title: Prognostic model based on nailfold capillaroscopy for identifying Raynaud's phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination).
abstract: OBJECTIVE
  To construct a prognostic index based on nailfold capillaroscopic examinations that is capable of predicting the 5-year transition from isolated Raynaud's phenomenon (RP) to RP secondary to scleroderma spectrum disorders (SSDs).
  
  
  METHODS
  The study involved 104 consecutive adult patients with a clinical history of isolated RP, and the index was externally validated in another cohort of 100 patients with the same characteristics. Both groups were followed up for 1-8 years. Six variables were examined because of their potential prognostic relevance (branching, enlarged and giant loops, capillary disorganization, microhemorrhages, and the number of capillaries).
  
  
  RESULTS
  The only factors that played a significant prognostic role were the presence of giant loops (hazard ratio [HR] 2.64, P = 0.008) and microhemorrhages (HR 2.33, P = 0.01), and the number of capillaries (analyzed as a continuous variable). The adjusted prognostic role of these factors was evaluated by means of multivariate regression analysis, and the results were used to construct an algorithm-based prognostic index. The model was internally and externally validated.
  
  
  CONCLUSIONS
  Our prognostic capillaroscopic index identifies RP patients in whom the risk of developing SSDs is high. This model is a weighted combination of different capillaroscopy parameters that allows physicians to stratify RP patients easily, using a relatively simple diagram to deduce the prognosis. Our results suggest that this index could be used in clinical practice, and its further inclusion in prospective studies will undoubtedly help in exploring its potential in predicting treatment response.
authors.affiliation: Department of Rheumatology, Instituto Gaetano Pini, University of Milan, Milan, Italy. francesca.ingegnoli@unimi.it
authors.forename: Francesca
authors.initials: F
authors.lastname: Ingegnoli
authors.forename: Patrizia
authors.initials: P
authors.lastname: Boracchi
authors.forename: Roberta
authors.initials: R
authors.lastname: Gualtierotti
authors.forename: Chiara
authors.initials: C
authors.lastname: Lubatti
authors.forename: Laura
authors.initials: L
authors.lastname: Meani
authors.forename: Lenka
authors.initials: L
authors.lastname: Zahalkova
authors.forename: Silvana
authors.initials: S
authors.lastname: Zeni
authors.forename: Flavio
authors.initials: F
authors.lastname: Fantini
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D009262:Nails; D011237:Predictive Value of Tests; D011379:Prognosis; D011928:Raynaud Disease / Q000175:diagnosis*; D018570:Risk Assessment; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000175:diagnosis*; D012720:Severity of Illness Index