Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement Full Text
Arthritis Research & Therapy, 08/20/2012
Valentini G et al. – Most early Systemic Sclerosis (SSc) but only a few Undifferentiated Connective Tissue Disease patients progress to definite SSc within a short–term follow–up. Measurement of circulating markers of T–cell and fibroblast activation might serve to identify early SSc patients who are more likely to develop features of definite SSc.Methods
- Thirty–nine early SSc and 37 UCTD patients were investigated.
- At baseline, all patients underwent clinical evaluation, B–mode echocardiography, lung function tests, and esophageal manometry to detect preclinical alterations of internal organs, and were re–assessed every year.
- Twenty–one early SSc and 24 UCTD patients, and 25 controls were also investigated for serum endothelial, T–cell and fibroblast activation markers.
- At baseline, 48.7% of early SSc and 37.8% of UCTD patients had at least one preclinical functional alteration (p > 0.05).
- Ninety–two percent of early SSc patients developed manifestations consistent with definite SSc (i.e. skin sclerosis, digital ulcers/scars, 2 or more teleangectasias, clinically visible nailfold capillaries, cutaneous calcinosis, X–ray bibasilar lung fibrosis, X–ray esophageal dysmotility, ECG signs of myocardial fibrosis, laboratory signs of renal crisis) within 5 years versus 17.1% of UCTD patients (X2=12.26; p=0.0005).
- Avascular areas (HR=4.39; 95% CI=1.18–16.3; p=0.02), increased levels of soluble IL–2 receptor alpha (HR=4.39; 95% CI=1.03–18.6; p=0.03), and of procollagen III aminopropeptide predicted disease evolution (HR=4.55; 95% CI=1.18–17; p=0.04).