To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions. Systemic involvement was characterized using ESSDAI definitions for the 10 clinical domains (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system and muscular). ESSDAI scores at diagnosis, during follow-up and cumulated at the last visit were calculated. The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI score of zero at the last visit. Organ by organ, the percentage of patients who developed activity during the follow-up (ESSDAI score ≥ 1 at any time) ranged between 1.4% and 56%, with articular, pulmonary and peripheral neurological involvement being the most common. Logistic multivariate regression analysis showed the following features at diagnosis and had the closest association with systemic activity (statistically significant independent variables in at least two domains): cryoglobulinaemia in five domains; anaemia, lymphopenia and low C3 levels in three domains each and age <35 years in two domains. Sicca features, ANA and RF at diagnosis were not associated with a higher cumulated activity score in any clinical domain. Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs. Cytopenias, hypocomplementaemia and cryoglobulinaemia at diagnosis strongly correlated with higher cumulated ESSDAI scores in the clinical domains. Clinically the ESSDAI provides a reliable picture of systemic involvement in primary SS.

Background: The present study aimed to assess the association of obesity and malnutrition with the mortality of hospitalised patients with acute exacerbation of chronic obstructive pulmonary disease (COPD) and the risk of readmission in <30 days.Methods: A retrospective chart review of consecutive patients admitted with COPD as the primary reason for discharge in Spain between 1 January 2006 and 31 December 2007 was performed. Patients with a diagnosis of obesity or malnutrition in the hospital discharge clinical report were identified. The in‐hospital mortality and re‐admittance 30 days after discharge indices of obese and malnourished patients were compared against the subpopulation without these diagnoses.Results: Of the 313 233 COPD admittances analysed, there were 22 582 (7.2%) diagnoses of obesity and 6354 (2.0%) diagnoses of malnutrition. In‐hospital global mortality and the re‐admittance risk were 12.0% and 16.7%, respectively. Obese patients showed a lower in‐hospital mortality risk [odds ratio (OR) = 0.52; 95% confidence interval (CI) = 0.49–0.55] and early re‐admittance risk (OR = 0.87; 95% CI = 0.85–0.92) compared to non‐obese patients. Malnourished patients had a much higher risk of death when in hospital (OR = 1.73; 95% CI = 1.62–1.85) or of being re‐admitted within 30 days after discharge (OR = 1.29; 95% CI = 1.22–1.38), even after adjusting for possible confounding factors.Conclusions: Obesity in patients hospitalised for COPD substantially reduces in‐hospital mortality risk and the possibility of early re‐admittance. Malnutrition is associated with an important increase in in‐hospital mortality and risk of re‐admittance in the 30 days following discharge.

Introduction: The purpose of this observational study was to analyze the rates, characteristics and associated risk factors of severe infections in patients with systemic autoimmune diseases (SAD) who were treated off-label with biological agents in daily practice. Methods: The BIOGEAS registry is an ongoing Spanish prospective cohort study investigating the long-term safety and efficacy of the off-label use of biological agents in adult patients with severe, refractory SAD. Severe infections were defined according to previous studies as those that required intravenous treatment or that led to hospitalization or death. Patients contributed person-years of follow-up for the period in which they were treated with biological agents. Results: A total of 344 patients with SAD treated with biological agents off-label were included in the Registry until July 2010. The first biological therapies included rituximab in 264 (77%) patients, infliximab in 37 (11%), etanercept in 21 (6%), adalimumab in 19 (5%), and 'other' agents in 3 (1%). Forty-five severe infections occurred in 37 patients after a mean follow-up of 26.76 months. These infections resulted in four deaths. The crude rate of severe infections was 90.9 events/1000 person-years (112.5 for rituximab, 76.9 for infliximab, 66.9 for adalimumab and 30.5 for etanercept respectively). In patients treated with more than two courses of rituximab, the crude rate of severe infection was 226.4 events/1000 person-years. A pathogen was identified in 24 (53%) severe infections. The most common sites of severe infection were the lower respiratory tract (39%), bacteremia/sepsis (20%) and the urinary tract (16%). There were no significant differences relating to gender, SAD, agent, other previous therapies, number of previous immunosuppressive agents received or other therapies administered concomitantly. Cox regression analysis showed that age (P = 0.015) was independently associated with an increased risk of severe infection. Survival curves showed a lower survival rate in patients with severe infections (log-rank and Breslow tests < 0.001). Conclusions: The rates of severe infections in SAD patients with severe, refractory disease treated depended on the biological agent used, with the highest rates being observed for rituximab and the lowest for etanercept. The rate of infection was especially high in patients receiving three or more courses of rituximab. In patients with severe infections, survival was significantly reduced. Older age was the only significant predictive factor of severe infection.