title: Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity.
abstract: OBJECTIVE
  To investigate whether patients affected by 1 of the 3 subsets of early systemic sclerosis (SSc; scleroderma), i.e., subset I, Raynaud's phenomenon with SSc marker autoantibodies and typical capillaroscopic findings; subset II, autoantibody positive only; and subset III, capillaroscopy positive only and not satisfying the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SSc at admission, differ from each other in the time to satisfy the criteria.
  
  
  METHODS
  Early SSc patients subdivided into the 3 subsets indicated above consecutively admitted to a rheumatology/angiology center were monitored for 12-102 months (median 36 months). Patients were reevaluated twice yearly to assess whether and when each patient satisfied the new ACR/EULAR classification criteria for SSc. Patients with undifferentiated connective tissue disease (UCTD) served as the comparator group.
  
  
  RESULTS
  During followup, 11 (52.3%) of 21 subset I, 10 (66.6%) of 15 subset II, 0 of 24 subset III, and 0 of 44 UCTD patients satisfied the criteria (P = 0.0001). The difference was significant between early SSc and UCTD patients (P = 0.0001) and, within the group of early SSc patients, between each of the 2 autoantibody-positive subsets (subsets I and II) and the capillaroscopic-positive/autoantibody-negative subset (subset I versus III: P = 0.0001; subset II versus III: P = 0.0009). There was no difference between the 2 autoantibody-positive subsets (P = 0.454). In addition to marker autoantibody positivity, preclinical lung or heart involvement was associated with an increased risk to satisfy the criteria during followup.
  
  
  CONCLUSIONS
  Our data demonstrated faster progression of SSc in autoantibody-positive patients, particularly in those with preclinical internal organ involvement at baseline, than in autoantibody-negative patients.
authors: Second University of Naples, Naples, Italy.
authors: Gabriele
authors: G
authors: Valentini
authors: Antonella
authors: A
authors: Marcoccia
authors: Giovanna
authors: G
authors: Cuomo
authors: Serena
authors: S
authors: Vettori
authors: Michele
authors: M
authors: Iudici
authors: Francesco
authors: F
authors: Bondanini
authors: Carlo
authors: C
authors: Santoriello
authors: Aldo
authors: A
authors: Ciani
authors: Domenico
authors: D
authors: Cozzolino
authors: Giovanni Maria
authors: GM
authors: De Matteis
authors: Salvatore
authors: S
authors: Cappabianca
authors: Filiberto
authors: F
authors: Vitelli
authors: Alberto
authors: A
authors: Spanò
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D007558:Italy; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011928:Raynaud Disease / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D012595:Scleroderma, Systemic / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D013997:Time Factors; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015415:Biomarkers