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==Overview==
==Overview==
If left untreated, patients with scleroderma may progress to develop [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]], [[interstitial lung disease]] and severe gastrointestinal disease. Common complications of scleroderma include [[pulmonary fibrosis]], [[Pulmonary hypertension|pulmonary arterial hypertension]], [[interstitial lung disease]] and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.
The symptoms of scleroderma usually develop in the third decade of life, and start with symptoms such as skin thickening and [[edema]] of the hands and feet. Common complications of scleroderma include [[pulmonary fibrosis]], [[Pulmonary hypertension|pulmonary arterial hypertension]], [[interstitial lung disease]] and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
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===Natural History===
===Natural History===
*The symptoms of scleroderma usually develop in the third decade of life, and start with symptoms such as skin thickening and [[edema]] of the hands and feet.<ref name="pmid12841294">{{cite journal |vauthors=Medsger TA |title=Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=255–73, vi |date=May 2003 |pmid=12841294 |doi= |url=}}</ref><ref name="pmid24646463">{{cite journal |vauthors=Alba MA, Velasco C, Simeón CP, Fonollosa V, Trapiella L, Egurbide MV, Sáez L, Castillo MJ, Callejas JL, Camps MT, Tolosa C, Ríos JJ, Freire M, Vargas JA, Espinosa G |title=Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients |journal=Medicine (Baltimore) |volume=93 |issue=2 |pages=73–81 |date=March 2014 |pmid=24646463 |pmc=4616306 |doi=10.1097/MD.0000000000000018 |url=}}</ref>  
*The symptoms of scleroderma usually develop in the third decade of life, and start with symptoms such as skin thickening and [[edema]] of the hands and feet.<ref name="pmid12841294">{{cite journal |vauthors=Medsger TA |title=Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=255–73, vi |date=May 2003 |pmid=12841294 |doi= |url=}}</ref><ref name="pmid24646463">{{cite journal |vauthors=Alba MA, Velasco C, Simeón CP, Fonollosa V, Trapiella L, Egurbide MV, Sáez L, Castillo MJ, Callejas JL, Camps MT, Tolosa C, Ríos JJ, Freire M, Vargas JA, Espinosa G |title=Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients |journal=Medicine (Baltimore) |volume=93 |issue=2 |pages=73–81 |date=March 2014 |pmid=24646463 |pmc=4616306 |doi=10.1097/MD.0000000000000018 |url=}}</ref>  
*If left untreated, patients with scleroderma may progress to develop [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]], [[interstitial lung disease]] and severe gastrointestinal disease.<ref name="pmid12841294">{{cite journal |vauthors=Medsger TA |title=Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=255–73, vi |date=May 2003 |pmid=12841294 |doi= |url=}}</ref>
*If left untreated, patients with scleroderma may progress to develop [[Pulmonary arterial hypertension|pulmonary arterial hypertension (PAH)]], [[interstitial lung disease]], and severe gastrointestinal disease.<ref name="pmid12841294">{{cite journal |vauthors=Medsger TA |title=Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=255–73, vi |date=May 2003 |pmid=12841294 |doi= |url=}}</ref>


===Complications===
===Complications===
*Common complications of scleroderma include:<ref name="pmid22269658">{{cite journal |vauthors=Barnes J, Mayes MD |title=Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers |journal=Curr Opin Rheumatol |volume=24 |issue=2 |pages=165–70 |date=March 2012 |pmid=22269658 |doi=10.1097/BOR.0b013e32834ff2e8 |url=}}</ref><ref name="pmid20551155">{{cite journal |vauthors=Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA |title=Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database |journal=Ann. Rheum. Dis. |volume=69 |issue=10 |pages=1809–15 |date=October 2010 |pmid=20551155 |doi=10.1136/ard.2009.114264 |url=}}</ref><ref name="pmid17329309">{{cite journal |vauthors=Steen VD, Medsger TA |title=Changes in causes of death in systemic sclerosis, 1972-2002 |journal=Ann. Rheum. Dis. |volume=66 |issue=7 |pages=940–4 |date=July 2007 |pmid=17329309 |pmc=1955114 |doi=10.1136/ard.2006.066068 |url=}}</ref>
*Common complications of scleroderma include:<ref name="pmid22269658">{{cite journal |vauthors=Barnes J, Mayes MD |title=Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers |journal=Curr Opin Rheumatol |volume=24 |issue=2 |pages=165–70 |date=March 2012 |pmid=22269658 |doi=10.1097/BOR.0b013e32834ff2e8 |url=}}</ref><ref name="pmid20551155">{{cite journal |vauthors=Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA |title=Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database |journal=Ann. Rheum. Dis. |volume=69 |issue=10 |pages=1809–15 |date=October 2010 |pmid=20551155 |doi=10.1136/ard.2009.114264 |url=}}</ref><ref name="pmid17329309">{{cite journal |vauthors=Steen VD, Medsger TA |title=Changes in causes of death in systemic sclerosis, 1972-2002 |journal=Ann. Rheum. Dis. |volume=66 |issue=7 |pages=940–4 |date=July 2007 |pmid=17329309 |pmc=1955114 |doi=10.1136/ard.2006.066068 |url=}}</ref><ref name="pmid29264402">{{cite journal |vauthors=Shah AA, Casciola-Rosen L |title=Mechanistic and clinical insights at the scleroderma-cancer interface |journal=J Scleroderma Relat Disord |volume=2 |issue=3 |pages=153–159 |date=2017 |pmid=29264402 |pmc=5734659 |doi=10.5301/jsrd.5000250 |url=}}</ref>
**Scleroderma renal crisis
**Scleroderma renal crisis
**[[Pulmonary fibrosis]]
**[[Pulmonary fibrosis]]
**[[Pulmonary arterial hypertension]]
**[[Pulmonary arterial hypertension]]
**[[Interstitial lung disease]]
**[[Interstitial lung disease]]
**[[Malignancy]]
**[[Malignancy]]<ref name="pmid26352736">{{cite journal |vauthors=Shah AA, Casciola-Rosen L |title=Cancer and scleroderma: a paraneoplastic disease with implications for malignancy screening |journal=Curr Opin Rheumatol |volume=27 |issue=6 |pages=563–70 |date=November 2015 |pmid=26352736 |pmc=4643720 |doi=10.1097/BOR.0000000000000222 |url=}}</ref>


===Prognosis===
===Prognosis===
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[[Category:Rheumatology]]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]

Overview

The symptoms of scleroderma usually develop in the third decade of life, and start with symptoms such as skin thickening and edema of the hands and feet. Common complications of scleroderma include pulmonary fibrosis, pulmonary arterial hypertension, interstitial lung disease and scleroderma renal crisis. The 10-year survival rate of patients with scleroderma is approximately 70%-80%.

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

  • The 10-year survival rate of patients with scleroderma is approximately 70%-80%.[8]
  • The presence of pulmonary arterial hypertension is associated with a particularly poor prognosis among patients with scleroderma.[9]
  • The survival rate of patients with scleroderma is negatively affected by older age of onset.[3]
  • The mean survival of patients with scleroderma is approximately 12 years from diagnosis.[10]

References

  1. ↑ 1.0 1.1 Medsger TA (May 2003). "Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being". Rheum. Dis. Clin. North Am. 29 (2): 255–73, vi. PMID 12841294.
  2. ↑ Alba MA, Velasco C, Simeón CP, Fonollosa V, Trapiella L, Egurbide MV, Sáez L, Castillo MJ, Callejas JL, Camps MT, Tolosa C, Ríos JJ, Freire M, Vargas JA, Espinosa G (March 2014). "Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients". Medicine (Baltimore). 93 (2): 73–81. doi:10.1097/MD.0000000000000018. PMC 4616306. PMID 24646463.
  3. ↑ 3.0 3.1 Barnes J, Mayes MD (March 2012). "Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers". Curr Opin Rheumatol. 24 (2): 165–70. doi:10.1097/BOR.0b013e32834ff2e8. PMID 22269658.
  4. ↑ Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (October 2010). "Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database". Ann. Rheum. Dis. 69 (10): 1809–15. doi:10.1136/ard.2009.114264. PMID 20551155.
  5. ↑ Steen VD, Medsger TA (July 2007). "Changes in causes of death in systemic sclerosis, 1972-2002". Ann. Rheum. Dis. 66 (7): 940–4. doi:10.1136/ard.2006.066068. PMC 1955114. PMID 17329309.
  6. ↑ Shah AA, Casciola-Rosen L (2017). "Mechanistic and clinical insights at the scleroderma-cancer interface". J Scleroderma Relat Disord. 2 (3): 153–159. doi:10.5301/jsrd.5000250. PMC 5734659. PMID 29264402.
  7. ↑ Shah AA, Casciola-Rosen L (November 2015). "Cancer and scleroderma: a paraneoplastic disease with implications for malignancy screening". Curr Opin Rheumatol. 27 (6): 563–70. doi:10.1097/BOR.0000000000000222. PMC 4643720. PMID 26352736.
  8. ↑ Korn JH (November 2003). "Scleroderma: a treatable disease". Cleve Clin J Med. 70 (11): 954, 956, 958 passim. PMID 14650470.
  9. ↑ Yaqub A, Chung L (January 2013). "Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis". Curr Rheumatol Rep. 15 (1): 302. doi:10.1007/s11926-012-0302-2. PMID 23292818.
  10. ↑ Mayes MD (May 2003). "Scleroderma epidemiology". Rheum. Dis. Clin. North Am. 29 (2): 239–54. PMID 12841293.

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