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scleroderma
SCLERODERMA
Treatment Guideline Chart
Scleroderma is a connective tissue disorder characterized by skin thickening and fibrosis. It is rare, autoimmune and chronic.
It has an idiopathic cause and not contagious.
Early microvascular damage, mononuclear cell infiltrates and slowly developing fibrosis are the important features of the tissue lesions.
The leading causes of death are pulmonary fibrosis, pulmonary arterial hypertension and primary cardiac damage.

Scleroderma References

  1. MedWorm http://www.medworm.com/rss/index.php/Dermatology/12/ http://www.medworm.com/rss/medicalfeeds/specialities/Dermatology-News.xml
  2. Denton CP. Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. UpToDate. https://www.uptodate.com. 20 Nov 2019. Accessed 13 Apr 2020.
  3. Denton C. Immunomodulatory and antifibrotic approaches to the treatment of systemic sclerosis (scleroderma). UpToDate. http://www.uptodate.com/contents/immunomodulatory-and-antifibrotic-approaches-to-the-treatment-of-systemic-sclerosis-scleroderma. Jul 2013.
  4. Denton C. Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. UpToDate. http://www.uptodate.com/contents/overview-of-the-treatment-and-prognosis-of-systemic-sclerosis-scleroderma-in-adults. Oct 2013.
  5. Jacobe H. Treatment of morphea (localized scleroderma) in adults. UpToDate. https://www.uptodate.com. 16 Apr 2018.
  6. American College of Rheumatology. Localized scleroderma. American College of Rheumatology. https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Localized_Scleroderma_(Juvenile)/. Jun 2012.
  7. American College of Rheumatology. Scleroderma (also known as systemic sclerosis). American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/scleroderma.asp. Feb 2013.
  8. Arthritis Foundation of Malaysia. Scleroderma. Arthritis Foundation of Malaysia. http://www.afm.org.my/info/scleroderma.htm.
  9. van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-1755. doi: 10.1136/annrheumdis-2013-204424. PMID: 24092682
  10. Varga J. Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults. UpToDate. http://www.uptodate.com/contents/overview-of-the-clinical-manifestations-of-systemic-sclerosis-scleroderma-in-adults. Aug 2013.
  11. Varga J. Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults. UpToDate. http://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-systemic-sclerosis-scleroderma-in-adults. Nov 2012.
  12. Fernández-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group. Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheumatol. 2018 Nov;70(11):1820-1828. doi: 10.1002/art.40560. Accessed 13 Apr 2020. PMID: 29781586
  13. Hopkins W, Rubin LJ. Treatment of pulmonary arterial hypertension (group 1) in adults: pulmonary hypertension-specific therapy. UpToDate. https://www.uptodate.com. 28 Jan 2020.
  14. Wigley FM. Treatment of Raynaud phenomenon: initial management. UpToDate. https://www.uptodate.com. 13 Nov 2019.
  15. Wigley FM. Treatment of Raynaud phenomenon: refractory or progressive ischemia. UpToDate. https://www.uptodate.com. 18 Jul 2019.
  16. Weatherald J, Montani D, Jevnikar M, et al. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2019 Jul 31;28(153):pii: 190023. doi: 10.1183/16000617.0023-2019. PMID: 31366460
  17. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015 Oct;46(4):903-975. doi: 10.1183/13993003.01032-2015. Accessed 19 May 2020. PMID: 26318161
  18. Denton CP, Hughes M, Gak N, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology (Oxford). 2016 Oct;55(10):1906-1910. doi: 10.1093/rheumatology/kew224. PMID: 27284161
  19. Kowal-Bielecka O, Fransen J, Avouac J, et al; EUSTAR Coauthors. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-1339. doi: 10.1136/annrheumdis-2016-209909. PMID: 27941129
  20. Hachulla E, Agard C, Allanore Y, et al. French recommendations for the management of systemic sclerosis. Orphanet J Rare Dis. 2021 Jul 26;16(Suppl 2):322. doi: 10.1186/s13023-021-01844-y. PMID: 34304732
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