title: Clinical significance of Cryofibrinogenemia: possible pathophysiological link with Raynaud's phenomenon.
abstract: OBJECTIVE
  To describe the clinical findings and prevalence of patients with cryofibrinogenemia (CF) and to determine whether CF is associated with primary Raynaud's phenomenon.
  
  
  METHODS
  Between June 2006 and December 2009, 227 patients were tested for CF in a single university hospital. Forty-five patients with primary Raynaud's phenomenon were tested for CF.
  
  
  RESULTS
  A total of 117 patients with CF without cryoglobulinemia were included. The main clinical manifestations included skin manifestations (50%) and arthralgia (35%). There were 67 patients with primary CF and 50 patients with secondary CF. There was no significant difference in the mean concentration of the cryoprecipitate in primary CF as compared to the secondary form (172 ± 18.6 vs 192 ± 20.9 mg/dl, respectively; p = 0.41). Highest concentrations of cryoprecipitate were observed in those containing fibrinogen only as compared to cryoprecipitates containing fibrinogen and fibronectin (301 ± 43.5 vs 125 ± 10.6 mg/dl; p < 0.001). Patients having skin necrosis (n = 3) had significantly higher values of cryofibrinogen compared to those without necrosis (638 ± 105 vs 160 ± 10.2 mg/dl; p = 0.0046). Among the 45 patients with primary Raynaud's phenomenon, 36 had associated CF. There was no significant difference in the mean concentration of the cryoprecipitate in these patients compared to those with primary CF.
  
  
  CONCLUSIONS
  There seems to be a significant correlation between cryofibrinogen concentration and the severity of the clinical signs, particularly when cryoprecipitate is composed of fibrinogen alone. CF might have a possible pathophysiological role in primary Raynaud's phenomenon.
authors.affiliation: Department of Rheumatology, Erasme Hospital, Université Libre de Bruxelles, 808 Route de Lennik, 1070 Brussels, Belgium. msoyfoo@ulb.ac.be
authors.forename: Muhammad S
authors.initials: MS
authors.lastname: Soyfoo
authors.forename: Ahmed
authors.initials: A
authors.lastname: Goubella
authors.forename: Elie
authors.initials: E
authors.lastname: Cogan
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Wautrecht
authors.forename: Annick
authors.initials: A
authors.lastname: Ocmant
authors.forename: Patrick
authors.initials: P
authors.lastname: Stordeur
mesh_terms: D003449:Cryoglobulinemia / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D005340:Fibrinogen / Q000378:metabolism; D005353:Fibronectins / Q000097:blood; D006801:Humans; D008297:Male; D008875:Middle Aged; D011928:Raynaud Disease / Q000097:blood* / Q000473:pathology / Q000503:physiopathology*; D012189:Retrospective Studies
chemical_list: D005353:Fibronectins; D005340:Fibrinogen