Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement.
BACKGROUND
We investigated early systemic sclerosis (SSc) (that is, Raynaud's phenomenon with SSc marker autoantibodies and/or typical capillaroscopic findings and no manifestations other than puffy fingers or arthritis) versus undifferentiated connective tissue disease (UCTD) to identify predictors of short-term disease evolution.


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.


RESULTS
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 (that is, skin sclerosis, digital ulcers/scars, two or more teleangectasias, clinically visible nailfold capillaries, cutaneous calcinosis, X-ray bibasilar lung fibrosis, X-ray esophageal dysmotility, ECG signs of myocardial fibrosis and laboratory signs of renal crisis) within five years versus 17.1% of UCTD patients (X² = 12.26; P = 0.0005). Avascular areas (HR = 4.39 95% CI 1.18 to 16.3; P = 0.02), increased levels of soluble IL-2 receptor alpha (HR = 4.39; 95% CI 1.03 to 18.6; P = 0.03), and of procollagen III aminopropeptide predicted disease evolution (HR = 4.55; 95% CI 1.18 to 17; P = 0.04).


CONCLUSIONS
Most early SSc but only a few UCTD 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.
Gabriele
G
Valentini
Serena
S
Vettori
Giovanna
G
Cuomo
Michele
M
Iudici
Virginia
V
D'Abrosca
Domenico
D
Capocotta
Gianmattia
G
Del Genio
Carlo
C
Santoriello
Domenico
D
Cozzolino
D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D020678:Microscopic Angioscopy / Q000379:methods; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000000981:diagnostic imaging*; D011237:Predictive Value of Tests; D011859:Radiography; D011928:Raynaud Disease / Q000097:blood / Q000000981:diagnostic imaging*; D012595:Scleroderma, Systemic / Q000097:blood / Q000000981:diagnostic imaging*; D055815:Young Adult
D015415:Biomarkers