A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations.Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated.866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3-and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar.Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.

Objective. To investigate the validity, reproducibility, and responsiveness of a simplified power Doppler ultrasound (PDUS) assessment of joint inflammation compared with a comprehensive 44-joint PDUS assessment in patients with rheumatoid arthritis (RA) who started therapy with a biologic agent. Methods. A total of 160 patients with active RA who started a biologic agent were prospectively recruited in 18 Spanish centers. The patients underwent clinical and laboratory assessment and blinded PDUS examination at baseline and 6 months. A PDUS examination of 128 synovial sites in 44 joints was performed. US synovitis and PD signal were semiquantitatively graded from 1 to 3 in all synovial sites. US count and index for synovitis and PD signal were obtained. PDUS intraobserver and interobserver reliability were evaluated. A process of data reduction based on the frequency of involvement of synovial sites by both synovitis and PD signal was conducted. Construct and discriminant validity of a simplified PDUS assessment was investigated. Results. A PDUS simplified assessment including 24 synovial sites from 12 joints detected 100% of patients with synovitis and 91% of patients with PD signal. There was a highly significant correlation between the 44-joint count and index for synovitis and PD signal and the 12-joint count and index for synovitis and PD signal at baseline and 6 months (r ‫؍‬ 0.84 -0.90, P < 0.0005). The smallest detectable difference was lower than the mean change in simplified PDUS variables. Conclusion. A 12-joint PDUS assessment of RA joint inflammation may be a valid, feasible method for multicenter monitoring of therapeutic response to biologic agents.

The aim of the present study is to evaluate the epidemiology, etiology and prognosis of pneumonia in lung transplant (LT) recipients. This is a prospective, multicenter study of a consecutive cohort of LT recipients in Spain. From September 2003 to November 2005, 85 episodes of pneumonia in 236 LT recipients were included (incidence 72 episodes per 100 LT/year). Bacterial pneumonia (82.7%) was more frequent than fungal (14%) and viral pneumonia (10.4%). The most frequent microorganisms in each etiological group were Pseudomonas aeruginosa (n = 14, 24.6%), CMV (n = 6, 10.4%) and Aspergillus spp. (n = 5, 8.8%). Incidence of Aspergillus spp. and CMV pneumonia is lower than previously reported, probably due to the spread of universal prophylaxis. Pneumonia caused by viruses appeared significantly later than pneumonia due to gram-negative bacilli, fungi and those without known etiology (p < 0.01, p = 0.03 and p = 0.02, respectively). The routine use of ganciclovir has changed the natural history of CMV infection, so that pneumonia appears later, once prophylaxis is suspended. The probability of survival during the first year of follow-up was significantly higher in the multivariate analysis in LT recipients who did not have a pneumonia episode compared with those that had at least one episode (p < 0.01).

This retrospective study of 251 lung transplant patients aimed to determine the prevalence, clinical presentation and mortality of Aspergillus infection in order to define specific risk factors and to compare survival in patients with and without infection. Aspergillus was isolated from 86 (33%) cases, which involved colonisation (n = 50), tracheobronchial lesions (n = 17) or invasive aspergillosis (n = 19). Overall, aspergillosis had an impact on survival (p < 0.05); in fact the 5-year mortality rate was substantially higher in single lung transplant recipients with bronchial anastomotic infection, and in those with late-onset infections and chronic rejection. A significant association (p < 0.05) was found between acute rejection and the time at which fungal infection was diagnosed. Aspergillus infection was not related to cytomegalovirus infection or treatment with corticosteroids. The mortality rate for invasive infections was 78% and was related to survival (p < 0.0001); invasive aspergillosis was also associated with chronic rejection (p < 0.05), but not with high corticosteroid doses (p 0.49) or use of tacrolimus (p 0.73). In conclusion, Aspergillus infection was associated with a reduction in the 5-year survival rate of lung transplant recipients, and this was particularly true for patients infected with the invasive forms and for patients with single lung transplants, bronchial anastomotic infection and chronic rejection. Isolation of Aspergillus spp. from respiratory samples preceded acute rejection, and may be a marker of graft dysfunction and/or airway inflammation. Close monitoring, or even pre-emptive antifungal therapy, is recommended for patients with chronic rejection or bronchial airway mechanical abnormalities and persistent Aspergillus colonisation.

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