Systemic Sclerosis: Prognosis and Mortality

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Age at Onset
Fear of Progression
Cardiac Involvement
Causes of Death
Lung Involvement
Nailfold Indicators
Prognostic Markers
Skin Thickness Progression
Symptoms of Systemic Scleroderma
Survival Rates


Years ago, only the worst cases of systemic sclerosis (scleroderma) were diagnosed, thus leading to the widespread belief that systemic scleroderma was always progressive and fatal.

Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much rosier longterm prognosis. (Also see What is Scleroderma?, Types of Scleroderma and Systemic Symptoms)

Mortality in systemic sclerosis-a single centre study from the UK. The most common cause of SSc-related mortality was pulmonary complications. Factors adversely affecting survival were older age at diagnosis, male gender, interstitial lung disease (ILD) and anti-RNA polymerase III antibody. PubMed, Clin Rheumatol, 2013 Jun 29.

Trends in mortality in patients with systemic sclerosis over 40 years. Our results confirm that systemic sclerosis is a devastating condition as reflected by a pooled standardized mortality ratios (SMRs) of 3.5. Additionally, SMR has not significantly changed over the past 40 years. Muriel Elhai, Oxford Journals, Rheumatology, 09/07/2011.

Age at Onset

Systemic sclerosis has a better prognosis when it onsets in the elderly.

Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. Peak age at onset of systemic sclerosis (SSc) is between 20 and 50 years, although SSc is also described in both young and elderly patients. We conducted the present study to determine if age at disease onset modulates the clinical characteristics and outcome of SSc patients. PubMed, Medicine (Baltimore). 2014 Mar;93(2):73-81. (Also see Types of Scleroderma)


Antibodies and Scleroderma. Dozens of antibodies can occur in systemic scleroderma patients. They can be useful for diagnosing, categorizing and predicting the likelihood of certain complications. ISN.


A Proteome derived longitudinal pharmacodynamic biomarker for diffuse systemic sclerosis (SSc) skin. In this study a large array of proteins were discovered, not previously associated with SSc, that provide insights into pathogenesis and potential targets for therapeutic intervention. PubMed, J Invest Dermatol, 09/14/2016.

Protagen and Inova Diagnostics Partner to Advance New Systemic Sclerosis Biomarker. The newly discovered anti-BICD2 autoantibodies are found in about 30% of patients with systemic sclerosis (SSc) and are highly associated with limited forms of the disease. Scleroderma News, 09/01/2016.

Increased risk of digital vascular events in scleroderma patients who have both anti–centromere (CENP) and anti–nterferon–inducible protein 16 (IF–16) antibodies. This study provides further evidence that anti–CENP and anti–IFI–16 antibodies are disease biomarkers that may be used for risk stratification of vascular events in scleroderma. PubMed, Arthritis Care Res (Hoboken), 07/07/2016. (Also see Antibodies in Systemic Scleroderma)

Biomarkers in systemic sclerosis (SSc): Their potential to predict clinical courses. Biomarkers that can predict clinical features, disease progress, therapeutic response and prognosis are heterogeneous among patients with systemic sclerosis. Wiley Online Library, 01/04/2016.

Biomarkers in rheumatic diseases: how can they facilitate diagnosis and assessment of disease activity? The ultimate goal of current and future biomarkers in rheumatic diseases is to enable early detection of these diseases and their clinical manifestations. PubMed, BMJ, 2015 Nov 26;351:h5079.

Decrease of LL-37 in systemic sclerosis (SSc): a new marker for interstitial lung disease (ILD)? The circulating levels of LL–37 in SSc patients were investigated as lower LL-37 levels may be associated with the development of ILD. PubMed, Clin Rheumatol, 01/20/2015. (Also see What is Pulmonary Fibrosis? and Pulmonary Fibrosis Biomarkers)

Cardiac biomarkers in systemic sclerosis: contribution of high-sensitivity cardiac troponin in addition to N-terminal pro-brain natriuretic peptide. The easily obtained biomarkers may be useful for systematic evaluation and stratification of SSc patients, especially to identify those at risk of pulmonary hypertension. PubMed, Arthritis Care Res (Hoboken), 01/20/2015. (Also see Scleroderma Cardiac Involvement)

Faecal levels of calprotectin (FC) in systemic sclerosis (SSc) are stable over time and are higher compared to primary Sjogren's syndrome and rheumatoid arthritis. FC could be a useful marker when novel, more specific drugs targeting the GI tract in SSc will be introduced. PubMed, Arthritis Res Ther, 2014 Feb 6.

Proteome-wide Analysis and CXCL4 as a Biomarker in Systemic Sclerosis. Levels of CXCL4 were elevated in patients with systemic sclerosis and correlated with the presence and progression of complications, such as lung fibrosis and pulmonary arterial hypertension. The New England Journal of Medicine, 12/18/2013. (Also see Pulmonary Hypertension and Pulmonary Fibrosis)

Abnormal cardiac enzymes in systemic sclerosis. Cardiac biomarkers such as troponin T (TnT) may be useful screening tools to identify subclinical cardiac disease and assess response to therapeutic intervention. Clinical Rheumatology, October 2013. (Also see Cardiac (Heart) Involvement)

Detection of hair-microRNAs as the novel potent biomarker: Evaluation of the usefulness for the diagnosis of scleroderma. Hairs are more accessible than sera among human samples. MicroRNAs levels in hair roots or hair shafts may become effective and independent biomarkers. Journal of Dermatological Science, 26 July 2013.

Fear of Progression

Validity of the Fear of Progression Questionnaire-Short Form in patients with systemic sclerosis. A valid measure is now available to assess fear of disease progression in patients with SSc, which is significant since fear of progression is one of the most important stressors in this patient population. PubMed, 2012 Jun;64(6):930-4.

Cardiac (Heart) Involvement

Epidemiology of Systemic Sclerosis (SSc). SSc-related myocardial disease death was 14% with most causes being related to arrhythmias. Curr Opin Rheumatol, 2012;24(2):165-170.

Causes of Death in Systemic Sclerosis

Mortality And Cause Of Death In An Australian Systemic Sclerosis Cohort. Pulmonary involvement in the form of PAH, ILD or both is the leading cause of death among patients in our cohort. Since cancer is the second leading cause of death in SSc, where possible, screening for early detection of malignancy should be considered. M. Nikpour. EULAR 2011 SAT 0449. Ann Rheum Dis 2011;70(Suppl3):668.

Life expectancy, standardized mortality ratios and causes of death of six rheumatic diseases in Hong Kong, China. Patients with rheumatic diseases such as lupus (SLE) and systemic sclerosis have increased mortality and reduced life expectancy. Infection is the leading cause of death, followed by cardiovascular complications and malignancies. PubMed, Arthritis & Rheumatism, 2011 Mar 9.

Infections, Intensive Care Unit

Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. PubMed, Clin Exp Rheumatol. (Also see Pulmonary Fibrosis)

Lung (Pulmonary) Involvement

Biomarkers in connective tissue disease-associated interstitial lung disease. Besides autoantibodies, an increase in serum or BALF of a biomarker of pulmonary origin may be able to predict or reflect the development of fibrosis, the impairment of lung function, and ideally also the prognosis. PubMed, Semin Respir Crit Care Med, 2014 Apr;35(2):181-200. (Also see Pulmonary Fibrosis Research)

Epidemiology and risk factors for pulmonary hypertension (PH) in systemic sclerosis (SSc). The ultimate objective in understanding the risk of developing PH in SSc is early diagnosis and early initiation of appropriate therapy with the hope for improved outcomes for patients with SSc-PH. PubMed, Curr Rheumatol Rep, 2013 Jan;15(1):302. (Also see Pulmonary Hypertension Prognosis)

Nailfold Indicators

Capillaroscopic Findings in Systemic Sclerosis — Are They Associated with Disease Duration and Presence of Digital Ulcers? The observation of an active phase scleroderma type pattern in patients without digital ulcers may therefore be used in the future as a predictor for the development of trophic changes. Discovery Medicine, 11/22/2011.

Prognostic Markers

Disease activity indices (DAI) in systemic sclerosis: a systematic literature review. Future studies are needed to fully validate the European Scleroderma Study Group activity index and the Combined Response Index for Systemic Sclerosis. PubMed, Clin Exp Rheumatol, 07/06/2016.

Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. Peak age at onset of systemic sclerosis (SSc) is between 20 and 50 years, although SSc is also described in both young and elderly patients. We conducted the present study to determine if age at disease onset modulates the clinical characteristics and outcome of SSc patients. PubMed, Medicine (Baltimore). 2014 Mar;93(2):73-81. Also see Prognosis and Mortality)

Risk factors for severity and manifestations in systemic sclerosis and prediction of disease course. This review discusses risk factors and markers that predict the disease course and the occurrence of disease manifestations, with an emphasis on major organ involvement. PubMed, Expert Rev Clin Immunol, 2015 Dec 2:1–21.

Frailty Index (FI) to Measure Health Status in People with Systemic Sclerosis. The FI quantifies overall health status in people with scleroderma and predicts mortality and it might help with decisions about who might best be served by more aggressive treatment, such as bone marrow transplantation. PubMed, J Rheumatol, 03/01/2014.

Dysregulation of angiogenic homeostasis in systemic sclerosis (SSc). Increased serum endostatin is associated with skin sclerosis severity and pulmonary fibrosis and favors SSc disease progression. PubMed, International Journal of Rheumatic Diseases, 2 Jul 2013. (Also see Common Medical Tests for Scleroderma and Pulmonary Fibrosis)


Predictors of Survival and Causes of Death in Japanese Patients with Systemic Sclerosis (SSc). The overall mortality rate of patients with SSc was higher than that of the general population, probably because of poor prognostic factors including organ involvement. These factors should be carefully monitored during followup. The Journal of Rheumatology. July 15, 2011. (Also see Causes of Scleroderma)

Skin Thickness Progression

Skin thickness progression rate (STPR): a predictor of mortality and early internal organ involvement in diffuse scleroderma. The STPR is an easy measure to perform at the time of initial evaluation for identifying those diffuse cutaneous systemic sclerosis (SSc) patients who are at increased risk of mortality and the development of renal crisis during the following 2 years. Ann Rheum Dis, 2011;70:104-109.

Symptoms of Systemic Sclerosis (Scleroderma)

Systemic Scleroderma Symptoms. Systemic sclerosis (scleroderma) can affect every part of the body, including the skin, blood vessels, and all of the internal organs. There are also dozens of associated conditions and related autoimmune diseases. ISN.

Autoimmune Diseases
Heart (Cardiac)
Kidney (Renal)
Lung (Pulmonary)
Photos of Symptoms
Similar Skin Diseases
Sjogren's Syndrome
Skeletal (Bones, Muscles)
Skin and Hair

Survival Rates

Scleroderma Prognosis. Many patients with systemic scleroderma experience a plateau in which the condition stabilizes. This plateau is followed by a period of improvement and skin softening. New York Times. 12/18/2012.

Also See

What is Scleroderma? ISN.

Types of Scleroderma ISN.

Go to Localized: Linear Scleroderma
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