R. M. Pujol

Mild presentation of systemic lupus erythematosus in elderly patients assessed by SLEDAI

Objective: Systemic lupus erythematosus (SLE) predominantly affects young patients. SLE starting in later life has a clinical presentation different than in younger patients. We have used the SLE Disease Activity Index (SLEDAI) to explore the relationship between age of onset and disease activity. Methods: We selected all patients controlled in our hospital at the moment of clinical diagnosis of SLE (100 patients; 85 females and 15 males). They were classified in two groups: those with early onset (<50 y) and those with late onset (>50 y) based on their age at the moment of clinical diagnosis of SLE. Results: In 12 patients the onset of SLE was > 50 y (10 females and two males; mean age 59 y). The early onset patients had significantly higher SLEDAI values at the presentation and during the first year of disease with respect to elderly patients. Antibodies to DNA and hypocomplementemia were detected more often in younger patients. Conclusion: Our results confirm using SLEDAI, that the lupus of the elderly patients is a distinct clinical subgroup with a milder course of disease.

Lipid and lipoprotein levels in premenopausal systemic lupus erythematosus patients.

The purpose of this study was to assess the prevalence of dyslipoproteinemia and to analyze the clinical variables that are associated with it in a sample of premenopausal systemic lupus erythematosus (SLE) patients. We studied 53 premenopausal (34.5 y) SLE outpatients and 45 controls. Clinical variables studied included patient age, weight, height, body mass index (BMI), age at disease onset, disease duration, clinical activity of SLE, renal involvement and drug therapy. Total cholesterol (TC), high-and low-density lipoprotein cholesterol (HDL-C and LDL-C), and triglycerides were measured using standard enzymatic techniques. Apolipoproteins (apo) A-I and B were determined by radial immunodiffusion. Twenty-nine patients (55%) and 14 controls (30%) had dyslipoproteinemia. An increase in TC, triglycerides, HDL3-C, apo A-I and apo B, and a decrease in HDL2-C and HDL-C/TC index was found in SLE patients in comparison with controls. TC (P ‘ 0.007), apo B (P ‘ 0.02), LDL-C (P ‘ 0.03) and triglycerides (P ‘ 0.0001) were significantly correlated with proteinuria. Patients on prednisone therapy had higher triglycerides levels (P ‘ 0.03) than untreated patients. TC (P ‘ 0.01), LDL-C (P ‘ 0.006) and triglycerides (P ‘ 0.04) were also correlated with the dose of prednisone. Dyslipoproteinemia is a common feature in adult SLE premenopausal patients which is characterized by an increase in TC, triglycerides and apo B, and an abnormal distribution of HDL subclasses. Corticosteroid therapy and proteinuria are the best predictors of dyslipoproteinemia in these patients.

Significant reductions of HIV prevalence but not of hepatitis C virus infections in injection drug users from metropolitan Barcelona: 1987–2001

OBJECTIVES To characterize trends from 1987 to 2001 in the prevalence of HIV and HCV infections among 2219 injection drug users (IDUs) starting treatment for substance abuse in two large hospitals in metropolitan Barcelona. METHODS The study population comprised IDUs with HIV tests completed from 1987 to 2001 and admitted for detoxification. Testing for HCV started in 1991 (n=1132). Characterization of temporal trends was carried out using logistic regression methods. Stratification was used to describe possible heterogeneities of the temporal trends. RESULTS The overall prevalence of HIV, HCV, and HBV (HBsAg+) was 55%, 88%, and 7%, respectively. Adjusted by duration of IDU, sex, and age at initiation, the prevalence of HIV infection declined significantly (p<0.001) from 1989 to 2004. The substantially higher prevalence of HCV showed a decline (p=0.065) of lesser magnitude. The decline of HIV infection was consistently observed among those with duration of IDU of less than 10 years. In turn, the decline of HCV was restricted to those with short duration of IDU (<4 years) because the prevalence of HCV infection was close to 100% for durations longer than 4 years in all calendar periods. CONCLUSIONS Preventive interventions and treatment for substance abuse might have contributed to the waning of the HIV epidemic in Spain. However, the extremely high levels of HCV infection and the underlying prevalence of HIV might lead to a large health burden of liver disease.

Morir en el hospital por enfermedad terminal no oncológica: análisis de la toma de decisiones

Dado el progresivo envejecimiento de la poblacion, ha aumentado el porcentaje de ancianos afectados de enfermedades cronicas en fase terminal, que acaban falleciendo en un hospital de agudos 1 . Por tanto, es basico que en dichos hospitales se otorgue al paciente terminal el derecho a morir con dignidad, pudiendo elegir y gestionar su propia vida, sus condiciones y su final 2 . En trabajos previos nuestro grupo ha detectado una baja implantacion de medidas paliativas en pacientes que fallecian por insuficiencia cardiaca congestiva (ICC) 3 terminal o en pacientes con demencia en fase terminal 4

Are the Pulmonary Function Tests and the Markers of Activity Helpful to Establish the Prognosis of Sarcoidosis

The prognosis of sarcoidosis is difficult to establish. It depends mainly on the persistence of activity over time and the degree of functional impairment of the involved organs. The aim of this study was to analyze whether the pulmonary function tests and the more commonly used markers of activity, such as serum angiotensin-converting enzyme (SACE) and gallium-67 scan, are helpful to evaluate the prognosis of sarcoidosis, besides the clinical data. Over a 14-year period (1974-1987), 209 patients were diagnosed as having sarcoidosis at the Bellvitge Hospital, a 1,000-bed teaching hospital in Barcelona, Spain. Clinical, radiological, pulmonary function tests, and activity markers (SACE and gallium-67 scan) data at diagnosis were collected and classified as variables, and a definition of disease activity was established. One hundred sixteen patients were on follow-up, had all the variables available and were included in the statistical analysis. A Cox proportional-hazard regression model identified the following variables as independently influencing the persistence of activity over time: absence of erythema nodosum [risk ratio (RR) = 2.78; 95% confidence interval (CI): 1.48-5.18], age > or = 40 years (RR = 1.67; 95% CI: 1.008-1.04), SACE level > or = mean + 2 SD U/ml (RR = 1.45; CI: 0.99-1.07), hyperglobulinemia (RR = 2.47; CI: 0.98-6.24), forced vital capacity < 80% predicted (RR = 2.17; CI: 0.97-4.85), and male sex (RR = 1.8; CI: 0.95-3.45). We conclude that the pulmonary function tests and the SACE level but not the gallium scan are helpful to identify the factors predicting persistence of activity in sarcoidosis. Therefore, we recommend to add these tests to the initial clinical evaluation of patients with sarcoidosis in order to establish the prognosis and improve the therapeutic approach.

Influence of serum amyloid A on the decrease of high density lipoprotein-cholesterol in active sarcoidosis

OBJECTIVE We have previously observed low levels of high density lipoprotein (HDL) cholesterol in active sarcoidosis. The aim of this study was to analyze the role of serum amyloid A (SAA) on this lipid disorder. METHODS Eighty five untreated sarcoid patients, 40 with active disease and 45 with inactive disease, were recruited. Sarcoidosis activity was evaluated by means of clinical, chest X-ray, gallium-67 scan, serum angiotensin converting enzyme (peptidyl-dipeptidase A) values, and pulmonary function tests. Analysis of lipoprotein metabolism included: serum cholesterol, low density lipoprotein (LDL)-cholesterol, HDL-cholesterol, HDL(2)-cholesterol, HDL(3)-cholesterol, apolipoprotein A-I (apo A-I), apolipoprotein B (apo B), and triglyceride concentrations. Serum amyloid A protein and lecithin-cholesterol acyltransferase (LCAT) activity were measured. RESULTS In active sarcoidosis we found significantly reduced levels of HDL-cholesterol (1.17+/-0.36 vs. 1. 44+/-0.39 mmol/l, P=0.002), HDL(3)-cholesterol (0.78+/-0.23 vs. 1. 02+/-0.21 mmol/l, P<0.0001), and apo A-I (1.36+/-0.29 vs. 1.61+/-0. 27 g/l, P<0.0001) and significantly increased levels of triglyceride (1.51+/-0.64 vs. 1.03+/-0.46 mmol/l, P<0.0001), and apo B (1.14+/-0. 25 vs. 0.99+/-0.27 g/l, P=0.012) versus inactive sarcoidosis. Serum amyloid A concentrations were significantly increased in the patients with active disease (155.45+/-154.01 mg/ml) compared to the inactive sarcoid patients (89.70+/-65.36 mg/ml) (P=0.011). There were no significant differences in cholesterol, LDL-cholesterol, HDL(2)-cholesterol or LCAT values between groups. Multivariate logistic regression analysis showed that HDL-cholesterol (regression coefficient b=-1.96; S.E.=0.87; P=0.02) and SAA (regression coefficient b=0.01; S.E.=0.004; P=0.01) were the two variables independently associated with disease activity. Moreover, a significant negative correlation was observed between SAA levels and both HDL-cholesterol (r=-0.39; P=0.01) and apo A-I (r=-0.35; P=0.03) levels, in the active sarcoid group. Conversely, no correlation was found in the inactive sarcoid group. CONCLUSION The low HDL-cholesterol and apo A-I concentrations seen in active sarcoid patients are associated with a significant increase of SAA levels. We suggest that the displacement of apo A-I by SAA on HDL accounts for the lower level of HDL-cholesterol seen in active sarcoidosis.

Documento de consenso sobre el diagnóstico y tratamiento del síndrome de fatiga crónica en Catalunya

La fatiga prolongada, entendida como una sensación persistente de agotamiento o dificultad para realizar una actividad física o intelectual continuada, es un síntoma prevalente, tanto en atención primaria como especializada, además de motivo de considerable preocupación tanto para el paciente que la sufre como para el médico que le atiende. Se considera que de un 5 a un 20% de la población general puede presentar fatiga durante más de un mes en algún momento de su vida, hecho que suele estar en relación con enfermedades o situaciones intercurrentes1. Entendemos por fatiga crónica la que se presenta de forma continuada o intermitente durante más de 6 meses, lo que acontece entre un 1 y un 10% de la población general1. Si esta situación tiene una causa conocida o relacionable se denominará fatiga crónica secundaria. El síndrome de fatiga crónica (SFC) debe diferenciarse de las dos situaciones previamente referidas y su definición requiere el cumplimiento de unos criterios específicos2,3. Estos criterios incluyen la presencia de fatiga persistente o intermitente, inexplicada e invalidante, que no es producto de un esfuerzo excesivo y no mejora con el descanso. Además, el paciente debe presentar de forma crónica y concurrente 4 o más síntomas de los relacionados como criterios asociados en la definición establecida para esta enfermedad (tabla 1)4. Los pacientes que presentan fatiga crónica no explicada pero que no reúnen los criterios de SFC entrarían en la situación de fatiga crónica idiopática3. Aunque en España se desconoce la prevalencia real del SFC, en un estudio poblacional realizado en 1995 en Estados Unidos5 se objetivó que era de 75 a 267 casos por 100.000 habitantes. En otro estudio poblacional australiano efectuado en 1990 se objetivó una prevalencia de 37 casos por 100.000 habitantes6. Extrapolando las cifras obtenidas en estas series a la población catalana de más de 16 años, la prevalencia estimada en Catalunya oscilaría entre 2.012 y 13.429 casos3. En los últimos años hemos asistido a un notable incremento de consultas de pacientes con fatiga, algunos de ellos con fatiga prolongada o SFC. Este hecho, junto con la ausencia de una clara etiología de este proceso3,7,8 y, por tanto, de un tratamiento satisfactorio, la importante morbilidad asociada, así como la falta de una conducta y actitud diagnóstico-terapéutica homogéneas por parte del personal asistencial, ha movido a este grupo multidisciplinario de trabajo a elaborar este documento de consenso con el fin de constatar el estado del SFC en Catalunya, así como a confeccionar una guía práctica de actuación que facilite y homogeneice la conducta a seguir en este grupo de pacientes.

Hiperhidrosis primaria: estudio prospectivo de 338 pacientes

Fundamento y objetivo: Este trabajo tiene por objetivo estudiar el perfil clinico del paciente con hiperhidrosis primaria (HP) y su repercusion social. Pacientes y metodo: La muestra acota un total de 338 pacientes con HP estudiados entre enero de 1998 y octubre de 2002. Todos cumplimentaron una encuesta epidemiologica preoperatoria donde se registraron: edad, sexo, profesion, antecedentes personales y familiares, inicio, localizacion, clinica cutanea acompanante y repercusion social de la HP. Sobre 179 pacientes se registro sintomatologia general acompanante. Resultados: La HP se inicia en la infancia en el 86% de los casos, el 71,5% son mujeres y la edad media es de 28,8 anos. Son pacientes con escasas enfermedades asociadas y con una clinica local cutanea acompanante en el 42,5% de los casos. Los familiares con HP alcanzan un 47,9% de los casos. La localizacion de la hipersudacion es palmar en el 96,4% de los casos, el 80,7% plantar, el 71,3% axilar, siendo menor en otras zonas. La clinica acompanante mas frecuente es: enrojecimiento facial (60,3%), palpitaciones (52,5%), tension muscular (48%), temblor (31,8%) y cefalea (30,8%). Las relaciones entre los amigos y el ambito laboral son las situaciones mas problematicas. Conclusiones: La HP es un trastorno de inicio en la infancia, con historia familiar de HP, con escasas enfermedades asociadas y con repercusion cutanea local. La clinica principal es la hipersudacion palmar, aunque no es exclusiva dicha localizacion. Se suele acompanar de sintomatologia indicativa de hiperfuncion simpatica como enrojecimiento facial, temblor o cefalea, sintomas dificiles de considerar si son causa o consecuencia.

Impact of Medical Comorbidity and Risk of Death in 680 Patients with Alcohol Use Disorders

BACKGROUND The association between alcohol use disorders and increased risk of mortality is well known; however, there have been few systematic evaluations of alcohol-related organ damage and its impact on survival in younger alcoholics. Therefore, we assessed medical comorbidity with a clinical index to identify subgroups of alcoholic patients at high risk of premature death. METHODS Hospital-based cohort of alcohol-dependent patients admitted for detoxification between 1999 and 2008 in Barcelona, Spain. At admission, sociodemographic characteristics and a history of alcohol dependence and abuse of illegal drugs were obtained through clinical interviews and questionnaires. Medical comorbidity was assessed with the Cumulative Illness Rating Scale (Substance Abuse) (CIRS-SA). Dates and causes of death were obtained from clinical records and death registers. Survival was analyzed using Kaplan-Meier methods, and Cox regression models were used to analyze the risk factors for premature death. RESULTS Median age of the patients (686 total, 79.7% men) was 43.5 years (interquartile range [IQR], 37.8 to 50.4), average alcohol consumption was 200 g/d (IQR, 120 to 280 g/d), and duration of alcohol use disorder was 18 years (IQR, 11 to 24). Medical comorbidity by CIRS-SA at admission showed that the organs/systems most affected were liver (99%), respiratory (86%), and cardiovascular (58%). After median follow-up of 3.1 years (IQR, 1.5 to 5.1), 78 (11.4%) patients died with a mortality rate of 3.28 × 100 person-years; according to Kaplan-Meier estimates, 50% (95% confidence interval [95% CI], 24 to 69%) of patients with severe medical comorbidity died in the first decade after treatment. In multivariate analysis, severe medical comorbidity (hazard ratio [HR], 5.5; 95% CI, 3.02 to 10.07) and being treated with methadone at admission (HR, 2.60; 95% CI, 1.50 to 4.51) were independent risk factors for premature death. CONCLUSIONS Systematic assessment of alcohol-related organ damage is relevant for the identification and treatment of those at increased risk of death.

High expression of p21Waf1 in sarcoid granulomas: a putative role for long‐lasting inflammation

In sarcoid granulomas, apoptotic events are reduced, which explains their characteristic long‐lasting inflammation. We have described that interferon‐γ (IFN‐γ) inhibits apoptosis in macrophages through the expression of p21Waf1. Here, we explore the molecular mechanisms involved in the inhibition of apoptosis in sarcoid granulomas. We analyzed skin biopsies from 19 sarcoidosis patients and 16 controls. Total RNA was subjected to semiquantitative reverse transcriptase‐polymerase chain reaction analysis. There was no difference found in the expression of proapoptotic (Bax and Bcl‐Xs) or antiapoptotic (Bcl‐2 and Bcl‐XL) genes nor in the expression of the tumor suppressor gene p53. Furthermore, the expression of IFN‐γ and the cdk inhibitors p21Waf1 and p27Kip1 were analyzed. IFN‐γ was detected in 37% of the sarcoidosis patients, and controls were negative (P<0.02). In addition, a higher proportion of patients expressing p21Waf1 (58%) versus controls (12%) was found (P<0.005). There was a significant correlation between the expression of IFN‐γ and p21Waf1 (r=0.69) and between p21Waf1 and fibronectin (r=0.65). Finally, using immunohistochemistry, high p21Waf1 reactivity was observed inside the granuloma. We conclude that the high levels of p21Waf1 in sarcoidosis may explain the absence of apoptosis in the granuloma and the persistence of inflammation.