Scleroderma natural history, complications and prognosis

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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 (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

  • Common complications of scleroderma include:[1][2][3]
    • Scleroderma renal crisis
    • Pulmonary fibrosis
    • Pulmonary arterial hypertension
    • Interstitial lung disease
    • Malignancy

Prognosis

  • Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
  • Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
  • The presence of pulmonary arterial hypertension is associated with a particularly poor prognosis among patients with scleroderma.[4]
  • [Subtype of disease/malignancy] is associated with the most favorable prognosis.
  • The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
  • The survival rate of patients with scleroderma is negatively affected by older age of onset.[1]

References

  1. 1.0 1.1 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.
  2. 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.
  3. 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.
  4. 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.

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