinson AH. Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. Am J Transplant. 2005 Oct;5(10):2565-9. PMID 16162209.</ref> | |
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| ===Lung disease and pulmonary hypertension===
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| Active alveolitis is often treated with pulses of [[cyclophosphamide]], often together with a small dose of steroids. The benefit of this intervention is modest.<ref>{{cite journal |author=Tashkin DP, Elashoff R, Clements PJ, ''et al'' |title=Cyclophosphamide versus placebo in scleroderma lung disease |journal=N. Engl. J. Med. |volume=354 |issue=25 |pages=2655–66 |year=2006 |month=June |pmid=16790698 |doi=10.1056/NEJMoa055120 |url=http://content.nejm.org/cgi/content/full/354/25/2655}}</ref><ref>{{cite journal |author=Hoyles RK, Ellis RW, Wellsbury J, ''et al'' |title=A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma |journal=Arthritis Rheum. |volume=54 |issue=12 |pages=3962–70 |year=2006 |month=December |pmid=17133610 |doi=10.1002/art.22204 | url=http://www3.interscience.wiley.com/cgi-bin/fulltext/113490260/HTMLSTART}}</ref>
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| Pulmonary hypertension may be treated with [[epoprostenol]], [[bosentan]] and possibly aerolized iloprost.<ref name=Zandberg/>
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| ===Experimental treatments===
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| ==Case Studies== | | ==Case Studies== |
| [[Scleroderma case study one|Case #1]] | | [[Scleroderma case study one|Case #1]] |
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| ==References==
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| {{Reflist|2}}
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| ==External links==
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| * [http://goldminer.arrs.org/search.php?query=scleroderma Goldminer: Scleroderma]
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| * [http://dermnetnz.org/immune/systemic-sclerosis.html DermnetNZ: Systemic sclerosis]
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| * [http://www.jsdn.org/ Juvenile Scleroderma Network]
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| * [http://www.juvenile-scleroderma.com/ Juvenile Systemic Sclerosis]
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| * [http://www.scleroderma.org/ Scleroderma Foundation]
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| * [http://www.sclerodermaontario.ca/ Scleroderma Society of Ontario]
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| * [http://www.sclero.org/index.html International Scleroderma Network]
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| * [http://www.sclerodermaresearch.org/ The Scleroderma Research Foundation]
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| * [http://www.sclerodermasociety.co.uk/ UK Scleroderma Society]
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| * [http://scleroderma.jhmi.edu/ Scleroderma Information from Johns Hopkins University]
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| {{Diseases of the musculoskeletal system and connective tissue}} | | {{Diseases of the musculoskeletal system and connective tissue}} |
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| [[Category:Autoimmune diseases]] | | [[Category:Autoimmune diseases]] |
| [[Category:Diseases involving the fasciae]] | | [[Category:Diseases involving the fasciae]] |
| | [[Category:Rheumatology]] |
| | [[Category:Up-To-Date]] |
| | [[Category:Primary care]] |
| | [[Category:Medicine]] |
| | [[Category:Dermatology]] |
| [[Category:Rheumatology]] | | [[Category:Rheumatology]] |
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