Best practices in scleroderma: an analysis of practice variability in SSc centres within the Canadian Scleroderma...

There is currently no consensus on best practice in systemic sclerosis (SSc). To determine if variability in treatment and investigations exists, practices among Canadian Sclerodermia Research Group (CSRG) centres were compared.

Prospective clinical and demographic data from adult SSc patients are collected annually from 15 CSRG treatment centres. Laboratory parameters, self-reported socio-demographic questionnaires, current and past medications and disease outcome measures are recorded. For centres with >50 patients enrolled, treatment practices were analysed to determine practice variability.

Data from 640 of 938 patients within the CSRG database met inclusion criteria, where 87.3% were female, the mean ± SEM age was 55.3±0.5, 48.9% had limited SSc and 47.8% had diffuse SSc (and 3.3% uncharacterised). Some investigation and treatment practices were inconsistent among 6 centres including proportion receiving: PDE5 (phosphodiesterase type 5) inhibitors for Raynaud's phenomenon (p=0.036); cyclophosphamide (p=0.037) and azathioprine (p=0.037) for treatment of ILD; and current use of D-penicillamine, although uncommon, varied among sites. Annual echocardiograms and PFTs were frequently done and did not vary among sites but the rate of pulmonary arterial hypertension (PAH) was directly related to site size and this was not the case for other organ involvement.

Despite routine tests within a database, site variation in SSc with respect to investigations and management among CSRG centres exists suggesting a need for a standardised approach to the investigation and treatment of SSc. One can speculate that larger centres are more export in detecting PAH.