Disease activity,damage and survival in Mexican patients with acute severe systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a clinical syndrome of varying severity. Although the survival and prognosis of SLE have steadily improved, there is a group of patients who present an acute fatal outcome despite aggressive therapy. We designed this study to evaluate the factors associated with mortality in patients with acute severe SLE. During 2004—06, 41 Mexican SLE patients that could not be managed in the out-patient clinic and with acute severe major organ system involvement [nephritis, severe thrombocytopenia (platelet count below 20 000 per μL) acute neuropsychiatric pulmonary, gastrointestinal or cardiac disease and generalized vasculitis] were studied. During the first admission, disease activity (SLE Disease Activity Index (SLEDAI), SLE Activity Measured), damage [SLE International Collaborating Clinics (SLICC)], and therapy were assessed. Survival using Kaplan—Meier curves, odd ratios with 95% confidence interval and logistic regression analysis were used to determine risk factors for mortality. Ninety percent were female with a mean age of 29 ± 19 years and mean disease duration of 21 ± 9 months. The principal causes of first admission were renal (27%), SNC (22%) and cardiopulmonary (15%). After a mean follow-up of 9.7 ± 6 months, 16 (39%) patients died. Deceased patients had significantly higher SLEDAI (P = 0.004), and SLICC (P = 0.03) scores. The manifestations associated with mortality were renal disease activity (odds ratio, OR 4.6, confidence interval, CI 95% 1.0—20.6), infections (OR 3.2 CI 95% 2.0—5.3) and thrombocytopenia (OR 4.0, CI 95% 1.0—15.9). The survival at 9.7 months was 72, 62 and 50% in patients with an SLEDAI score of 3—10, 11—20 and ≥21, respectively. The SLEDAI score, the presence of damage and infection were associated with death in patients with acute severe SLE. Lupus (2007) 16, 997—1000.

Keywords

acute severe SLE damage disease activity survival

Get full access to this article

View all access options for this article.

References

Bernatzky S. , Boivin JF , Joseph L. et al. Mortality in systemic lupus erythematosus . Arthritis Rheum 2006; 54: 2550—2557.

Petri M. , Genovese M. Incidence of and risk factors for hospitalizations in systemic lupus erythematosus: a prospective study of the Hopkins lupus cohort. J Rheumatol 1992; 19: 1559—1565.

Cervera R. , Khamashta MA , Font J. et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine 2003; 82: 299—308.

Petri M. , Genovese M. , Engle E. , Hochberg M. Definition, incidence, and clinical description of flare in systemic lupus erythematosus. A prospective cohort study. Arthritis Rheum 1991; 34: 937—944.

Zonana-Nacach A. , Salas M. , Sánchez ML , Camargo-Coronel A. , Bravo-Gatica C. , Mintz GJ Measurement of clinical activity of systemic lupus erythematosus and laboratory abnormalities: twelve-months prospective study. Rheumatol 1995; 22: 45—49.

Barr SG , Zonana-Nacach A. , Madger LS , Petri M. Patterns of disease activity in systemic lupus erythematosus. Arthritis Rheum 1999; 42: 2682—2688.

Tan EM , Cohen AS , Fries JF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271—1277.

Liang MH , Socher SA , Larson MG , Schur PH Reliability and validity of six systems for the clinical assessment of disease activity in systemic lupus erythematosus. Arthritis Rheum 1989; 32: 1107—1118.

Bombardier C. , Gladman D. , Urowitz M. et al. Derivation of SLEDAI: a disease activity index for lupus patients. Arthritis Rheum 1992; 19: 53—59.

10.

Gladman DD , Goldsmith CH , Urowitz MB et al. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index for Systemic Lupus Erythematosus International Comparison. J Rheumatol 2000 ; 27: 373—376.

11.

Edwards CJ , Lian TY , Badsha H. , Teh CL , Arden N. , Chng HH Hospitalization of individuals with systemic lupus erythematosus:characteristics and predictors of outcome. Lupus 2003; 12: 672—676.

12.

Krishnan E. Hospitalization and mortality of patients with systemic lupus erythematosus. J Rheumatol 2006; 33: 1770—1774.

13.

Zonana-Nacach A. , Camargo-Coronel A. , Yañez P. et al. Measurement of damage in 210 Mexican patients with systemic lupus erythematosus: relationship with disease duration. Lupus 1998; 7: 119—123.

14.

Zonana-Nacach A. , Barr SG , Magder LS , Petri M. Damage in systemic lupus erythematosus and its association with corticosteroids. Arthritis Rheum 2000; 43: 1801—1808.

15.

Cook RJ , Gladman DD , Pericak D. , Urowitz MB Prediction of short term mortality in systemic lupus erythematosus with time dependent measures of disease activity. J Rheumatol 2000; 27: 1892—1895.

16.

Rahman P. , Gladman DD , Urowitz MB , Hallett D. , Tam LS Early damage as measured by the SLICC/ACR damage index is a predictor of mortality in systemic lupus erythematosus. Lupus 2001 ; 10: 93—96.