title: Myofibroblasts and hyalinized collagen as markers of skin disease in systemic sclerosis.
abstract: OBJECTIVE
  To investigate the correlation between the degree of dermal fibrosis and myofibroblast infiltration using clinical assessments of skin thickness and hardness in systemic sclerosis (SSc).
  
  
  METHODS
  Eleven patients with diffuse SSc and 10 healthy controls were evaluated using the modified Rodnan skin thickness score and durometry (hardness measurement). Biopsy samples were obtained from the dorsal mid-forearm in all subjects at the baseline visit and again 6-12 months later in patients with SSc. Five of the patients with SSc received treatment with cyclophosphamide (CYC) in the interval between skin biopsies. Biopsy sections were assessed for myofibroblast and hyalinized collagen content by 2 blinded observers.
  
  
  RESULTS
  Myofibroblast and hyalinized collagen scores each correlated with the forearm skin score (r = 0.83, P < 0.0001 and r = 0.78, P < 0.0001, respectively) and with the forearm durometry score (r = 0.72, P < 0.0004 and r = 0.69, P < 0.0008, respectively). The change in the dermal hyalinized collagen score correlated with the change in the forearm durometry score (r = 0.74, P < 0.0213). The myofibroblast score decreased in all 5 patients who received CYC and increased in those receiving non-CYC treatments (P < 0.01 for the difference).
  
  
  CONCLUSIONS
  Myofibroblasts play an important role in the pathogenesis of fibrosis, and our data imply that quantification of myofibroblasts and hyalinized collagen in skin may be a useful outcome measure in clinical studies of SSc.
authors.affiliation: Boston University Medical Center, Boston, Massachusetts, USA. eugene.kissin@bmc.org
authors.forename: Eugene Y
authors.initials: EY
authors.lastname: Kissin
authors.forename: Peter A
authors.initials: PA
authors.lastname: Merkel
authors.forename: Robert
authors.initials: R
authors.lastname: Lafyatis
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000378:metabolism*; D001706:Biopsy; D003094:Collagen / Q000378:metabolism*; D020405:Dermis / Q000378:metabolism / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000473:pathology*; D006801:Humans; D006818:Hyalin / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012595:Scleroderma, Systemic / Q000378:metabolism* / Q000473:pathology*
chemical_list: D015415:Biomarkers; D003094:Collagen