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Systemic Sclerosis (SSc)

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Prognosis

Systemic sclerosis (SSc) is a disease characterized by fibrosis and microvasculopathy that leads to an increased deposition of collagen in the skin and the internal organs.1,2 SSc is linked to a high mortality rate with the highest case-specific mortality of all collagen vascular diseases.3 However, the prognosis of SSc has improved over the last 30 years, with 5-year survival rates reaching up to 80%.3 

General Prognosis of SSc

Many studies have been performed to identify not only the causes of death in SSc, but also to establish survival prognosis and predictors of poor outcomes.1,2 Scleroderma renal crisis (SRC) remained the main cause of death in patients with SSc for many years, but a progressive change in the causes of death in SSc has been observed. In the last 40 years, SRC mortality has decreased from 42% in the 1970s to 6% in 2010, partly due to the use of angiotensin-converting enzyme (ACE) inhibitors.1,3 The 1-year survival rate of patients with SRC is now over 85%.3 Lung involvement, specifically pulmonary fibrosis and pulmonary arterial hypertension (PAH), now represents the leading cause of death in SSc.1,3 

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Prognostic Factors in Systemic Sclerosis

Various studies suggest that the prognosis of SSc is related to factors such as disease subtypes, antibody profile, and internal organ involvement.2,3 Overall, prognosis is worse in men, patients who develop the disease at an older age, patients with diffuse cutaneous SSc (dcSSc), and patients with internal organ involvement, such as the heart, lungs, or kidneys.1 

Effects of Disease Subtype on SSc Prognosis

The prognosis of patients with limited SSc (CREST syndrome) is more favorable than other types of SSc.4 In patients with limited SSc, the disease may remain stable for many years, while in patients with diffuse cutaneous SSc (dcSSc), lack of treatment may quickly escalate the disease and increase the risk of death.5 Patients with dcSSc are at a higher risk of developing progressive end-organ damage than patients with limited disease.6 Overall, about 92% of patients with limited disease and 65% of patients with dcSSc live for at least 10 years following diagnosis.4 

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Effects of Internal Organ Involvement on SSc Prognosis

Prognosis is also influenced by the involvement of internal organs. Reports have shown that patients presenting with severe skin, lung, heart, gastrointestinal, or kidney involvement had a 9-year cumulative survival rate of 38%, while patients without organ involvement had a comparative survival rate of 78%.7

Patients with SSc who develop pulmonary arterial hypertension have a worse prognosis and survival.5,8 These patients with secondary PAH also present a poorer prognosis compared to patients with idiopathic PAH.3 Patients with PAH have a 2-year survival rate of less than 50%.1,3 In addition, cigarette smoking is linked to reduced survival.5

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Effects of Laboratory Markers on SSc Prognosis

Laboratory variables that can function as factors of poor prognosis include anemia, increased erythrocyte sedimentation rate, abnormal urine sedimentation, hypergammaglobulinemia, and the presence of anti-Scl-70 antibodies, which can point to dcSSc, interstitial lung disease, or cardiac involvement.1,3 The presence of anti-RNA polymerase III antibodies is also a prognostic factor that can indicate rapidly progressive and aggressive diffuse skin involvement, poor outcomes, and SRC.3 The presence of anti-topoisomerase I antibodies and/or anti-Th/T0 antibodies has been associated with increased mortality.6

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Although SSc is more common in women, with a pooled prevalence and incidence rate 5 times higher than that of men9, there is a higher mortality in male patients with SSc. One study found male sex to be a considerable factor of worse prognosis, with a calculated survival rate of 77% in 5 years and 64% in 10 years, compared to 90% at 5 years and 86% at 10 years for females with SSc.1 

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Much research has proposed that age, specifically older age at disease onset, be included as an individual predictor of poor outcome. A 2020 study examined disease progression in patients with disease onset later than age 60 and found a significant acceleration of internal organ involvement in the group, leading to worsened prognosis.10  

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Studies suggest that the incidence of SSc is higher in Black individuals, who develop severe disease early with interstitial lung disease and SRC, presenting a worse prognosis.2,3 Studies also suggested that Black Americans were more likely to have diffuse skin involvement, lung dysfunction, and digital ulcers, compared to White Americans.1 

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Patients with SSc are at a high risk of developing malignancies, such as lung cancer. Additionally, patients may have an increased risk of developing esophageal adenocarcinoma when SSc is associated with chronic gastroesophageal reflux leading to Barrett’s esophagus.3 A previous study involving 5860 patients with SSc has additionally reported that 41% of deaths observed were related to non-SSc causes, including infections, cardiovascular causes, and malignancies.1 

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References

1. 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.111582

2. 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

3. Adigun R, Goyal A, Hariz A. Systemic sclerosis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2023. Updated May 8, 2022. Accessed April 11, 2023.

4. Nevares AM. Systemic sclerosis. MSD Manual Consumer Version. Updated October 2022. Accessed April 11, 2023.

5. Schwartz RA. Systemic sclerosis: prognosis. Medscape. Updated April 16, 2021. Accessed April 11, 2023.

6. 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

7. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437-2444. doi:10.1002/1529-0131(200011)43:11<2437::AID-ANR10>3.0.CO;2-U

8. Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304-318. doi:10.1016/S0140-6736(22)01692-0

9. Bairkdar M, Rossides M, Westerlind H, Hesselstrand R, Arkema EV, Holmqvist M. Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis. Rheumatology (Oxford). 2021;60(7):3121-3133. doi:10.1093/rheumatology/keab190

10. Moinzadeh P, Kuhr K, Siegert E, et al. Older age onset of systemic sclerosis – accelerated

disease progression in all disease subsets. Br J Rheumatol. 2020;59:3380–3389.

doi:10.1093/rheumatology/keaa127

Reviewed by Kyle Habet, MD, on 4/14/2023.

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