title: Autologous stem cell transplantation in diffuse scleroderma: impact on hand structure and function.
abstract: BACKGROUND
  The aim of the study was to assess the structural and functional effects of autologous stem cell transplantation (ASCT) on scleroderma finger clawing.
  
  
  METHODS
  Using photocopies of hands of five scleroderma patients who underwent ASCT using photocopies of hands. Functional assessments used a standardized questionnaire.
  
  
  RESULTS
  Pre-ASCT, synovitis and tenosynovitis were present in five and four patients, respectively. Modified Rodnan hand skin scores ranged from 6-12/12. Following pulsed chemotherapy, synovitis resolved. Tenosynovitis often did not. Post-ASCT, skin scores fell in four patients (range 0-6/12). Hand tenosynovitis resolved. With disease remission hand function globally improved. Functional improvement, noted early (+3 months) and continuously (+12 months) in disease remitters, occurred in all areas of function. Greatest hand-functional improvement related to paid employment, followed by self-care and hygiene, home-care activities and least by hobbies/sports. The second to fifth metacarpophalangeal width was reproducible and independent of ASCT therapy. In contrast, hand length and measures of abducted finger span (first to fifth fingertip and second to fifth fingertip distance) improved. Finger abduction (abducted first to fifth fingertips/second to fifth metacarpophalangeal width) was a more sensitive discriminator of finger clawing than hand length or hand length/second to fifth metacarpophalangeal width.
  
  
  CONCLUSIONS
  ASCT improved hand scleroderma over 12 months and resolved previously refractory tenosynovitis. ASCT was unnecessary to treat scleroderma synovitis. ASCT secondarily improved hand function (paid employment, followed by self-care, home care, then by sport/hobbies). Loss of finger abduction was a more sensitive measure of finger clawing than apparent loss of hand length.
authors.affiliation: Department of Rheumatology, Westmead Hospital, Sydney, New South Wales, Australia. helenen@westgate.wh.usyd.edu.au
authors.forename: H
authors.initials: H
authors.lastname: Englert
authors.forename: S
authors.initials: S
authors.lastname: Kirkham
authors.forename: J
authors.initials: J
authors.lastname: Moore
authors.forename: T S
authors.initials: TS
authors.lastname: Poon
authors.forename: C
authors.initials: C
authors.lastname: Katelaris
authors.forename: N
authors.initials: N
authors.lastname: McGill
authors.forename: L
authors.initials: L
authors.lastname: Schrieber
authors.forename: N
authors.initials: N
authors.lastname: Manolios
mesh_terms: D000328:Adult; D005260:Female; D006225:Hand / Q000473:pathology* / Q000502:physiology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D045743:Scleroderma, Diffuse / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery*; D033581:Stem Cell Transplantation / Q000379:methods*; D014182:Transplantation, Autologous; D055815:Young Adult