| What is Scleroderma? (MAIN MENU) |
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| Types of Scleroderma: Overview |
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Systemic Sclerosis: Prognosis and Mortality |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Overview |
| There are two main types of scleroderma: Localized and Systemic. |
| The localized forms, such as morphea and linear primarily affect the skin and are never fatal. |
| However, the systemic forms -- diffuse, limited, CREST, and overlap -- can any part of the body (skin, blood vessels, and internal organs) and are frequently associated with a decreased lifespan. |
| 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. ISN. |
| 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. |
| Clinical Prediction of 5-Year Survival in Early Scleroderma: Validation of a Simple Prognostic Model in a Multi-Centre Study. A simple prognostic model using 3 disease factors to predict 5 year survival at diagnosis in SSc showed reasonable discriminatory performance upon validation in a European multi-centre study. Update of the formula may increase discrimination and precision of the predictions. J. Fransen. (FRI0319) EULAR 2009. |
| Antibodies |
| Systemic Sclerosis Patients Have Activating Antibodies Targeting Both Endothelin Receptor Type A And Angiotensin Ii Type 1 Receptor Predicting Worse Prognosis. Anti-AT1R and anti-ETAR antibodies are a biomarker for severe disease and worse prognosis and could explain pathogenic features found in systemic sclerosis. The detection of these antibodies could identify SSc patients that might benefit from a receptor blockade or from a specific modulation of the antibody-receptor interaction. G. Riemekasten OP0162 EULAR 2007. (Also see: Causes of Scleroderma: Endothelin and Antibodies) |
| 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 |
| Causes and Risk Factors for Death in Systemic Sclerosis - A Study from the EULAR Scleroderma Trials and Research (EUSTAR) Database. Pulmonary fibrosis and isolated PAH are pre-eminent causes of death in SSc. In addition, causes of death such as septicaemia and pneumonia could be directly related to SSc-associate comorbidity in many cases. Other independent predictors of death included DLCO, pulmonary hypertension, modified Rodnan skin score, age at Raynaud's onset, dyspnoea and proteinuria. A.J. Tyndall. (OP-0078) EULAR 2009. (Also see: Prognosis and Mortality) |
| Cardiac Involvement in Systemic Sclerosis: The Strongest Predictive Factor of Prognosis in Patients with Scleroderma. The results disclosed that most frequent cardiac manifestation at the initial evaluation of scleroderma is subclinical arrhythmia. More importantly, cardiac involvement at early stage of the disease is the strongest predictive factor for death. Sumiaki Tanaka. 13/13. ACR 2007. (Also see: Cardiac Involvement) |
| Causes of Death in Systemic Sclerosis |
| Survival, Causes of Death, and Risk Factors Associated With Mortality in Spanish Systemic Sclerosis Patients: Results From a Single University Hospital. Ten-year survival is over 70% in Spanish SSc patients. The main causes of death are lung and cardiac involvement, and to a lesser extent, peripheral vascular disease and coexisting malignancy. Diffuse subset, proteinuria, PH, elevated erythrocyte sedimentation rate and older age at diagnosis are the main risk factors for mortality. Beatriz E. Joven, MD Seminars in Arthritis and Rheumatism. 27 September 2009. |
| Changes in causes of death in systemic sclerosis, 1972- 2002. Survival of scleroderma has changed since the treatment of renal crisis became possible. The change in pattern of scleroderma- related mortality over the past 30 years implicates the lung (both pulmonary hypertension and pulmonary fibrosis) as the primary causes of scleroderma related deaths today. PubMed. Ann Rheum Dis. 2007 Feb 28. (Also see: Pulmonary Involvement and Renal Involvement) |
| Histological |
| Mortality And Histological Characteristics Of SSc - A Retrospective Study Of 12 Autopsy Patients. SSc is a progressive multifocal process characterized by histological (vascular and interstitial) changes co-existing in different stages of their progression. In the course of the disease new foci develop, which increase in size and number, may become confluent, ultimately leading to diffuse, systemic interstitial sclerosis. Á. Apáthy THU0235 EULAR 2007. |
| 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. 2006 Jul-Aug;24(4):380-6. (Also see: Pulmonary Fibrosis) |
| Lung (Pulmonary) Involvement |
| Risk factors for death and the 3-year survival of patients with systemic sclerosis (SSc): the French ItinérAIR-Sclérodermie study. This 3-year study observed survival and mortality estimates that were comparable with previous reports. Pulmonary artirial hypertension increased the Hazard Ratio for mortality in patients with SSc, justifying yearly echocardiographic screening. Eric Hachulla. Rheumatology 2009 48(3):304-30. January 27, 2009. (Also see: Pulmonary Hypertension) |
| Risk Factors For Mortality In Patients With Systemic Sclerosis (SSc) And Interstitial lung disease (ILD). Mortality in patients with SSc and ILD is increased in those with an early and severe impairment of pulmonary function, concomitant cardiac involvement and elevated ESR (Sed rate), but not in Scl-70 positive patients. Beatriz E. Joven. 6/6. ACR 2007. (Also see: Pulmonary Fibrosis) |
| Nailfold Indicators |
| Scleroderma patients nailfold videocapillaroscopic patterns are associated with disease subset and disease severity. Nailfold videocapillaroscopy, a simple, non-invasive and non-expensive investigation, is useful in staging scleroderma patients and also provides prognostic information. Rheumatology 2007 46(10):1566-1569. (Also see: Nailfold Capillaroscopy) |
| Prognostic Markers |
| Myopathy Independently Predicts Mortality in Scleroderma Patients. Myopathy in scleroderma is significantly associated with diffuse skin subtype and lung disease and independently predicts mortality. Early recognition of muscle disease may identify scleroderma patients at high risk for poor outcomes and provide an opportunity for therapeutic intervention. Valiyil, Ritu, Hummers, Laura K., Wigley, Fredrick M.; Arthritis Rheum 2009;60 Suppl 10 :1737. (Also see: Myopathy) |
| Prognostic markers for systemic sclerosis. The prognosis of systemic sclerosis depends chiefly on the extent of the skin lesions, which correlates with the severity of the cardiovascular, pulmonary, and renal manifestations. PubMed. Joint Bone Spine. 2006 Oct;73(5):490-4. |
| Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study. We conclude that a diagnosis of SSc at an older age appears to be a poor prognostic indicator related to both disease severity and comorbidities. A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality. PubMed. Clin Rheumatol. 2006 Nov;25(6):831-4. |
| Predictive markers for development of severe organ involvement in patients with systemic sclerosis (SSc). Prognosis of SSc is associated with the extent of skin involvement and the presence of lung, heart, kidney, and/or digestive tract damage. To avoid irreversible tissue injury, early detection of visceral involvement is crucial for prompt initiation of therapy. PubMed. Ann N Y Acad Sci. 2005 Jun;1051:455-64. |
| Changes in Causes of Death in Systemic Sclerosis Over the Past 30 Years. Throughout the past 30 years, the frequency of deaths from RC (renal crisis) has dramatically decreased and at the same time the frequency of PF (pulmonary fibrosis) increased. However, only 10% of SSc patients surviving RC have died of PF, which may be because patients with the highest frequency of RC have a low frequency of PF. Over the past 10 years, SSc patients have had improved survival, longer disease duration at the time they die and are less likely to die from scleroderma related complications. Pulmonary hypertension and pulmonary fibrosis now account for 50% of SSc related deaths and 25% of all causes of death in SSc patients. Virginia Steen. 1052/432. ACR 2004. (Also see: Renal Involvement, Pulmonary Fibrosis, and Pulmonary Hypertension.) |
| Race/Ethnicity |
| Impairment of the antifibrotic effect of hepatocyte growth factor (HGF) in lung fibroblasts from African Americans: Possible role in systemic sclerosis. Reduced levels of HGF as well as a deficiency in c-Met receptor function appear to be present in African American patients with SSc. These findings may explain in part the greater disease severity and worse prognosis observed in African Americans with SSc. Arthritis and Rheumatism. Volume 56, Issue 7, Pages 2432 - 2442. (Also see: Pulmonary Fibrosis) |
| 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. |
