Open Access Highly Accessed Research article

Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers

Gabriele Valentini1*, Antonella Marcoccia2, Giovanna Cuomo1, Serena Vettori1, Michele Iudici1, Francesco Bondanini3, Carlo Santoriello4, Aldo Ciani5, Domenico Cozzolino6, Giovanni Maria De Matteis7, Salvatore Cappabianca8, Filiberto Vitelli9 and Alberto Spanò3

Author affiliations

1 Rheumatology Unit, Second University of Naples, via Pansini 5, 80131 Naples, Italy

2 Angiology Unit, Sandro Pertini Hospital, via dei Monti Tiburtini 385, 00157 Rome, Italy

3 Clinical Biochemistry Service, Sandro Pertini Hospital, via dei Monti Tiburtini 385, 00157 Rome, Italy

4 Respiratory Physiopathology Unit, ASL-SA1, via Santoriello 2, Cava dei Tirreni (SA), Italy

5 Pneumology Unit, Sandro Pertini Hospital, via dei Monti Tiburtini 385, 00157 Rome, Italy

6 Internal Medicine Unit, Second University of Naples, via Pansini 5, 80131 Naples, Italy

7 Cardiology Unit, Sandro Pertini Hospital, via dei Monti Tiburtini 385, 00157 Rome, Italy

8 Radiology, Radiotherapy and Nuclear Medicine Unit, Second University of Naples, Piazza Miraglia 5, 80131 Naples, Italy

9 Radiology Unit, Sandro Pertini Hospital, via dei Monti Tiburtini 385, 00157 Rome, Italy

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Citation and License

Arthritis Research & Therapy 2013, 15:R63  doi:10.1186/ar4236

Published: 29 May 2013

Abstract

Introduction

Early systemic sclerosis (SSc) is characterized by Raynaud's phenomenon together with scleroderma marker autoantibodies and/or a scleroderma pattern at capillaroscopy and no other distinctive feature of SSc. Patients presenting with marker autoantibodies plus a capillaroscopic scleroderma pattern seem to evolve into definite SSc more frequently than patients with either feature. Whether early SSc patients with only marker autoantibodies or capillaroscopic positivity differ in any aspect at presentation is unclear.

Methods

Seventy-one consecutive early SSc patients were investigated for preclinical cardiopulmonary alterations. Out of these, 44 patients and 25 controls affected by osteoarthritis or primary fibromyalgia syndrome were also investigated for serum markers of fibroblast (carboxyterminal propeptide of collagen I), endothelial (soluble E-selectin) and T-cell (soluble IL-2 receptor alpha) activation.

Results

Thirty-two of the 71 patients (45.1%) had both a marker autoantibody and a capillaroscopic scleroderma pattern (subset 1), 16 patients (22.5%) had only a marker autoantibody (subset 2), and 23 patients (32.4%) had only a capillaroscopic scleroderma pattern (subset 3). Patients with marker autoantibodies (n = 48, 67.6%) had a higher prevalence of impaired diffusing lung capacity for carbon monoxide (P = 0.0217) and increased serum levels of carboxyterminal propeptide of collagen I (P = 0.0037), regardless of capillaroscopic alterations. Patients with a capillaroscopic scleroderma pattern (n = 55, 77.5%) had a higher prevalence of puffy fingers (P = 0.0001) and increased serum levels of soluble E-selectin (P = 0.0003) regardless of marker autoantibodies.

Conclusion

These results suggest that the autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Longitudinal studies are warranted to investigate whether these subsets undergo a different disease course over time.

Keywords:
Raynaud's phenomenon; early systemic sclerosis; systemic sclerosis marker autoantibodies; nailfold videocapillaroscopy; preclinical organ involvement; puffy fingerscirculating activation markers; carboxyterminal propeptide of collagen I; soluble E-selectin; soluble IL-2 receptor alpha