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Systemic Sclerosis (SSc)

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Life Expectancy

Systemic sclerosis (SSc) is a rare connective tissue disease that is characterized by inflammation, vasculopathy, and excessive fibrosis. SSc can cause thickening of the skin and internal organs, as well as damage to the blood vessels, muscles, and joints. The extension of skin lesions in SSc allows for further classification of the disease into limited cutaneous and diffuse cutaneous SSc.1 

Both the severity and prognosis of SSc are variable.2 SSc more commonly develops between the ages of 30 and 50 years, and it is estimated to affect 1 in 10,000 people.2,3 

General Life Expectancy of SSc

There is an increased risk of death in patients with SSc.4 The mortality rate of patients with SSc is 3.5 times higher than that of healthy individuals of similar ages.2 Over recent years, however, survival of patients with SSc has improved. Previous studies have shown a 10-year survival rate of 50%, while more recent data report 5- and 10-year survival rates of 90% and 84%, respectively.5 Because there are still no disease-modifying treatments available, improvements in survival may derive from improvements in diagnosis and the quality of medical care provided to treat the disease and its complications.1,5

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Life Expectancy Relative to Internal Organ Involvement

Internal organ involvement is a very frequent cause of death in patients with SSc, and life expectancy is influenced by the extent and severity of this involvement.2,6 Scleroderma renal crisis (SRC) was long considered a main cause of death in SSc; however, 1-year mortality rates have decreased with the introduction of angiotensin-converting enzyme inhibitors.7-9 The mortality linked to gastrointestinal involvement is now higher than the reported renal-related mortality.6

Pulmonary involvement is an important prognostic factor in SSc, with many studies showing a worse prognosis and higher mortality in patients with interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH).6 The 5-year survival rate of patients with SSc is greater than 90% when there is no ILD involved and about 70% when patients present with ILD.2 PAH is a complication of SSc, and even though the associated prognosis has improved due to early diagnosis and adequate treatment, the 3-year mortality rate is between 21% and 48%, while 1-year survival can reach 55%.2,9 

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Life Expectancy Relative to Disease Subtype

The subtype of SSc influences life expectancy in patients with SSc. The life expectancy of patients with limited cutaneous SSc (lcSSc) is similar to that of the general population, but patients with diffuse cutaneous SSc (dcSSc) experience significant early mortality.2 Approximately 92% of patients with lcSSC and 65% of patients with dcSSc live for at least 10 years after receiving a diagnosis.10 Patients with lcSSC can remain stable for years, and the hardening of the skin may halt after 2 years following disease onset.2 In patients with dcSSc, damage to internal organs occurs earlier, and the prognosis is worse with possible end-organ damage that increases the risk of death.11,12

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Other Factors Influencing Life Expectancy 

Life Expectancy Relative to Autoantibodies

The presence of autoantibodies can provide prognostic information in patients with SSc. The presence of anticentromere antibodies is associated with a 93% 10-year survival rate, while anti-Scl-70 and anti-RNA-polymerase III antibodies are linked to 10-year survival rates of 66% and 30%, respectively.2

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Life Expectancy Relative to Comorbidities

With the increase in life expectancy, comorbidities and long-term complications may become relevant over the course of the disease. Non-SSc related deaths can derive from cardiovascular or cerebral disease, as well as from infections and tumors.5,7 Several studies report an elevated incidence of cancer in patients with SSc.5 

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References

  1. Kennedy N, Walker J, Hakendorf P, Roberts-Thomson P. Improving life expectancy of patients with scleroderma: results from the South Australian Scleroderma Register. Intern Med J. 2018;48(8):951-956. doi:10.1111/imj.13799
  2. Odonwodo A, Badri T, Hariz A. Scleroderma. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2023. Updated August 1, 2022. Accessed April 28, 2023.
  3. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi:10.1016/S0140-6736(17)30933-9
  4. Truchetet ME, Brembilla NC, Chizzolini C. Current concepts on the pathogenesis of systemic sclerosis. Clin Rev Allergy Immunol. 2023;64:262–283. doi:10.1007/s12016-021-08889-8
  5. Hu S, Hou Y, Wang Q, Li M, Xu D, Zeng X. Prognostic profile of systemic sclerosis: analysis of the clinical EUSTAR cohort in China. Arthritis Res Ther. 2018;20(1):235. doi:10.1186/s13075-018-1735-4
  6. Poudel DR, Derk CT. Mortality and survival in systemic sclerosis: a review of recent literature. Curr Opin Rheumatol. 2018;30(6):588-593. doi:10.1097/BOR.0000000000000551
  7. Sampaio-Barros PD, Bortoluzzo AB, Marangoni RG, et al. Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients. J Rheumatol. 2012;39(10):1971-1978. doi:10.3899/jrheum.111582Adigun R, Goyal A, 
  8. Hariz A. Systemic sclerosis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2023. Updated May 8, 2022. Accessed April 28, 2023.
  9. Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304-318. doi:10.1016/S0140-6736(22)01692-0
  10. Nevares AM. Systemic sclerosis (scleroderma). MSD Manual Consumer Version. Updated October 2022. Accessed April 28, 2023.
  11. Schwartz RA. Systemic sclerosis: prognosis. Medscape. Updated April 16, 2021. Accessed April 28, 2023.
  12. Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous manifestations of scleroderma and scleroderma-like disorders: a comprehensive review. Clin Rev Allergy Immunol. 2017;53(3):306-336. doi:10.1007/s12016-017-8625-4

Reviewed by Hasan Avcu, MD, on 4/29/2023.

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