title: Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.forename: Antonella
authors.initials: A
authors.lastname: Marcoccia
authors.forename: Giovanna
authors.initials: G
authors.lastname: Cuomo
authors.forename: Serena
authors.initials: S
authors.lastname: Vettori
authors.forename: Michele
authors.initials: M
authors.lastname: Iudici
authors.forename: Francesco
authors.initials: F
authors.lastname: Bondanini
authors.forename: Carlo
authors.initials: C
authors.lastname: Santoriello
authors.forename: Aldo
authors.initials: A
authors.lastname: Ciani
authors.forename: Domenico
authors.initials: D
authors.lastname: Cozzolino
authors.forename: Giovanni Maria
authors.initials: GM
authors.lastname: De Matteis
authors.forename: Salvatore
authors.initials: S
authors.lastname: Cappabianca
authors.forename: Filiberto
authors.initials: F
authors.lastname: Vitelli
authors.forename: Alberto
authors.initials: A
authors.lastname: Spanò
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001168:Arthritis / Q000453:epidemiology / Q000209:etiology; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D015415:Biomarkers / Q000097:blood*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000453:epidemiology / Q000209:etiology; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D015995:Prevalence; D011928:Raynaud Disease / Q000453:epidemiology / Q000209:etiology; D012595:Scleroderma, Systemic / Q000276:immunology* / Q000473:pathology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D015415:Biomarkers