title: [Extracorporeal photopheresis in progressive systemic sclerosis: discrimination of responders and non-responders].
abstract: BACKGROUND
  The benefit of extracorporeal photopheresis (ExP) in progressive systemic sclerosis (PSS) is controversial. There is limited experience with the long-term use of ExP in PSS. The purpose of the present study was to distinguish between responders and non-responders by using ExP in PSS and to evaluate activation markers for PSS.
  
  
  METHODS
  20 subjects with PSS were treated for 12 months with ExP (interval: 1x/month) as an immunomodulating monotherapy (11/20) or combination therapy (9/20). The course of PSS was assessed by both specially designed clinical score and serological parameters (CRP, ANA, beta-galactosidase, P-III-P, CD4/CD8-ratio, TNF-alpha, 11-2-R, 11-6).
  
  
  RESULTS
  After 12 cycles of ExP, 30% of the subjects showed a partial remission and 25%, stable disease (55% responders) while 45% had a progression (non-responders). Although there was no correlation between the clinical course and the serological parameters, an increase of beta-galactosidase during therapy marked a progression of PSS in non-responders. Responders with a short PSS-course before ExP, moderate ANA titres, normal TNF-alpha levels and lack of Scl-70 had a good prognosis.
  
  
  CONCLUSIONS
  About the half of the subjects with PSS profited by the long-term use of ExP. Thereby the mild immunomodulating effect of ExP seems to be insufficient to control markedly progressive courses of PSS.
authors.affiliation: Klinik für Dermatologie und Allergologie der Ruhr-Universität, Bochum, Deutschland. S.Reich@elis-stiftung.de
authors.forename: Stefanie
authors.initials: S
authors.lastname: Reich
authors.forename: Michael
authors.initials: M
authors.lastname: Radenhausen
authors.forename: Peter
authors.initials: P
authors.lastname: Altmeyer
authors.forename: Klaus
authors.initials: K
authors.lastname: Hoffmann
mesh_terms: D000328:Adult; D000368:Aged; D017675:CREST Syndrome / Q000175:diagnosis / Q000628:therapy; D003627:Data Interpretation, Statistical; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D017893:Photopheresis; D011379:Prognosis; D011446:Prospective Studies; D012074:Remission Induction; D012595:Scleroderma, Systemic / Q000097:blood / Q000175:diagnosis / Q000201:enzymology / Q000628:therapy*; D013997:Time Factors; D001616:beta-Galactosidase / Q000097:blood
chemical_list: D001616:beta-Galactosidase