Newswise — Even without any one medication proven to successfully treat their disease, people with scleroderma are living longer lives due to improved monitoring and treatment, according to research presented this week at the American College of Rheumatology Annual Scientific Meeting in Philadelphia, Pa.
Scleroderma (more formally called systemic sclerosis) is a disease affecting the skin and other organs that is one of the autoimmune rheumatic diseases. Scleroderma results in the thickening and tightening of skin, as well as a build-up of scar tissue and damage to internal body organs. Effective treatments are available for some forms of the disease, although scleroderma is not yet curable.
Researchers in the United Kingdom recently compared the medical information of two groups of patients with systemic sclerosis to determine if patients were experiencing fewer complications with their major organs due to the disease, and to determine if patients were living longer lives with the disease.
Researchers followed 234 patients who were diagnosed between 1990 and 1993, and 286 patients who were diagnosed between 2000 and 2003. While comparing five years of medical records of the two groups, researchers found a greater life expectancy among the patients who had diffuse cutaneous systemic sclerosis—a type of systemic sclerosis that causes the skin to thicken all over the body, rather than just on the forearms, hands, legs, feet, and face as it does in limited cutaneous systemic sclerosis—from the group of patients diagnosed between 2000 and 2003, with an estimated 85 percent likelihood of surviving five-years after diagnosis (compared to 69 percent in the group diagnosed between 1990 and 1993). However, there was no significant improvement in the patients with limited cutaneous systemic sclerosis.
Researchers did find that the number of patients who developed kidney and heart problems had not changed over the years.
“Our research confirms that current management with regular screening for organ complications is leading to earlier detection and treatment of clinically significant scleroderma-related lung disease,” explains Christopher P. Denton, PhD, FRCP; professor of experimental rheumatology; Centre for Rheumatology, division of medicine, UCL Medical School, Royal Free Hospital, London, and an investigator in the study. “More importantly, this is associated with improved survival in the diffuse cutaneous subset of systemic sclerosis – the worst form of the disease. Longer patient follow up may eventually demonstrate a similar survival benefit among people with the limited cutaneous subset of the disease, but our study cannot confirm this.”
The ACR is an organization of and for physicians, health professionals, and scientists that advances rheumatology through programs of education, research, advocacy and practice support that foster excellence in the care of people with or at risk for arthritis and rheumatic and musculoskeletal diseases. For more information on the ACR’s annual meeting, see www.rheumatology.org/annual.
Editor’s Notes: Svetlana I. Nihtyanova, MBBS will present this study during the ACR Annual Scientific Meeting at the Pennsylvania Convention at 2:45 PM on Sunday, October 18 at 2:45 PM in the Auditorium.
Presentation Number: 604
Improving Survival Despite Greater Disease Burden in Systemic Sclerosis
Svetlana I. Nihtyanova, MBBS , Center for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, London, United Kingdom
Edward C. Tang , Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, London, United Kingdom Carol M. Black, FRCP , Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College Medical School, London, United Kingdom Christopher P. Denton, FRCP, PhD , Centre For Rheumatology and Connective Tissue Diseases, UCL Medical School, London, United Kingdom
Purpose: To determine whether the ascertainment of major organ complications of systemic sclerosis (SSc) has changed over time and if this is associated with better 5 year survival in two groups of cases seen a decade apart at a single tertiary SSc centre.
Methods: Subjects under regular review at our centre with a definite diagnosis of SSc were identified. We analysed patients with disease onset between years 1990 and 1993 (historical cohort) and patients with disease onset between 2000 and 2003 (contemporary cohort). Case notes were reviewed and demographic and clinical data were recorded. Kaplan-Meier estimates of survival and cumulative frequency of moderate to severe organ-based complications in the different subgroups observed over the first 5 years from disease onset were compared.
Results: A total of 520 SSc patients (234 in the historical cohort and 286 in the contemporary cohort) were included. As there was a significant difference between the two cohorts in terms of subset distribution, with the contemporary cohort having larger proportion of patients with the diffuse cutaneous (dc) subset compared to the historical cohort (45% v 32%, p=0.002), survival and frequency of organ complications were compared separately for the limited cutaneous (lc) SSc and dcSSc subjects. There were no significant differences between the historical and contemporary cohorts in terms of gender, age at onset or proportion of patients lost to follow-up.
We found reduction in all cause mortality among the dcSSc patients from the contemporary cohort with estimated 5-year survival probability of 85% compared to 69% for the historical cohort (p=0.012). There was no significant improvement in survival among the lcSSc patients (92% for the 2000-03 group and 91% for the 1990-93 group, p=0.639).
At 5 years from disease onset, a higher proportion of the patients from the contemporary cohort had a diagnosis of clinically significant pulmonary fibrosis (17% of the lcSSc and 39% of the dcSSc subjects) compared to 3% and 7% (limited and diffuse subset respectively) of the historical cohort, p<0.001. Similarly, the diagnosis of pulmonary arterial hypertension was more frequent among the patients from the contemporary cohort compared to those from the historical cohort over the first 5 years of disease (8% v 1%, p=0.002 for lcSSc and 7% v 1%, p=0.132 for dcSSc). Analysis of the proportion of patients who developed scleroderma renal crisis (SRC) or cardiac scleroderma during the first five years of follow-up showed no significant difference between the historical and the contemporary cohorts. In particular among the dcSSc subjects, the cumulative incidence of SRC was 16% and 19% for the 2000-03 and 1990-93 cohorts respectively, p=0.815.
Conclusions: Despite the absence of proven disease modifying therapy, survival has substantially improved for dcSSc. There is also more complete ascertainment of lung complications of SSc, probably through greater awareness and more systematic annual screening for organ based complications.
Disclosure: S. I. Nihtyanova, None; E. C. Tang, None; C. M. Black, None; C. P. Denton, None.
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