title: Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis).
abstract: OBJECTIVE
  To characterize the course of early scleroderma and to delineate prognostic factors present within 1 year of disease onset that might identify patients at high risk.
  
  
  METHODS
  Inception cohort study.
  
  
  METHODS
  Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program.
  
  
  METHODS
  Forty-eight patients who had had scleroderma for less than 1 year.
  
  
  METHODS
  Fifteen patients with early scleroderma who died were compared with those still living during the initial study period (1982 to 1992). Kaplan-Meier survival estimation and Cox proportional hazards analysis were used to analyze baseline variables for their ability to predict survival duration.
  
  
  RESULTS
  Eight of 15 deaths were due to cardiac or pulmonary system failure. The estimated 5-year survival rate was 68%. Baseline factors that were the most predictive of a poor outcome included the presence of abnormal cardiopulmonary signs and abnormal urine sediment (pyuria, hematuria).
  
  
  CONCLUSIONS
  Evidence of early cardiopulmonary disease, renal disease, inflammation, or immune activation may identify a subset of patients with scleroderma who will experience rapidly progressive disease and early death.
authors.affiliation: Cooperative Systematic Studies of the Rheumatic Diseases Program, University of Utah, Salt Lake City.
authors.forename: K J
authors.initials: KJ
authors.lastname: Bulpitt
authors.forename: P J
authors.initials: PJ
authors.lastname: Clements
authors.forename: P A
authors.initials: PA
authors.lastname: Lachenbruch
authors.forename: H E
authors.initials: HE
authors.lastname: Paulus
authors.forename: J B
authors.initials: JB
authors.lastname: Peter
authors.forename: M S
authors.initials: MS
authors.lastname: Agopian
authors.forename: J Z
authors.initials: JZ
authors.lastname: Singer
authors.forename: V D
authors.initials: VD
authors.lastname: Steen
authors.forename: D O
authors.initials: DO
authors.lastname: Clegg
authors.forename: C M
authors.initials: CM
authors.lastname: Ziminski
authors.forename: G S
authors.initials: GS
authors.lastname: Alarcon
authors.forename: M E
authors.initials: ME
authors.lastname: Luggen
authors.forename: R P
authors.initials: RP
authors.lastname: Polisson
authors.forename: R F
authors.initials: RF
authors.lastname: Willkens
authors.forename: J C
authors.initials: JC
authors.lastname: Reading
authors.forename: H J
authors.initials: HJ
authors.lastname: Williams
authors.forename: J R
authors.initials: JR
authors.lastname: Ward
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D005260:Female; D006331:Heart Diseases / Q000401:mortality; D006801:Humans; D008171:Lung Diseases / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D015375:Receptors, Interleukin-2 / Q000032:analysis; D012595:Scleroderma, Systemic / Q000378:metabolism / Q000401:mortality* / Q000503:physiopathology; D016019:Survival Analysis; D013997:Time Factors
chemical_list: D015375:Receptors, Interleukin-2