Abstract
Background: Systemic lupus erythematosus has a wide spectrum of immunological and clinical manifestations. Its course is characterised by exacerbations which may result in mortality or morbidity to vital organs/systems.
Objective: To determine clear and early prognostic markers to avoid further complications.
Methods: 245 adult patients diagnosed between January 1978 and March 2001 were studied. Clinical manifestations and laboratory findings both at onset and during the clinical course were collected. The number, type, and severity of the flares were also noted. Statistical analyses between disease features at onset, subsequent flares, and mortality were performed.
Results: 239 patients entered the study. Their mean age at onset was 30 years. The mean time between onset and diagnosis was 36 months and the mean evolution time was 114 months. 205 patients developed 915 flares; 205 (22.4%) of these flares were major flares, and affected 110 patients. Cardiac, neurological, or renal affection at onset were associated with a higher probability of developing cardiac (p=0.022), neurological (p<0.001), and renal (p<0.001) exacerbations, respectively, during the evolution. Lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) were predictors of stroke (aCL, p=0.000; LA, p=0.001). Age at diagnosis (p=0.003) and valvular disease at onset (p=0.008) were independent predictors of low survival.
Conclusions: Renal, cardiac, or neurological involvement and the presence of LA or aCL positivity at onset were predictors of renal, cardiac, or neurological flares, respectively. Age and valvular involvement at onset were found to be independent adverse outcome predictors for low survival.
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Selected References
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- Amigo M. C., Khamashta M. A., Hughes G. R. Antiphospholipid syndrome in SLE. Baillieres Clin Rheumatol. 1998 Aug;12(3):477–493. doi: 10.1016/s0950-3579(98)80031-9. [DOI] [PubMed] [Google Scholar]
- Appel G. B., Cohen D. J., Pirani C. L., Meltzer J. I., Estes D. Long-term follow-up of patients with lupus nephritis. A study based on the classification of the World Health Organization. Am J Med. 1987 Nov;83(5):877–885. doi: 10.1016/0002-9343(87)90645-0. [DOI] [PubMed] [Google Scholar]
- Austin H. A., 3rd, Boumpas D. T., Vaughan E. M., Balow J. E. High-risk features of lupus nephritis: importance of race and clinical and histological factors in 166 patients. Nephrol Dial Transplant. 1995;10(9):1620–1628. [PubMed] [Google Scholar]
- Blanco F. J., Gómez-Reino J. J., de la Mata J., Corrales A., Rodríguez-Valverde V., Rosas J. C., Gómez de la Camara A., Pascual E. Survival analysis of 306 European Spanish patients with systemic lupus erythematosus. Lupus. 1998;7(3):159–163. doi: 10.1191/096120398678919930. [DOI] [PubMed] [Google Scholar]
- Bombardier C., Gladman D. D., Urowitz M. B., Caron D., Chang C. H. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum. 1992 Jun;35(6):630–640. doi: 10.1002/art.1780350606. [DOI] [PubMed] [Google Scholar]
- Cervera R., Khamashta M. A., Font J., Sebastiani G. D., Gil A., Lavilla P., Aydintug A. O., Jedryka-Góral A., de Ramón E., Fernández-Nebro A. Morbidity and mortality in systemic lupus erythematosus during a 5-year period. A multicenter prospective study of 1,000 patients. European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore) 1999 May;78(3):167–175. doi: 10.1097/00005792-199905000-00003. [DOI] [PubMed] [Google Scholar]
- Cervera R., Khamashta M. A., Font J., Sebastiani G. D., Gil A., Lavilla P., Doménech I., Aydintug A. O., Jedryka-Góral A., de Ramón E. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore) 1993 Mar;72(2):113–124. [PubMed] [Google Scholar]
- DUBOIS E. L., TUFFANELLI D. L. CLINICAL MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS. COMPUTER ANALYSIS OF 520 CASES. JAMA. 1964 Oct 12;190:104–111. doi: 10.1001/jama.1964.03070150014003. [DOI] [PubMed] [Google Scholar]
- Dajee H., Hurley E. J., Szarnicki R. J. Cardiac valve replacement in systemic lupus erythematosus. A review. J Thorac Cardiovasc Surg. 1983 May;85(5):718–726. [PubMed] [Google Scholar]
- Donadio J. V., Jr, Hart G. M., Bergstralh E. J., Holley K. E. Prognostic determinants in lupus nephritis: a long-term clinicopathologic study. Lupus. 1995 Apr;4(2):109–115. doi: 10.1177/096120339500400206. [DOI] [PubMed] [Google Scholar]
- Drenkard C., Villa A. R., Alarcón-Segovia D., Pérez-Vázquez M. E. Influence of the antiphospholipid syndrome in the survival of patients with systemic lupus erythematosus. J Rheumatol. 1994 Jun;21(6):1067–1072. [PubMed] [Google Scholar]
- Drenkard C., Villa A. R., Alarcón-Segovia D., Pérez-Vázquez M. E. Influence of the antiphospholipid syndrome in the survival of patients with systemic lupus erythematosus. J Rheumatol. 1994 Jun;21(6):1067–1072. [PubMed] [Google Scholar]
- Exner T., Triplett D. A., Taberner D., Machin S. J. Guidelines for testing and revised criteria for lupus anticoagulants. SSC Subcommittee for the Standardization of Lupus Anticoagulants. Thromb Haemost. 1991 Mar 4;65(3):320–322. [PubMed] [Google Scholar]
- Grishman E., Gerber M. A., Churg J. Patterns of renal injury in systemic lupus erythematosus: light and immunofluorescence microscopic observations. Am J Kidney Dis. 1982 Jul;2(1 Suppl 1):135–141. [PubMed] [Google Scholar]
- Gulko P. S., Reveille J. D., Koopman W. J., Burgard S. L., Bartolucci A. A., Alarcón G. S. Survival impact of autoantibodies in systemic lupus erythematosus. J Rheumatol. 1994 Feb;21(2):224–228. [PubMed] [Google Scholar]
- Haider Y. S., Roberts W. C. Coronary arterial disease in systemic lupus erythematosus; quantification of degrees of narrowing in 22 necropsy patients (21 women) aged 16 to 37 years. Am J Med. 1981 Apr;70(4):775–781. doi: 10.1016/0002-9343(81)90532-5. [DOI] [PubMed] [Google Scholar]
- Hochberg M. C. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep;40(9):1725–1725. doi: 10.1002/art.1780400928. [DOI] [PubMed] [Google Scholar]
- Jonsson H., Nived O., Sturfelt G. Outcome in systemic lupus erythematosus: a prospective study of patients from a defined population. Medicine (Baltimore) 1989 May;68(3):141–150. [PubMed] [Google Scholar]
- Khamashta M. A., Cervera R., Asherson R. A., Font J., Gil A., Coltart D. J., Vázquez J. J., Paré C., Ingelmo M., Oliver J. Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus. Lancet. 1990 Jun 30;335(8705):1541–1544. doi: 10.1016/0140-6736(90)91373-i. [DOI] [PubMed] [Google Scholar]
- Kumar A., Malaviya A. N., Singh R. R., Singh Y. N., Adya C. M., Kakkar R. Survival in patients with systemic lupus erythematosus in India. Rheumatol Int. 1992;12(3):107–109. doi: 10.1007/BF00290264. [DOI] [PubMed] [Google Scholar]
- Massardo L., Martínez M. E., Jacobelli S., Villarroel L., Rosenberg H., Rivero S. Survival of Chilean patients with systemic lupus erythematosus. Semin Arthritis Rheum. 1994 Aug;24(1):1–11. doi: 10.1016/0049-0172(94)90094-9. [DOI] [PubMed] [Google Scholar]
- McLaughlin J., Gladman D. D., Urowitz M. B., Bombardier C., Farewell V. T., Cole E. Kidney biopsy in systemic lupus erythematosus. II. Survival analyses according to biopsy results. Arthritis Rheum. 1991 Oct;34(10):1268–1273. doi: 10.1002/art.1780341010. [DOI] [PubMed] [Google Scholar]
- Moder K. G., Miller T. D., Tazelaar H. D. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc. 1999 Mar;74(3):275–284. doi: 10.4065/74.3.275. [DOI] [PubMed] [Google Scholar]
- Nossent J. C. Course and prognostic value of Systemic Lupus Erythematosus Disease Activity Index in black Caribbean patients. Semin Arthritis Rheum. 1993 Aug;23(1):16–21. doi: 10.1016/s0049-0172(05)80023-x. [DOI] [PubMed] [Google Scholar]
- Ordi J., Selva A., Monegal F., Porcel J. M., Martinez-Costa X., Vilardell M. Anticardiolipin antibodies and dependence of a serum cofactor. A mechanism of thrombosis. J Rheumatol. 1993 Aug;20(8):1321–1324. [PubMed] [Google Scholar]
- Pistiner M., Wallace D. J., Nessim S., Metzger A. L., Klinenberg J. R. Lupus erythematosus in the 1980s: a survey of 570 patients. Semin Arthritis Rheum. 1991 Aug;21(1):55–64. doi: 10.1016/0049-0172(91)90057-7. [DOI] [PubMed] [Google Scholar]
- Pujol M., Ribera A., Vilardell M., Ordi J., Feliu E. High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus. Br J Haematol. 1995 Jan;89(1):137–141. doi: 10.1111/j.1365-2141.1995.tb08919.x. [DOI] [PubMed] [Google Scholar]
- Reveille J. D., Bartolucci A., Alarcón G. S. Prognosis in systemic lupus erythematosus. Negative impact of increasing age at onset, black race, and thrombocytopenia, as well as causes of death. Arthritis Rheum. 1990 Jan;33(1):37–48. doi: 10.1002/art.1780330105. [DOI] [PubMed] [Google Scholar]
- Rubin L. A., Urowitz M. B., Gladman D. D. Mortality in systemic lupus erythematosus: the bimodal pattern revisited. Q J Med. 1985 Apr;55(216):87–98. [PubMed] [Google Scholar]
- Studenski S., Allen N. B., Caldwell D. S., Rice J. R., Polisson R. P. Survival in systemic lupus erythematosus. A multivariate analysis of demographic factors. Arthritis Rheum. 1987 Dec;30(12):1326–1332. doi: 10.1002/art.1780301202. [DOI] [PubMed] [Google Scholar]
- Swaak A. J., Nossent J. C., Bronsveld W., Van Rooyen A., Nieuwenhuys E. J., Theuns L., Smeenk R. J. Systemic lupus erythematosus. I. Outcome and survival: Dutch experience with 110 patients studied prospectively. Ann Rheum Dis. 1989 Jun;48(6):447–454. doi: 10.1136/ard.48.6.447. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Urowitz M. B., Bookman A. A., Koehler B. E., Gordon D. A., Smythe H. A., Ogryzlo M. A. The bimodal mortality pattern of systemic lupus erythematosus. Am J Med. 1976 Feb;60(2):221–225. doi: 10.1016/0002-9343(76)90431-9. [DOI] [PubMed] [Google Scholar]
- Vlachoyiannopoulos P. G., Karassa F. B., Karakostas K. X., Drosos A. A., Moutsopoulos H. M. Systemic lupus erythematosus in Greece. Clinical features, evolution and outcome: a descriptive analysis of 292 patients. Lupus. 1993 Oct;2(5):303–312. doi: 10.1177/096120339300200505. [DOI] [PubMed] [Google Scholar]
- Wallace D. J., Podell T., Weiner J., Klinenberg J. R., Forouzesh S., Dubois E. L. Systemic lupus erythematosus--survival patterns. Experience with 609 patients. JAMA. 1981 Mar 6;245(9):934–938. doi: 10.1001/jama.245.9.934. [DOI] [PubMed] [Google Scholar]
- Ward M. M., Pyun E., Studenski S. Causes of death in systemic lupus erythematosus. Long-term followup of an inception cohort. Arthritis Rheum. 1995 Oct;38(10):1492–1499. doi: 10.1002/art.1780381016. [DOI] [PubMed] [Google Scholar]