Early undifferentiated connective tissue disease

III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis)

Ken J. Bulpitt, Philip J. Clements, Peter A. Lachenbruch, Harold E. Paulus, James B. Peter, Mel S. Agopian, Joyce Z. Singer, Virginia D. Steen, Daniel O. Clegg, Carol M Ziminski, Graciela S. Alarcón, Michael E. Luggen, Richard P. Polisson, Robert F. Willkens, James C. Reading, H. James Williams, John R. Ward

Research output: Contribution to journalArticle

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. Design: Inception cohort study. Setting. Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program. Patients: Forty-eight patients who had had scleroderma for less than 1 year. Measurements: 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). Conclusion: 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.

Original languageEnglish (US)
Pages (from-to)602-609
Number of pages8
JournalAnnals of Internal Medicine
Volume118
Issue number8
StatePublished - Apr 15 1993
Externally publishedYes

Fingerprint

Connective Tissue Diseases
Systemic Scleroderma
Pyuria
Survival
Rheumatology
Hematuria
Rheumatic Diseases
Cohort Studies
Survival Rate
Urine
Inflammation
Kidney
Lung

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Bulpitt, K. J., Clements, P. J., Lachenbruch, P. A., Paulus, H. E., Peter, J. B., Agopian, M. S., ... Ward, J. R. (1993). Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis). Annals of Internal Medicine, 118(8), 602-609.

Early undifferentiated connective tissue disease : III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis). / Bulpitt, Ken J.; Clements, Philip J.; Lachenbruch, Peter A.; Paulus, Harold E.; Peter, James B.; Agopian, Mel S.; Singer, Joyce Z.; Steen, Virginia D.; Clegg, Daniel O.; Ziminski, Carol M; Alarcón, Graciela S.; Luggen, Michael E.; Polisson, Richard P.; Willkens, Robert F.; Reading, James C.; Williams, H. James; Ward, John R.

In: Annals of Internal Medicine, Vol. 118, No. 8, 15.04.1993, p. 602-609.

Research output: Contribution to journalArticle

Bulpitt, KJ, Clements, PJ, Lachenbruch, PA, Paulus, HE, Peter, JB, Agopian, MS, Singer, JZ, Steen, VD, Clegg, DO, Ziminski, CM, Alarcón, GS, Luggen, ME, Polisson, RP, Willkens, RF, Reading, JC, Williams, HJ & Ward, JR 1993, 'Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis)', Annals of Internal Medicine, vol. 118, no. 8, pp. 602-609.
Bulpitt KJ, Clements PJ, Lachenbruch PA, Paulus HE, Peter JB, Agopian MS et al. Early undifferentiated connective tissue disease: III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis). Annals of Internal Medicine. 1993 Apr 15;118(8):602-609.
Bulpitt, Ken J. ; Clements, Philip J. ; Lachenbruch, Peter A. ; Paulus, Harold E. ; Peter, James B. ; Agopian, Mel S. ; Singer, Joyce Z. ; Steen, Virginia D. ; Clegg, Daniel O. ; Ziminski, Carol M ; Alarcón, Graciela S. ; Luggen, Michael E. ; Polisson, Richard P. ; Willkens, Robert F. ; Reading, James C. ; Williams, H. James ; Ward, John R. / Early undifferentiated connective tissue disease : III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis). In: Annals of Internal Medicine. 1993 ; Vol. 118, No. 8. pp. 602-609.
@article{a8df7b28d23941709435d35cd871b49f,
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. Design: Inception cohort study. Setting. Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program. Patients: Forty-eight patients who had had scleroderma for less than 1 year. Measurements: 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). Conclusion: 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.",
author = "Bulpitt, {Ken J.} and Clements, {Philip J.} and Lachenbruch, {Peter A.} and Paulus, {Harold E.} and Peter, {James B.} and Agopian, {Mel S.} and Singer, {Joyce Z.} and Steen, {Virginia D.} and Clegg, {Daniel O.} and Ziminski, {Carol M} and Alarc{\'o}n, {Graciela S.} and Luggen, {Michael E.} and Polisson, {Richard P.} and Willkens, {Robert F.} and Reading, {James C.} and Williams, {H. James} and Ward, {John R.}",
year = "1993",
month = "4",
day = "15",
language = "English (US)",
volume = "118",
pages = "602--609",
journal = "Annals of Internal Medicine",
issn = "0003-4819",
publisher = "American College of Physicians",
number = "8",

}

TY - JOUR

T1 - Early undifferentiated connective tissue disease

T2 - III. Outcome and prognostic indicators in early scleroderma (systemic sclerosis)

AU - Bulpitt, Ken J.

AU - Clements, Philip J.

AU - Lachenbruch, Peter A.

AU - Paulus, Harold E.

AU - Peter, James B.

AU - Agopian, Mel S.

AU - Singer, Joyce Z.

AU - Steen, Virginia D.

AU - Clegg, Daniel O.

AU - Ziminski, Carol M

AU - Alarcón, Graciela S.

AU - Luggen, Michael E.

AU - Polisson, Richard P.

AU - Willkens, Robert F.

AU - Reading, James C.

AU - Williams, H. James

AU - Ward, John R.

PY - 1993/4/15

Y1 - 1993/4/15

N2 - 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. Design: Inception cohort study. Setting. Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program. Patients: Forty-eight patients who had had scleroderma for less than 1 year. Measurements: 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). Conclusion: 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.

AB - 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. Design: Inception cohort study. Setting. Ten university-based rheumatology clinics participating in the Cooperative Systematic Studies of Rheumatic Diseases Program. Patients: Forty-eight patients who had had scleroderma for less than 1 year. Measurements: 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). Conclusion: 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.

UR - http://www.scopus.com/inward/record.url?scp=0027414628&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027414628&partnerID=8YFLogxK

M3 - Article

VL - 118

SP - 602

EP - 609

JO - Annals of Internal Medicine

JF - Annals of Internal Medicine

SN - 0003-4819

IS - 8

ER -