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Majority of early SSc patients progressed to full disease within 5 years
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Most patients with early systemic sclerosis progressed to full systemic sclerosis within 5 years of presentation, according to study results.
Researchers evaluated 76 patients (median age 41 years; 97% women) with Raynaud’s phenomenon. Thirty-nine were diagnosed with early systemic sclerosis (SSc), defined as Raynaud’s phenomenon with SSc marker autoantibodies and/or typical capillaroscopic findings and no manifestations other than puffy fingers or arthritis, and 37 patients were diagnosed with undifferentiated connective tissue disease (UCTD). B-mode echocardiography, lung function test and esophageal manometry were used annually to detect internal organ alterations. Researchers also evaluated 21 of the patients with early SSc, 24 patients with UCTD and 25 control participants affected with osteoarthritis or primary fibromyalgia syndrome for serum endothelial, T-cell and fibroblast activation markers.
Baseline measures indicated 48.7% of early SSc patients and 37.8% of UCTD patients with at least one preclinical functional alteration (P>.05). At 5-year follow-up, 92% of patients with early SSC developed definite SSc manifestations, including skin sclerosis, digital ulcers and scars, two or more telangiectasia, clinically visible nail-fold capillaries, X-ray esophageal dysmotility, and laboratory signs of renal crisis, compared with 17.1% of UCTD patients at 2 years after presentation (X2=12.26; P=.0005).
Researchers evaluated serologic, capillaroscopic, clinical and functional parameters to determine if they were predictive of development of additional clinical and/or functional alterations in patients with early SSC. They found that avascular areas (HR=4.39; 95% CI, 1.18-16.3), increased levels of soluble IL-2 receptor alpha (HR=4.39; 95% CI, 1.03-18.6) and procollagen III aminopropeptide (HR=4.55; 95% CI, 1.18-17) were predictors.
“The results of our study should prompt the clinician to investigate early SSc patients for preclinical, functional internal organ involvement and to put them under strict surveillance,” the researchers concluded. “Measurements of circulating markers of T-cell and fibroblast activation might serve to identify early SSc patients who are more likely to develop features to definite SSc.”
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