Systemic lupus erythematosus in Europe at the change of the millennium: Lessons from the “Euro-Lupus Project”
Introduction
Systemic lupus erythematosus (SLE) is the most diverse of the autoimmune diseases because it may affect any organ of the body and display a broad spectrum of clinical and immunological manifestations. One question that arises is whether the age at onset of the disease, the sex, or the autoantibody pattern, among other factors, can modify the disease expression and define some specific SLE subsets. Several investigators have addressed this problem with controversial results. This is probably due to the small number of patients that have been analyzed, the disparity in selection criteria for patient inclusion, or the definition of the variables.
On the other hand, in recent years, both SLE morbidity and mortality have been modified due to a number of possible reasons, including a better knowledge of its pathogenetic mechanisms and prognostic factors as well as the use of immunosuppressive regimes. It has been suggested that the spectrum of clinical manifestations as well as the causes of death are different depending on the time of evolution of the disease. Furthermore, it has been postulated that SLE tends to enter into remission in many patients after a long time of evolution.
In an attempt to clarify the long-term evolution of patients with SLE in Europe, the “Euro-Lupus Project” was started in 1991. The “Euro-Lupus” cohort was composed by 1000 patients with SLE that were gathered by a consortium that included more than 40 investigators from seven European countries. This consortium was originated as part of the network promoted by the “European Working Party on SLE” [1].
Section snippets
Objectives of the “Euro-Lupus Project”
The "Euro-Lupus Project” started with a multicenter, consecutive and prospective design. In order to gather a sizeable series of patients, twelve tertiary referral university centers agreed to take part in the study. The final cohort included 1000 unselected patients that were prospectively followed during 10 years. The objectives of the project were: i) to determine the incidence of the different clinical manifestations at the onset of the disease and during the follow-up; ii) to analyze the
General characteristics of the “Euro-Lupus Cohort”
The general characteristics of this cohort at the beginning of the study were published in 1993 [2]. Although SLE is being recognized with increasing frequency in medical practice, the diversity of its clinical and laboratory features at presentation makes precise diagnosis a real challenge for the clinician and this has been reflected in the “Euro-LupusProject”. In this cohort, less than a half of the patients presented the typical SLE malar rash at the onset of the disease. On the contrary, a
Patterns of disease expression in specific SLE subsets
An important question that has been raised by several authors is whether the age at onset of the disease, the gender, or the autoantibody pattern, among other factors, can modify the disease expression and define some specific SLE subsets.
Morbidity and mortality during the follow-up
The natural history of SLE is characterized by episodes of relapses or flares, interchanging with remissions, and the outcome is highly variable ranging from permanent remission to death. However, both morbidity and mortality have been modified in recent years due to a number of possible reasons, including the more conservative use of corticosteroids and of modified immunosuppressive regimens. Additionally, a better knowledge of the prognostic factors for morbidity and mortality can improve the
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List of authors of the “European Working Party on Systemic Lupus Erythematosus” In Alphabetical Order: M. Abarca-Costalago, D. Abramovicz, F. Allegri, P. Annunziata, A.O. Aydintug, M.R. Bacarelli, F. Bellisai, I. Bernardino, E. Biernat-Kaluza, D. Blockmans, K. Boki, L. Bracci, V. Campanella, M.T. Camps, C. Carcassi, R. Cattaneo, A. Cauli, R. Cervera, H. Chwalinska-Sadowska, L. Contu, J.P. Cosyns, M.G. Danieli, D. D´Cruz, G. Depresseux, H. Direskeneli, I. Domènech, G. Espinosa, A. Fernández-Nebro, G.B. Ferrara, J. Font, M.A. Frutos, M. Galeazzi, M. García-Carrasco, M.F. García Iglesias, A. García-Tobaruela, J. George, A. Gil, P. González-Santos, M. Grana, A. Gül, H.J. Haga, M. de Haro-Liger, F. Houssiau, G.R.V. Hughes, M. Ingelmo, A. Jedryka-Góral, M.A. Khamashta, P. Lavilla, Y. Levi, M. López-Dupla, A. López-Soto, H. Maldykowa, R. Marcolongo, A. Mathieu, G. Morozzi, N. Nicolopoulou, C. Papasteriades, G. Passiu, I. Perelló, P. Petera, R. Petrovic, J.C. Piette, V. Pintado, O. de Pita, R. Popovic, G. Pucci, P. Puddu, E. de Ramón, M. Ramos-Casals, J. Rodríguez-Andreu, G. Ruiz-Irastorza, J. Sánchez-Lora, G. Sanna, R. Scorza, G.D. Sebastiani, Y. Sherer, Y. Shoenfeld, A. Simpatico, R.A. Sinico, J. Smolen, A. Tincani, G. Tokgöz, A. Urbano-Márquez, C. Vasconcelos, J.J. Vázquez, M. Veronesi, J. Vianna, and J. Vivancos.