ASCIA‐P73: THE IMPROVING SURVIVIAL OF PATIENTS WITH SCLERODERMA OVER A 30 YEAR PERIOD

Abstract

To investigate life expectancy and duration of disease in a large cohort of patients with scleroderma over a 30 year period. The South Australian Scleroderma Register (SASR) is a population based register that has sought to identify all patients with Scleroderma in South Australia (SA). We examined the duration of disease and age of death over 3 time periods 1985–94, 1995–2004 and 2005–2014. Analyses of scleroderma subtypes were performed and comparisons were made with the SA population. 413 deceased patients were identified. 315 patients were female, 98 male. Mean and median duration of disease was 18.7 and 16 years. Mean and median age of death was 71.6 and 74 years. Those with limited disease had significantly better survival than those with diffuse or overlap disease. (p < 0.001) Over 30 years the mean age of death improved from 66.4 years to 74.5 years. (p < 0.001) Average duration of disease improved from 12.4 to 22.7 years. This improvements in survival was seen in limited (p = 0.001), diffuse (p = 0.04) and overlap (p = 0.04) subgroups. Patients with limited disease now have a median age of death rivaling that of the general SA population (79 vs 83 years). This study shows that over the last 30 years scleroderma survival has significantly improved. As there remains no disease modifying drugs that have consistently been shown to alter disease course, this improvement is likely attributable to general improvements in medical care including that of scleroderma related complications such as scleroderma renal crisis and pulmonary hypertension. While the life expectancy for limited disease is now close to that of the general population, patients with diffuse and overlap disease continue to suffer from significant early mortality. This improvement in patient survival does not highlight the considerable morbidity scleroderma continues to cause.