Sònia Jiménez

Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults

OBJECTIVE To define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE). METHODS Prospective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE. RESULTS Thirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no difference compared with the adult onset group). Mean age of this group at disease onset was 11 years (range 5–14) compared with 32 years (15–48) for the remaining patients. The childhood onset patients more often had nephropathy (20%v 9% in adult onset SLE, p=0.04; OR:2.7; 95%CI:1.1, 7), fever (41% v 21%, p=0.006; OR:2.6, 95%CI:1.2, 5.7), and lymphadenopathy (6%v 0.5%, p=0.03, OR: 12.3, 95%CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p=0.002; OR:3.7; 95%CI:1.5, 9.5) and chorea (9% v 0%, p<0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p=0.017; OR:2.9, 95%CI:1.2, 6.8). No significant differences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15% v 6%, p=0.00004; OR:11.2; 95%CI:2.8, 44.9) but no differences were detected between the groups concerning side effects or drug toxicity. CONCLUSIONS The presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease.

Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center.

Cryoglobulins are immunoglobulins (Ig) that precipitate when serum is incubated at temperatures lower than 37 C. The existence of circulating cryoglobulins (cryoglobulinemia) is not always related to the presence of symptomatology, and the term “cryoglobulinemic syndrome” is used when patients present clinical manifestations (44). In 1933, Wintrobe and Buell (55) described cryoprecipitation as a laboratory phenomenon. In 1966, Meltzer and Franklin (27) described the typical clinical symptoms associated with cryoglobulinemia, particularly the triad of purpura, arthralgia, and weakness. In 1974, Brouet et al (7) classified cryoglobulins into 3 types: Type I cryoglobulins were those composed of single monoclonal immunoglobulins, and types II and III were those formed by monoclonal (type II) or polyclonal (type III) IgM with rheumatoid factor activity plus the corresponding antigen (usually polyclonal IgG). For this reason, types II and III are classically referred to as “mixed cryoglobulinemia.” Recently, new types of cryoglobulins have been described (41). Cryoglobulins have been observed in a wide variety of diseases, including malignancies, infections, and systemic autoimmune diseases (17, 40). When there is no demonstrable underlying disease, the condition is called “essential cryoglobulinemia.” Since the initial report (36) in 1990 of the association between cryoglobulinemia and hepatitis C virus (HCV) infection, it has become clear that most cases of socalled essential cryoglobulinemia are in fact associated with HCV infection. This has led to important changes in the etiology, classification, and treatment of cryoglobulinemia in the last 10 years. Several studies have analyzed cryoglobulinemia in patients with HCV infection, but studies performed in groups of patients covering all etiologies of cryoglobulinemia are scarce (7, 14, 17, 19, 30). In this study, we analyzed the etiology, clinical manifestations, and immunologic features of a large series of consecutive patients with cryoglobulinemia from a single center (Hospital Clinic, Barcelona, Spain).

Hepatitis C virus infection mimicking systemic lupus erythematosus : Study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus

OBJECTIVE To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in patients with systemic lupus erythematosus (SLE). METHODS We investigated 134 consecutive SLE patients (121 women and 13 men; mean age 35 years) who fulfilled the 1982 revised criteria for SLE of the American College of Rheumatology. Two hundred consecutive volunteer blood donors were also studied. Serum from all patients and controls was tested for antibodies to HCV (by third generation enzyme-linked immunosorbent assay and confirmed by third generation recombinant immunoblot assay [RIBA-3]). RESULTS Antibodies to HCV were present in 18 patients with SLE (13%) and in 2 (1%) of the blood donors studied. Among the anti-HCV-positive group, HCV infection was confirmed (by RIBA-3 and polymerase chain reaction) in 15 SLE patients (11%) and in the 2 blood donors (1%) (P < 0.001). We observed a lower frequency of cutaneous SLE features (40% versus 76%; P = 0.01) and positivity for anti-double-stranded DNA (anti-dsDNA) (33% versus 81%; P < 0.001), and a higher frequency of hepatic involvement (93% versus 2%; P < 0.001), low C4 levels (73% versus 39%; P = 0.002), low CH50 levels (73% versus 44%; P = 0.03), and cryoglobulins (60% versus 22%; P = 0.02) in SLE patients with HCV infection compared with SLE patients without infection. CONCLUSION The prevalence of HCV infection in SLE patients was higher than in blood donors from the same geographic area. SLE HCV-positive patients showed a lower frequency of cutaneous SLE features and anti-dsDNA antibodies, and a higher prevalence of liver involvement, hypocomplementemia, and cryoglobulinemia. HCV testing should be considered in the diagnosis of SLE, especially in patients who lack the typical cutaneous features of SLE or who have low titers of autoantibodies, cryoglobulinemia, or liver involvement.

The epidemiology of systemic lupus erythematosus

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. Although previously considered a rare disease, SLE now appears to be relatively common in certain groups of the population. This is probably due to the development of several immunological tests that have allowed the description of many atypical or benign cases that otherwise might not be diagnosed. Furthermore, with the introduction since 1982 of a set of more sensitive criteria for SLE classification, more cases can nowadays be detected. In the present article, we review the most important data regarding the incidence and prevalence of this disease in the general population, the epidemiologic information on the patterns of disease expression in specific subsets and the studies on mortality in SLE. An important amount of information comes from the data obtained from the “Euro-Lupus Cohor”, a series of 1000 patients with SLE from several European countries that have been followed prospectively since 1991.

Cryoglobulinemia in primary Sjögren's syndrome: Prevalence and clinical characteristics in a series of 115 patients

OBJECTIVES To determine the prevalence and nature of cryoglobulins in a large series of patients with primary Sjögrens syndrome (SS) and identify the clinical and immunologic features related to their presence. METHODS In a cross-sectional study, we investigated 115 consecutive patients (107 women and eight men) with primary SS. All patients fulfilled four or more of the preliminary diagnostic criteria for SS proposed by the European Community Study Group in 1993. Serum cryoglobulinemia was measured in all patients. Serum samples were obtained at 37 degrees C, and cryoglobulinemia was estimated by centrifugation after incubation at 4 degrees C for 7 days. The type of cryoglobulinemia was identified by agarose gel electrophoresis and immunofixation. RESULTS Cryoglobulins were detected in the sera of 18 (16%) of our patients with primary SS; most were IgMkappa monoclonal/IgG polyclonal. When compared with patients without cryoglobulins, those with cryoglobulins presented a higher prevalence of leukocytoclastic cutaneous vasculitis (56% v8%, P < .001), hypocomplementemia (75% v 2%; P < 0.001) and antibodies to hepatitis C virus (HCV) (47% v8%, P < .001). Liver involvement (clinical signs, biochemical features, or ultrasound/histological data of liver disease) was present in all patients (100%) with cryoglobulins and HCV infection but in only 11% of patients with cryoglobulins without HCV infection (P < .001). CONCLUSIONS Leukocytoclastic cutaneous vasculitis, hypocomplementemia, and HCV infection are associated with the presence of cryoglobulins in the sera of patients with primary SS. Testing for HCV infection is recommended for patients with SS and cryoglobulinemia because of its high prevalence and its strong association with liver disease.

Análisis de la actividad de una unidad estructural de dolor torácico en un servicio de urgencias hospitalario

Introduccion y objetivos Establecer la prevalencia y caracteristicas de los pacientes con dolor toracico (DT) no traumatico y la calidad de proceso de una unidad de dolor toracico (UDT) estructural. Calcular teoricamente la dimension de futuras UDT estructurales. Metodos Estudio prospectivo que incluyo, en un grupo, a 1.000 pacientes consecutivos visitados por DT en la UDT y, en el otro, al resto de pacientes visitados por otros motivos. Se recogieron datos clinicos, diagnostico final, destino, tiempo de espera y tiempo hasta el alta. En la UDT, ademas se registro el tiempo puerta-ECG y, cuando procedia, el puerta-aguja y el puerta-balon. Para el dimensionado, se utilizo el numero de pacientes con DT y el tiempo hasta el alta o ingreso. Resultados La prevalencia del DT fue del 4,4% (22.468 visitas totales). Comparados con el resto, los pacientes con DT eran mas frecuentemente varones, de mayor edad, esperaron menos, pero ingresaron mas. De los 1.000 pacientes, el 25,9% tenia un sindrome coronario agudo (SCA), el 64,7% no resentaba un SCA, y el 9,4% quedo sin diagnostico por no efectuarse una prueba de esfuerzo (PE). Los pacientes con SCA eran mayores y con mas factores de riesgo, pero sin diferencias de sexo. El tiempo puerta-ECG fue de 10 min, el puerta-aguja de 26 min, y el puerta-balon de 51 min. Se precisaria una camilla de UDT por cada 13.000 urgencias anuales. Conclusiones La prevalencia y diferente perfil de los pacientes con DT apoyan el desarrollo de UDT estructurales. Se detectan limitaciones en el uso de la PE, pero se alcanzan los estandares de calidad de proceso del SCA.

Double heterozygosity polymorphisms for platelet glycoproteins Ia/IIa and IIb/IIIa increases arterial thrombosis and arteriosclerosis in patients with the antiphospholipid syndrome or with systemic lupus erythematosus

Objective: We analysed the genetic polymorphisms in platelet glycoproteins (GP) Ib-α, Ia/IIa and IIb/IIIa and their correlation with the development of arterial thrombosis and preclinical arteriosclerosis in patients with antiphospholipid syndrome (APS) or with systemic lupus erythematosus (SLE). Methods: We included 131 patients with APS (86 with primary APS and 45 with APS associated with SLE), 102 patients with SLE and 160 healthy controls. GP Ib-α VNTR polymorphism, GP Ia/IIa 807 C/T polymorphism and GP IIb/IIIa PlA1/2 polymorphism were determined by polymerase chain reaction. Thrombotic events were assessed clinically and confirmed by objective methods. The presence of preclinical arteriosclerosis was evaluated by a carotid ultrasound study in a subgroup of 70 patients with SLE measuring the intima-media wall thickness and the presence of arteriosclerotic plaque. Results: A total of 50 episodes of arterial thrombosis in 36 patients with APS have been registered. We found a significant correlation between the 807 T/T genotype of GP Ia/IIa and arterial thrombosis (22% vs 7%, p = 0.04; OR 3.59, 95% CI 1.20 to 10.79). The VNTR Ib-α and P1A1/2 IIb/IIIa polymorphisms were not associated with arterial thrombosis in patients with APS when individually analysed. The coexistence of both 807 T and PlA2 alleles increased the arterial thrombosis risk (28% vs 7%, p = 0.005; OR 4.84, 95% CI 1.67 to 13.96). In patients with SLE, no relationship was found between the presence of carotid arteriosclerotic plaque and separate polymorphisms of platelet GP. The coexistence of alleles 807 T of GP Ia/IIa and PlA2 of GP IIb/IIIa was associated with the presence of carotid plaque (35% vs 4%, p = 0.002; OR 12.92, 95% CI 2.39 to 69.81). Conclusions: The T/T genotype of 807 C/T polymorphism of GP Ia/IIa may be an additional risk for the development of arterial thrombosis in APS. The coexistence of both 807 T and PlA2 alleles increased the arterial thrombosis risk in patients with APS and preclinical arteriosclerosis in patients with SLE.

Saturación del servicio de urgencias: factores asociados y cuantificación

Fundamento y objetivo La utilizacion de los servicios de urgencias hospitalarios (SUH) es cadavez mayor, lo que conduce a su masificacion. El objetivo del presente trabajo es definir la «saturacion» de un SUH y determinar y cuantificar los factores que la condicionan. Pacientes y metodo Durante tres semanas consecutivas de anos distintos (2000-2002) se contabilizaroncada 3 h las entradas, el indice de ocupacion (IO) de los pacientes que permanecianen el SUH, en el area de primera asistencia (APA) y en el area de observacion (AO) segun lacausa de dicha permanencia. Los datos se sometieron a analisis de regresion logistica multiplecon la variable dependiente «saturacion/no saturacion» de cada una de las areas (SUH, APA yAO). Se definio la saturacion cuando el IO era igual o superior al 100%. Los modelos de cadaarea se calibraron por la prueba de Hosmer-Lemeshow y se discriminaron por metodologia ROC.Los modelos explicativos se armaron separando aleatoriamente dos grupos: seleccion (88% dedatos) y validacion (12% de datos). Resultados Las variables que se asociaron de forma significativa a la saturacion en el modelodel SUH fueron el IO debido a los pacientes que esperaban resultados, ir a una cama, encontrarcama, exploraciones complementarias y en evolucion. En el modelo del APA, lo fueron elIO debido a los que estaban visitandose y esperaban resultados. Finalmente, para el modelodel AO lo fueron el IO debido a los que esperaban ir a una cama, encontrar cama, exploracionescomplementarias y en evolucion. Todos los modelos mostraron sensibilidad y especificidadsuperiores al 85% y un area ROC superior a 0,97. En ningun caso el numero de pacientes queacuden a urgencias participo del modelo explicativo final. En el grupo de validacion se confirmaronestos resultados. Conclusiones Los pacientes que permanecen en el servicio de urgencias por factores dependientestanto del hospital (esperando ir a una cama o encontrar una cama) como del propio serviciode urgencias (esperando evolucion) son la principal causa de saturacion de los SUH.

Efectividad de la incorporación de un médico especialista en medicina familiar y comunitaria en un servicio de urgencias hospitalario

Fundamento y objetivos Conocer el efecto que tiene la incorporacion de un medico especialista en medicina familiar y comunitaria (MFC) en la efectividad, la eficiencia y la calidad de un servicio de urgencies hospitalario (SUH). Pacientes y metodo Estudio prospectivo e intervencionista realizado en el area de visita rapida (AVR) que idealmente esta abierta de las 8.00 a las 24.00 h y asistida por dos residentes. La intervencion consistio en sustituir 8 h de guardia de un residente (16.00 a 24.00) por 8 h de guardia de un especialista de MFC. El periodo de estudio fue agosto de 2002 (presencia de un especialista de MFC) y el periodo control, octubre de 2002. De forma aleatoria se evaluaron 10 dias y 100 pacientes de cada periodo. Se analizaron para cada dia las siguientes variables: numero de visitas (P), revisitas en 72 h y fugados, tiempo de apertura (TA) real del AVR y porcentaje de observaciones e ingresos. Se analizaron para cada paciente diversos aspectos: caracteristicas clinicas y epidemiologicas, tiempo de espera para ser visitado (TE), numero de exploraciones complementarias realizadas, tiempo de demora en la administracion del tratamiento (TT), tiempo total desde el inicio de la visita hasta la salida (TO) y numero de pacientes dados de alta sin necesidad de exploraciones complementarias o consulta a otros especialistas de guardia del hospital. Se realizo un analisis de la calidad percibida mediante encuesta telefonica. Se definieron y calcularon 3 indices de efectividad: P/TE (E1), P/TA (E2) y valoracion global de la visita/valoracion del TE (E3). Finalmente, se calcularon los costes (C) fijos y variables de ambos periodos, y se realizo un analisis coste-efectividad (C/E). Resultados El numero de visitas y las caracteristicas clinicas y epidemiologicas de los pacientes fueron similares en ambos periodos. Durante el periodo de estudio (presencia de MFC) todas las variables mejoraron significativamente: reduccion del 20% en el TE, el 25% en el TT, el 36% en el TO y el 17,5% en el TA. Se registro una reduccion del 41% en el numero de exploraciones complementarias, del 78% en el porcentaje de observaciones y del 75% en el porcentaje de revisitas. Finalmente, el E1 y el E2 mejoraron un 77 y un 51%. El analisis coste-efectividad mostro una disminucion, durante el periodo de estudio, para el C/E1 del 55%, para el C/E2 del 33% y para el C/E3 del 6%. La encuesta telefonica no revelo diferencias en ambos periodos pero el TE durante el periodo de estudio fue percibido con mas frecuencia como adecuado. Conclusiones La presencia de un medico de MFC en el AVR supuso una mejoria de la efectividad y de la calidad percibida por los pacientes. Ademas, esta presencia fue eficiente. Por ello, es una intervencion que deberia ser considerada por la administracion para una mejor utilizacion de los SUH.

Splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.

The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive Coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.