[Skip to Content]
Navigation
Home > Medical Directory > What is Scleroderma? > Types of Scleroderma > Systemic

Systemic Sclerosis: Prognosis and Mortality

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview of Prognosis and Mortality
Antibodies
Biomarkers
Fear of Progression
Cardiac Involvement
Causes of Death
Lung Involvement
Nailfold Indicators
Prognostic Markers
Race/Ethnicity
Skin Thickness Progression
Symptoms of Systemic Scleroderma
Survival Rates

Overview of Prognosis and Mortality in Systemic Sclerosis (Scleroderma)

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.

Antibodies

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.

Biomarkers

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.
Northwestern to explore personalized medicine for scleroderma. Northwestern is one of four sites to receive funding through the new NIH grants. Northwestern will serve as the lead institution for proteomics research and will explore biomarkers at the protein level. EurekaAlert, 12/01/2011.
Fecal calprotectin: A biomarker of gastrointestinal disease in systemic sclerosis. Fecal calprotectin is increased in a majority of patients with systemic sclerosis (SSc). It correlates with objective and clinically important features of GI disease, and fecal concentrations do not vary with plasma concentrations. Intern Med 2011; 270: 50-57 (Also see: Scleroderma Gastrointestinal Involvement)
Biomarkers for skin involvement and fibrotic activity in scleroderma. This review summarizes all non-invasive physical and laboratory examinations, which permit a better understanding of the fibrotic activity of the disease, can be effectively used to assess potential therapeutic response and help to find better treatment options. PubMed, J Eur Acad Dermatol Venereol. 2011 Aug 8. (Also see: Skin Fibrosis)
Clinical prediction of 5-year survival in systemic sclerosis (SSc): validation of a simple prognostic model in EUSTAR centres A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study. Ann Rheum Dis. July 2011.
Increased Serum Comp Predicts Mortality But Is Not Related To Interstitial Lung Disease In Systemic Sclerosis (SSc). Increased serum COMP predicts mortality in SSc. As biomarker in SSc, serum COMP is useful in the assessment of skin but not of pulmonary involvement. EULAR 2011 OPO193. Ann Rheum Dis 2011;70(Suppl3):134.

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

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)
HLA Markers for Poor Prognosis in Systemic Sclerosis Brazilian Patients. This study evaluated human leukocyte antigen (HLA) involvement in the disease expression and poor prognostic clinical features (pulmonary fibrosis and pulmonary arterial hypertension) in patients diagnosed with systemic sclerosis (SSc). Hindawi, Disease Markers Volume 35 (2013), Issue 2, Pages 73–78.

Race/Ethnicity

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.
Associated Conditions
Brain
Dental/Mouth
Eyes
Fatigue
Gastrointestinal
Heart (Cardiac)
Kidney (Renal)
Liver
Lung (Pulmonary)
Photos of Symptoms
Pregnancy
Raynaud's
Similar Skin Diseases
Sjogren's Syndrome
Skeletal (Bones, Muscles)
Skin and Hair
Vascular

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.

See Also

What is Scleroderma? ISN.
Types of Scleroderma ISN.
 
Go to Localized: Linear Scleroderma
 

SCLERO.ORG is operated by the International Scleroderma Network, which is a full-service U.S. nonprofit 501(c)(3) established in 2002. We provide stellar worldwide research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. Donate or Shop Now.

Questions? Post a message in Sclero Forums or email us directly at isn@sclero.org. Or call our Scleroderma Hotline (English only), Toll Free in U.S. 1-800-564-7099 or Direct at 1-952-831-3091. Ask for our Welcoming Email.

Our headquarters postal mailing address is: International Scleroderma Network (ISN), 7455 France Ave So #266, Edina, MN 55435-4702, United States.

The most important thing in the world to know about scleroderma...is sclero.org!
Home   Medical   News   Sclero Forums   Support   Translations  Donate or Shop
Copyright 1998-2014, International Scleroderma Network. AKA Scleroderma from A to Z and SCLERO.ORG. All Rights Reserved.