Scleroderma Australia

Project: Assessing early pulmonary hypertension in scleroderma

Investigators: A/Prof David Prior & Dr Wendy Stevens

As previously reported, the aims of this project were to develop a new non-invasive method for assessment of early changes in the pulmonary vasculature in patients with scleroderma at risk of pulmonary arterial hypertension (PAH). The Scleroderma Australia / Arthritis Australia project grant allowed us to commence this project in 2009 by providing funding for consumables for performing the low dose dobutamine stress echocardiograms, test strips to measure B-type natriuretic peptide) BNP levels and a part-time study nurse to perform 6 minute walk tests.

The initial support from Scleroderma Australia / Arthritis Australia allowed us to obtain a project grant from the Heart Foundation to allow us to study not just the baseline, cross-sectional findings in the study subjects, but also to study how stable the new measures of pulmonary vascular function are over a 2 year period with repeated testing.

Thus far we have recruited 54 patients with scleroderma (48 without known PAH and 6 with known PAH on therapy) and 6 normal control subjects. This represents an additional 15 subjects since the last report, in addition to performing yearly follow-up testing on most participants. We continue to both recruit new subjects to reach our target numbers of 80 patients with Scleroderma and 20 control subjects and to continue follow-up testing in some of the participants to test the stability of our new index. Drs Jennifer Coller, Alex Mclellan and Ben Pang have assisted with patient recruitment, stress test supervision and data analysis over the duration of the study.

Because we are continuing to recruit, we have delayed group statistical analysis of the baseline study findings until we complete recruitment. For organisational reasons, we will need to stop further enrolment during 2012 even if we do not meet the targets we have in our protocol and we can then proceed with analysis.
For this reason, there have been no publications or presentations to date.