Systemic lupus erythematosus in Europe at the change of the millennium: lessons from the "Euro-Lupus Project".

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium--the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Lessons from the "Euro-Lupus Cohort".

The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Priones: concepto y enfermedades / Prions: definition and diseases

En este artículo se revisan el concepto y la terminología de los priones, su replicación y algunas hipótesis actuales de la naturaleza de estos agentes infecciosos que causan enfermedades neurodegenerativas. También se resumen los aspectos etiopatogénicos, epidemiológicos, clínicos y neuropatológicos de las enfermedades producidas por priones o encefalopatías espongiformes transmisibles humanas, y algunos métodos para su rápido diagnóstico. Finalmente, se comenta la posible relación entre la encefalopatía espongiforme bovina y los nuevos casos de enfermedad de Creutzfeldt-Jakob identificados en el Reino Unido (AU)

[Prions: definition and diseases]. / Priones: concepto y enfermedades.

In this article we review the concept and terminology of prions, their replication and some current hypothesis on the nature of these infectious agents causing neurodegenerative diseases. This revision also summarizes the etiopathogenic, epidemiological, clinical and neuropathological features of the prion diseases or human transmissible spongiform encephalopathies, and some methods for their early diagnosis. Finally, we discuss the possible link between the bovine spongiform encephalopathy and the new cases of Creutzfeldt-Jakob disease identified in the United Kingdom.

[Acute myocardial infarction secondary to Wegener's granulomatosis]. / Infarto agudo de miocardio secundario a granulomatosis de Wegener.

Wegener's granulomatosis (WG) is a necrotizing and granulomatous vasculitis that usually affects the upper and lower respiratory tract and the kidneys. Cardiac involvement is rare although pericarditis, coronary arteritis, myocarditis, valvulitis and arrhythmias have been described. Acute myocardial infarction with clinical expression is an exceptional complication of Wegener's granulomatosis. We report a case of a 30-year-old man with Wegener's granulomatosis who suffered an acute myocardial infarction during the initial phase of the disease, following seven days of treatment with glucocorticoids and cyclophosphamide. Transthoracic echocardiography showed abnormal regional wall motion with septal hypokinesia and apical akinesia. Cardiac catheterization revealed an ectasic segment in the proximal left anterior descending coronary artery and total occlusion in the mid-segment. Medical therapy with prednisone and cyclophosphamide was continued. No complications and initial remission were achieved.

[Prevalence of psychiatric disturbances and psychopathologic status in patients with systemic lupus erythematosus]. / Prevalencia de trastornos psiquiátricos y estado psicopatológico en pacientes con lupus eritematoso sistémico.

BACKGROUND: Psychiatric disorders are commonly associated with systemic lupus erythematosus (SLE). Some authors consider that these disorders can be associated with cognitive impairment. OBJECTIVES: The objectives of this study were to establish the particular prevalence of psychiatric disorders in these patients and to investigate the relationship between psychiatric disorders, cognitive impairment, life quality, psychological status and adjustment to illness. MATERIAL AND METHODS: A cross-sectional study is reported of 46 spanish patients with systemic lupus erythematosus (SLE) attending the outpatients clinics of the Internal Medicine and Rheumatology Departments at a University Hospital. The following instruments were used: a Structured Clinical Interview for DSM-III-R, the Nottingham Health Profile, the Symptom Check-List 90-Revised, the Psychological Adjustment to Illness Scale and the Integrated Program of Neuropsychological Assessment Test-Barcelona. RESULTS: The prevalence of psychiatric disorders at interview was 33%. The most common diagnoses were affective disorders and psycho-organic syndrome. Affective disorders were associated with a poorer life quality, number of hospitalizations and psychiatric background. Adjustment to illness accounted for the psychologic status of the patients.

Hepatic amyloidosis associated with systemic lupus erythematosus.

The association between amyloidosis and systemic lupus erythematosus has rarely been described. We report a case of a 37-year-old man with a long-standing SLE who developed clinical and laboratory signs of hepatic dysfunction. A liver biopsy revealed secondary amyloidosis.