Systemic Sclerosis: Prognosis and Mortality |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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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) |
| 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, 09/07/11. |
| The Association Between Disease Activity and Duration in Systemic Sclerosis. Disease activity declined with time and patients with diffuse disease had consistently higher SDAI ( Valentini Scleroderma Disease Activity Index) scores. Depression was found to be associated with higher patient activity scores and strongly associated with patient self-response questions. Jennifer G. Walker Journal of Rheumatology, September 1, 2010 (Also see: Depression) |
| Evidence-based management of rapidly progressing systemic sclerosis. Systemic sclerosis has the highest case-specific mortality of any of the auto-immune rheumatic diseases, as well as causing major morbidity. However, this situation is changing. D. Khanna. Best Practice & Research: Clinical Rheumatology. Vol 24, Issue 3, Pp 387-400 (June 2010). |
| Age-related survival and clinical features in systemic sclerosis (SSc) patients older or younger than 65 at diagnosis. In elderly patients, the lSSc subset is more prevalent than the diffuse. Pulmonary and cardiac involvement are more prevalent in aged patients and appears sooner after the disease diagnosis. SSc is clearly related to increased mortality, although it is not significant in the elderly group. Carmen Pérez-Bocanegra. Rheumatology, March 11, 2010. |
| Greater Awareness and Earlier Screening Improve Outcomes for Scleroderma Despite a Lack of Proven Medications, Patients Are Living Longer Lives. Researchers followed 234 patients who were diagnosed between 1990 and 1993, and 286 patients who were diagnosed between 2000 and 2003. Newswise. 10/05/09. |
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 |
| 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, 12/01/11. |
| 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. K Andréasson, 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. Moinzadeh P. (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. J Fransen, 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. R. Hesselstrand. EULAR 2011 OPO193. Ann Rheum Dis 2011;70(Suppl3):134. |
| Biomarkers in the Management of Scleroderma: An Update. Many biomarker candidates have been identified in the past two decades; however, fully validated measures are still lacking with regard to aiding in the early diagnosis and reflecting the disease activity, severity, prognosis, and response to therapy. Abignano G. (PubMed) Curr Rheumatol Rep. 2010 Nov 3. |
| Altered immune phenotype in peripheral blood cells of patients with scleroderma-associated pulmonary hypertension. Our goal is to identify differentially expressed genes in peripheral blood mononuclear cells in scleroderma patients with and without pulmonary hypertension as biomarkers of disease. Ribano MG., (PubMed) Clin Transl Sci. 2010 Oct;3(5):210-8. |
| Biological markers useful in the diagnostic of autoimmune status. Biological markers used to assess autoimmune condition can provide useful information on physio-pathological mechanisms of self-aggression installation during various chronic or acute diseases. Vlad C. (PubMed) Bacteriol Virusol Parazitol Epidemiol. 2010 Jan-Mar;55(1):51-4. |
Fear of Progression |
| Fear of Progression: Forms of Fear-Expressions in Patients with Rheumatic Diseases. Fear of Progression is a high relevant problem for patients with rheumatic diseases, can differentiated in a variety of fear-expressions in intensity, forms, contents and can be reduced by individual learned coping-strategies. U. Engst-Hastreiter SAT0493-AHP EULAR 2008. (Also see: Emotional Adjustment) |
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. C. Mok, Arthritis & Rheumatism (PubMed) 2011 Mar 9. |
| Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc. Tyndall AJ. (PubMed) Ann Rheum Dis. 2010 Jun 15. (Also see: Pulmonary and Cardiac) |
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 |
| 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 |
| Disability in Systemic Sclerosis (SSc) - A Longitudinal Observational Study. Our study provides strong evidence that SSc causes increased disability over time, with breathing problems and disease type being the strongest predictors of disability. Statistical modeling accounting for informative patient dropout is necessary to properly assess the outcomes of patients followed longitudinally. Mireille Schnitzer, Journal of Rheumatology, December 15, 2010. (Also see: Disability) |
| Assessing microvascular changes in systemic sclerosis diagnosis and management. Microvascular damage and dysfunction represent the earliest morphological and functional markers of systemic sclerosis (SSc), a progressive connective tissue disease characterized by vascular abnormalities and diffuse fibrosis in the skin and internal organs. M Cutolo. (PubMed) Nat Rev Rheumatol. 2010 Aug 10. (Also see: Vascular Involvement) |
| Joint Involvement Predicts Aggressive Systemic Sclerosis. Synovitis, joint contractures, and tendon friction rubs more prevalent in patients with the diffuse cutaneous subtype noted Dr. Avouac of Cochin Hospital, Paris. Bruce Jancin. Skin & Allergy News. 08/30/10. (Also see: Joint and Hand Involvement) |
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. Atsushi Hashimoto. The Journal of Rheumatology. July 15, 2011. (Also see: Causes of Scleroderma) |
Skin Thickness Progression |
| Skin Thickness Progression Rate (STPR) in Systemic Sclerosis with Diffuse Cutaneous Involvement: A Predictor of Outcome. Rapid STPR at first evaluation in early dcSSc patients is a predictor of both internal organ involvement at one year after onset of skin thickening and 5 year mortality. Assessment of individual risk in dcSSc patients and planning of clinical trials involving these patients should include evaluation of STPR. (Also see: Diffuse SSc, and Skin Fibrosis). |
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. |
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Survival Rates |
| Scleroderma Outlook Improves as Survival Increases. Professor Virginia Steen, M.D., studied 2,000 patients with scleroderma (also known as systemic sclerosis) treated between 1972 and 2001 at the University of Pittsburgh and found that 10-year survival steadily improved over those years by 12 percent-- from 54 percent to 66 percent. Georgetown University Medical Center. 07/10/07. |
See Also |
| What is Scleroderma? ISN. |
| Types of Scleroderma ISN. |
