Scleroderma natural history, complications and prognosis: Difference between revisions
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===Prognosis=== | ===Prognosis=== | ||
* | *The 10-year survival rate of patients with scleroderma is approximately 70%-80%.<ref name="pmid14650470">{{cite journal |vauthors=Korn JH |title=Scleroderma: a treatable disease |journal=Cleve Clin J Med |volume=70 |issue=11 |pages=954, 956, 958 passim |date=November 2003 |pmid=14650470 |doi= |url=}}</ref> | ||
*The presence of pulmonary arterial hypertension is associated with a particularly poor prognosis among patients with scleroderma.<ref name="pmid23292818">{{cite journal |vauthors=Yaqub A, Chung L |title=Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis |journal=Curr Rheumatol Rep |volume=15 |issue=1 |pages=302 |date=January 2013 |pmid=23292818 |doi=10.1007/s11926-012-0302-2 |url=}}</ref> | *The presence of pulmonary arterial hypertension is associated with a particularly poor prognosis among patients with scleroderma.<ref name="pmid23292818">{{cite journal |vauthors=Yaqub A, Chung L |title=Epidemiology and risk factors for pulmonary hypertension in systemic sclerosis |journal=Curr Rheumatol Rep |volume=15 |issue=1 |pages=302 |date=January 2013 |pmid=23292818 |doi=10.1007/s11926-012-0302-2 |url=}}</ref> | ||
*The survival rate of patients with scleroderma is negatively affected by older age of onset.<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> | *The survival rate of patients with scleroderma is negatively affected by older age of onset.<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> | ||
*The mean survival of patients with scleroderma is approximately 12 years from diagnosis.<ref name="pmid12841293">{{cite journal |vauthors=Mayes MD |title=Scleroderma epidemiology |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=239–54 |date=May 2003 |pmid=12841293 |doi= |url=}}</ref> | *The mean survival of patients with scleroderma is approximately 12 years from diagnosis.<ref name="pmid12841293">{{cite journal |vauthors=Mayes MD |title=Scleroderma epidemiology |journal=Rheum. Dis. Clin. North Am. |volume=29 |issue=2 |pages=239–54 |date=May 2003 |pmid=12841293 |doi= |url=}}</ref> | ||
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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
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
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.[1]
- If left untreated, patients with scleroderma may progress to develop pulmonary arterial hypertension (PAH), interstitial lung disease and severe gastrointestinal disease.[1]
Complications
- Common complications of scleroderma include:[2][3][4]
- Scleroderma renal crisis
- Pulmonary fibrosis
- Pulmonary arterial hypertension
- Interstitial lung disease
- Malignancy
Prognosis
- The 10-year survival rate of patients with scleroderma is approximately 70%-80%.[5]
- The presence of pulmonary arterial hypertension is associated with a particularly poor prognosis among patients with scleroderma.[6]
- The survival rate of patients with scleroderma is negatively affected by older age of onset.[2]
- The mean survival of patients with scleroderma is approximately 12 years from diagnosis.[7]
References
- ↑ 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.
- ↑ 2.0 2.1 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.
- ↑ 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.
- ↑ 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.
- ↑ Korn JH (November 2003). "Scleroderma: a treatable disease". Cleve Clin J Med. 70 (11): 954, 956, 958 passim. PMID 14650470.
- ↑ 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.
- ↑ Mayes MD (May 2003). "Scleroderma epidemiology". Rheum. Dis. Clin. North Am. 29 (2): 239–54. PMID 12841293.