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Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement.
- Source :
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Arthritis research & therapy [Arthritis Res Ther] 2012 Aug 17; Vol. 14 (4), pp. R188. Date of Electronic Publication: 2012 Aug 17. - Publication Year :
- 2012
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Abstract
- Introduction: 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.<br />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.<br />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).<br />Conclusion: 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.
- Subjects :
- Adolescent
Adult
Aged
Biomarkers blood
Early Diagnosis
Female
Follow-Up Studies
Humans
Male
Microscopic Angioscopy methods
Middle Aged
Mixed Connective Tissue Disease blood
Predictive Value of Tests
Radiography
Raynaud Disease blood
Scleroderma, Systemic blood
Young Adult
Disease Progression
Mixed Connective Tissue Disease diagnostic imaging
Raynaud Disease diagnostic imaging
Scleroderma, Systemic diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1478-6362
- Volume :
- 14
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Arthritis research & therapy
- Publication Type :
- Academic Journal
- Accession number :
- 22901779
- Full Text :
- https://doi.org/10.1186/ar4019