Improving life expectancy of patients with scleroderma: results from the South Australian Scleroderma Register

  title={Improving life expectancy of patients with scleroderma: results from the South Australian Scleroderma Register},
  author={Nicholas Kennedy and Jenny G Walker and Paul Hakendorf and Peter Roberts-Thomson},
  journal={Internal Medicine Journal},
BACKGROUND Scleroderma is a rare connective tissue disorder characterised by inflammation, vasculopathy and excessive fibrosis. [] Key MethodMETHODS Utilising the South Australian Scleroderma Register, deceased patients were identified. We examined changes in age of death and duration of disease in these patients over three time periods: 1985-1994, 1995-2004 and 2005-2015. Analyses of scleroderma subtypes were performed, and comparisons were made to the general South Australian population.

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Outcomes in people with scleroderma hospitalized with serious infections have improved over time, except higher hospital charges, and over the study period, hospital charges increased, while length of hospital stay and in-hospital mortality decreased, overall and for each serious infection.

Systemic sclerosis is associated with knee arthroplasty outcomes: a National US study

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Outcomes in people with SSc hospitalized with serious infections have improved over time, except higher hospital charges, and multivariable-adjusted analyses showed that sepsis, age, and Deyo-Charlson score were associated with significantly higher odds of healthcare utilization and in-hospital mortality.

Assessment of Toxic Effects Associated With Dose-Fractionated Radiotherapy Among Patients With Cancer and Comorbid Collagen Vascular Disease

The receipt of hypofractionated radiotherapy was associated with a lower likelihood of developing late toxic effects in the multivariable analysis, and the incidence of severe toxic effects among patients with cancer and comorbid collagen vascular disease was similar across several dose-fractionation radiotherapy regimens.



Survival in scleroderma: results from the population‐based South Australian Register

Aim:  To ascertain the mortality risk and investigate clinical and serological factors influencing survival of patients listed on the South Australian Scleroderma Register (SASR).

South Australian Scleroderma Register: autoantibodies as predictive biomarkers of phenotype and outcome

Aim:  To investigate the relationship between scleroderma‐specific autoantibodies and clinical phenotype and survival in South Australian patients with scleroderma.

Incidence of cancer among patients with systemic sclerosis

A population‐based retrospective cohort study was performed to determine cancer risk among patients with systemic sclerosis and localized scleroderma and found that smoking and alcohol use among these patients increased the risk of cancer.

Interpretation of an Extended Autoantibody Profile in a Well‐Characterized Australian Systemic Sclerosis (Scleroderma) Cohort Using Principal Components Analysis

To determine the relationships between systemic sclerosis (SSc)–related autoantibodies, as well as their clinical associations, in a well‐characterized Australian patient cohort, a large number of patients were diagnosed with SSc.

Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database

A database to prospectively gather key data of patients with SSc using a minimal essential dataset provides an abundance of information on the true clinical face of SSc that will be helpful in improving the classification of S Sc and its subsets and for developing more specific therapeutic recommendations.

Changes in causes of death in systemic sclerosis, 1972–2002

The change in the pattern of scleroderma-related deaths over the past 30 years demonstrates that the lung (both pulmonary hypertension and PF) is the primary cause of sclerosis- related deaths today.

Predictors of Survival and Causes of Death in Japanese Patients with Systemic Sclerosis

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, and these factors should be carefully monitored during followup.

Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors.

Improved survival and successful discontinuation of dialysis are possible when ACE inhibitors are used to treat scleroderma renal crisis and patients with systemic sclerosis who develop hypertension should be treated with an ACE inhibitor.

The Effect of Male Sex on Survival in Systemic Sclerosis

The differential effect of disease between sexes is small, yet males have decreased survival compared to females with SSc, and a relative survival analysis compared SSc survival to the general population.

Sildenafil for pulmonary arterial hypertension associated with connective tissue disease.

In patients with PAH-CTD, sildenafil improves exercise capacity, hemodynamic measures, and functional class after 12 weeks of treatment.