Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis).
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.
Bulpitt
K J
KJ
Cooperative Systematic Studies of the Rheumatic Diseases Program, University of Utah, Salt Lake City.
Clements
P J
PJ
Lachenbruch
P A
PA
Paulus
H E
HE
Peter
J B
JB
Agopian
M S
MS
Singer
J Z
JZ
Steen
V D
VD
Clegg
D O
DO
Ziminski
C M
CM
Alarcon
G S
GS
Luggen
M E
ME
Polisson
R P
RP
Willkens
R F
RF
Reading
J C
JC
Williams
H J
HJ
Ward
J R
JR
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
D015375:Receptors, Interleukin-2