title: Scleroderma lung study (SLS): differences in the presentation and course of patients with limited versus diffuse systemic sclerosis.
abstract: OBJECTIVE
  Pulmonary fibrosis is a leading cause of death in systemic sclerosis (SSc). This report examines the differences at baseline and over 12 months between patients with limited versus diffuse cutaneous SSc who participated in the Scleroderma Lung Study.
  
  
  METHODS
  SSc patients (64 limited; 94 diffuse) exhibiting dyspnoea on exertion, restrictive pulmonary function and evidence of alveolitis on bronchoalveolar lavage and/or high-resolution computed tomography (HRCT) were randomised to receive cyclophosphamide (CYC) or placebo and serially evaluated over 12 months.
  
  
  RESULTS
  Baseline measures of alveolitis, dyspnoea and pulmonary function were similar in limited and diffuse SSc. However, differences were noted with respect to HRCT-scored fibrosis (worse in limited SSc), and to functional activity, quality of life, skin and musculoskeletal manifestations (worse in diffuse SSc) (p<0.05). When adjusted for the baseline level of fibrosis, both groups responded similarly to CYC with regard to lung function and dyspnoea (p<0.05). Cyclophosphamide was also associated with more improvement in skin score in the diffuse disease group more than in the limited disease group (p<0.05).
  
  
  CONCLUSIONS
  After adjusting for the severity of fibrosis at baseline, CYC slowed the decline of lung volumes and improved dyspnoea equally in the limited and the diffuse SSc groups. On the other hand, diffuse SSc patients responded better than limited patients with respect to improvements in skin thickening.
authors.affiliation: Divisions of Rheumatology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA. pclements@mednet.ucla.edu
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Clements
authors.forename: Michael D
authors.initials: MD
authors.lastname: Roth
authors.forename: Robert
authors.initials: R
authors.lastname: Elashoff
authors.forename: Donald P
authors.initials: DP
authors.lastname: Tashkin
authors.forename: Jonathan
authors.initials: J
authors.lastname: Goldin
authors.forename: Richard M
authors.initials: RM
authors.lastname: Silver
authors.forename: Mildred
authors.initials: M
authors.lastname: Sterz
authors.forename: James R
authors.initials: JR
authors.lastname: Seibold
authors.forename: Dean
authors.initials: D
authors.lastname: Schraufnagel
authors.forename: Robert W
authors.initials: RW
authors.lastname: Simms
authors.forename: Marcy
authors.initials: M
authors.lastname: Bolster
authors.forename: Robert A
authors.initials: RA
authors.lastname: Wise
authors.forename: Virginia
authors.initials: V
authors.lastname: Steen
authors.forename: M D
authors.initials: MD
authors.lastname: Mayes
authors.forename: Kari
authors.initials: K
authors.lastname: Connelly
authors.forename: Mark
authors.initials: M
authors.lastname: Metersky
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
mesh_terms: D000328:Adult; D018893:Bronchoalveolar Lavage; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents; D007596:Joints / Q000473:pathology; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012129:Respiratory Function Tests; D045743:Scleroderma, Diffuse / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D014057:Tomography, X-Ray Computed
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide