Best practices in scleroderma: An analysis of practice variability in SSc centres within the Canadian Scleroderma Research Group (CSRG)

University of Western Ontario, London, ON, Canada.
Clinical and experimental rheumatology (Impact Factor: 2.72). 06/2012; 30(2 Suppl 71):S38-43.
Source: PubMed


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.

Full-text preview

Available from:
  • [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: We aimed to assess the prevalence of patients with either primary Sjögren's syndrome (pSS) and positive anticentromere antibodies (ACA) and secondary Sjögren's syndrome (sSS) and limited cutaneous ACA positive-systemic sclerosis (SSc) in two large cohorts of patients with pSS and SSc¸ and also to compare the clinical features of these two subsets with those of patients affected by 'ACA-positive SSc without sicca symptoms' and 'pSS'. Methods: In this retrospective monocentric study, the case records of 'overlap' patients fulfilling both the classification criteria for SS and the LeRoy criteria for early SSc were identified from two datasets of patients with limited cutaneous ACA positive SSc (209 subjects) and with pSS (402 subjects) who attended our Rheumatology Unit in the years between 1989 and 2011. Control groups were represented by SSc subjects without sicca symptoms ('SSc group') and ACA negative Pss patients ('pSS group'). SSc patients with sicca symptoms ('Sicca-SSc group') who did not complete the diagnostic algorithm for SS were excluded from the analysis. Demographic, clinical and immunological data of the patients enrolled were collected cumulatively over the entire follow up period. Statistical analysis was performed using SPSS 13 (SPSS Inc., Chicago IL, USA). Results: Out of the two datasets 41 'overlap' patients were selected. The control groups were represented by 102/209 SSc subjects without sicca symptoms ('SSc group') and 387/402 pSS patients ('pSS group'). Eighty-one 'sicca-SSc' with an incomplete work-up for SS were excluded from the analysis. The prevalence of ACA positive pSS patients among pSS was 3.7% (15/402), while the frequency of patients with definite sSS in the SSc cohort was 20% (26/128). No differences were detected between 'overlap' patients and control groups, relatively to demographic characteristics. 'Overlap patients' were characterised by a milder SSc disease (i.e. lower frequency of sclerodactily, negative evolution of the capillaroscopy pattern or absence of severe systemic involvement) whereas, as far as the SS-related manifestations were concerned, although often lacking in specific autoantibodies (i.e. rheumatoid factor, anti-Ro/SSA, anti-La/SSB), the 'overlap patients' displayed a full blown SS phenotype with recurrent salivary gland enlargement, purpura, fatigue, arthralgias, and leukocytopenia. It is noteworthy that the prevalence of non-Hodgkin's lymphoma in the 'overlap patients' was higher than in pSS. Conclusions: Taken together, the results of our work emphasise the existence of a novel distinct clinical entity which might tentatively be called 'ACA-positive limited scleroderma/SS overlap syndrome' characterised by a benign SSc clinical course but at a high risk of non-Hodgkin's lymphoma.
    No preview · Article · Jan 2013 · Clinical and experimental rheumatology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: Since the 1980 ACR classification criteria for systemic sclerosis (SSc) do not identify 20% with SSc, revised criteria are necessary. Methods: Suggested new criteria from the literature were sent in random order to 96 SSc experts. A 3-round Delphi Consensus eliminated criteria. Then cluster analysis reduced items. The Canadian Scleroderma Research Group (CSRG) database was used to determine the prevalence of each item. Results: Seventy-one of 96 (71%) completed all 3 rounds; 47 items were expanded to 76 in round 2. Thirty items had at least 50% consensus and 18 had >75% agreement to include (a priori cut point). Clustering occurred for 4 categories: proximal to MCP skin involvement, vascular abnormalities, autoantibodies and tissue damage. Proximal to MCPs skin involvement identified 80% of patients. Adding one item from each of the other 3 categories or 1 or more items from 2 of 3 remaining categories increased the proportion of patients classified to 94% in CSRG patients. Categories included (1) Vascular (dilated capillaries, telangiectasia, Raynaud's phenomenon [RP]), (2) Autoantibodies (anticentromere [ACA] or antitopoisomeraseI [Topo1]) and (3) Fibrosis/damage (esophogeal dysmotility dysphagia, sclerodactyly, digital ulcers). In the CSRG, 98% were identified if using proximal skin involvement; or sclerodactyly plus one of: RP, ACA or Topo1. Conclusions: This is a first step toward developing new SSc classification criteria. A Delphi exercise alone cannot suffice for item reduction. Also, validation prospectively in SSc patients and diseases that mimic SSc is needed in order to calculate sensitivity and specificity of future criteria.
    No preview · Article · Apr 2013 · Clinical and experimental rheumatology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Diffuse systemic sclerosis carries a high morbidity and mortality. The Prospective Registry of Early Systemic Sclerosis (PRESS), a multicentre incident cohort study of patients with early diffuse cutaneous systemic sclerosis, has the goal of advancing the understanding of disease pathogenesis and identifying novel biomarkers. In this review, PRESS investigators discuss the evidence pertaining to the more commonly used treatments for early diffuse SSc skin disease including methotrexate, mycophenolate, cyclophosphamide, azathioprine, and intravenous immunoglobulin. This review highlights the unmet need for effective treatment in early diffuse SSc as well as its more rigorous study. Nonetheless, the PRESS investigators aim to decrease intra- and inter-institutional variability in prescribing in order to improve the understanding of the clinical course of early diffuse SSc skin disease.
    No preview · Article · Aug 2013 · Clinical and experimental rheumatology
Show more