2018 Research Program

1. Prospective validation of the ASIG PAH screening algorithm

One of ASIG’s key activities is the Australian Scleroderma Screening Program for early identification of heart and lung complications, such as pulmonary arterial hypertension (PAH).

We have developed and validated a novel, cost-effective screening algorithm for PAH, using NT proBNP blood levels. It’s advantage is that it is independent (but does not exclude) of the transthoracic echocardiogram which can under-estimate the systolic pulmonary arterial pressure used in traditional screening to identify patients with PAH.

Prospective validation of this algorithm is required for it to become widely used for Australian scleroderma patients. Patients who did not have PAH at the time of the original studies are being followed to determine whether they have developed PAH at a later time.

Focus will be on assessment of NT proBNP levels taken every year and whether these patients fulfilled the algorithm in order to define the negative predictive value (ie can the algorithm be used to discount the possibility of PAH being present?).

In addition, data continue to be gathered for development of a “biomarker-only” screening tool (using NT-proBNP and another blood test, ADMA) at the same time as the prospective validation study using a prospective cohort design. The ‘gold standard’ against which the predictive accuracy of these tools is assessed will be a composite of measures from the transthoracic echocardiogram and right heart catheterisation.

2. Developing a multi-disciplinary care model for a complex multi-organ disease

Due to the complex multifaceted nature of SSc, only a multidimensional approach to patient care and research, delivered by a multidisciplinary team, has the potential to have a profound impact on disease burden and HRQoL.

The current model of care for SSc patients can be inefficient, with patients attending multiple clinic appointments on different days to have a range of specialist opinions and management of a single disease manifestation such as DU.

Outcomes could be improved with a more streamlined multidisciplinary care model (MDM).

Australia, having one of the highest reported prevalence of SSc worldwide, is the prime country to develop, evaluate and implement such a MDM in SSc.

This exemplary model can in turn serve as a framework for development of similar models in other complex multi-organ chronic diseases.

The hypothesis of this study is that the development and implementation of a MDM incorporating a range of medical specialists, nurse practitioners and allied health professionals will reduce the associated healthcare burden and cost, including hospitalisations, and emergency department and ambulatory care visits.

Furthermore, we anticipate such a model of care will lead to improved patient reported outcome measures such as health-related quality of life and patient satisfaction with the healthcare system, compared with the current model of care.