[2009] [SP0084] TREATMENT OF SYSTEMIC SCLEROSIS – STRIKE WHILE THE IRON IS HOT

A. Tyndall Dept of Rheumatology, University of Basle, Basle, Switzerland

Until recent years a rather nihilistic attitude toward the treatment of systemic sclerosis (SSc) prevailed, partly reality based and partly due to a failure to recognise early and less severe cases. In the early 1980's the ACE inhibitors changed the outcome of renal crisis, and more recently effective agents against the vascular aspects of SSc i.e. anti endothelin receptor e.g. bosentan, and modulators of smooth muscle cyclic AMP e.g. prostanoids and GMP e.g. phosphodiesterase 5 inhibitors) plus more structured intense immunosuppression stategies have given new hope.
Currently a pathogenesis paradigm exists of a sequential process in which vascular disturbance leads to inflammatory/immunological overactivity resulting in fibroblasts differentiating into contractile collagen secreting myofibroblasts. Treatment has been directed at each of these phases assuming (perhaps wrongly) that it is a temporal continuum. ''Early'' disease with just Raynauds and sclerodactily would receive only calcium channel blockers, with intense immunosuppression with cyclophosphamide being reserved for more ''advanced'' cases. This may be as wrong as treating all '' early '' breast cancer the same, independent of histological grade, stage and receptor status.
Two things are evolving to change this in SSc. The first is a more evidence based attempt to recognise and treat early SSc – the Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) project under the auspices of the EULAR Scleroderma Trials and Research (EUSTAR) group. Early prognosis prediction is a major aim. The second is a realisation that in patients who have undergone immunoablation for SSc via autologous stem cell transplantation, remodelling of miscrovasculature and regression of fibrosis have been observed. This suggest ''resetting'' of a dysfunctional network of cells and mediators, since endothelial cells and fibroblasts alone are little influenced by immunoablation, giving hope that early and appropriate intervention may reverse the chronification of the process in SSc leading to end organ fibrosis and failure.
These concepts as well as the recently developed evidence based EULAR recommendations on the treatment of SSc will be presented.
Disclosure of Interest: None declared

Ann Rheum Dis 2009;68(Suppl3):27

New trends in systemic sclerosis