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Pulmonary hypertension (PH) is present in an important proportion of patients with interstitial lung diseases (ILDs), encompassing a large, heterogeneous group of diffuse parenchymal lung diseases. Development of ILD-related PH is associated with reduced exercise capacity, increased need for supplemental oxygen, decreased quality of life and earlier death. Diagnosis of ILD-related PH is important and requires a high index of suspicion. Noninvasive diagnostic assessment can suggest the presence of PH, although right heart catheterisation remains the gold standard to confirm the diagnosis and to assess its severity. A comprehensive assessment is needed to make sure reversible causes of PH have been ruled out, including thromboembolic events, untreated hypoxaemia and sleep disordered breathing. The results of trials concerning pulmonary vasodilators in this particular patient group have been disappointing and, in some cases, were even associated with an increased risk of harm. Newer strategies such as medications administered through inhalation and combinations with antifibrotic drugs show encouraging results. Moreover, unravelling the role of the vasculature in the pathophysiology of pulmonary fibrosis and ILD-related PH may potentially unlock new therapeutic opportunities.

The epidemiology of interstitial lung disease (ILD) in systemic sclerosis (SSc) in Belgium is unknown. In literature, its prevalence varies between 19% and 52% in limited/diffuse cutaneous SSc (LcSSc/DcSSc). However, its prevalence in "early" SSc (pre-clinically overt SSc without [yet] skin involvement), nor its incidence rate in SSc (LcSSc/DcSSc/"early" SSc) has ever been described. Against this background, we aimed to determine the prevalence/incidence (rate) and progression of ILD in SSc. 12-year follow-up data of consecutive SSc patients, included in two Flemish cohorts (University Hospitals Ghent and Leuven), were retrospectively analysed. ILD was classified according to the simplified Goh algorithm. Progression of ILD was defined as a relative decline of FVC ≥10%, a combined relative decline of FVC 5-10% and DLCO ≥15%, or as an increase in HRCT extent. 722 patients (60% LcSSc/ 20% DcSSc/ 20% "early" SSc, median (IQR) follow-up 39 [12-80] months) had baseline HRCT. 243 were rated to have ILD at baseline and 39 during follow-up (prevalence of 34%/ incidence rate of 20.3/1000PY, 95%CI:14.5-27.8). Amongst those with baseline ILD, 60% had lung functional progression at five years of follow-up. In the "early" SSc subgroup, eight patients were rated to have ILD at baseline and three during follow-up (prevalence of 6%/ incidence rate of 5.8/1000 PY, 95%CI:1.2-17.0). Both LcSSc and DcSSc patients should be monitored for ILD evolution. The low prevalence and incidence of ILD in the "early" SSc subgroup may instruct future decisions on the construction of uniform patient follow-up pathways in "early" SSc.

Objectives: Following results in open-label studies of rituximab in patients with systemic sclerosis, a Belgian three-centre initiative was launched to explore safety and efficacy of rituximab in early, diffuse cutaneous systemic sclerosis (dcSSc). Methods: Open-label study of 17 patients with early dcSSc, treated with two courses of rituximab, at month 0 and 6. Clinical examination, lung function testing, echocardiography, disease activity score (DAS) and functional status were performed at baseline and over 24 months of follow-up. Results: Modified Rodnan skin score (MRSS) changed significantly over time, with a mean of 25.5 (standard deviation [SD] 6.0) at baseline to 12.6 (SD 5.1) at month 24 (Mixed Model Analysis [MMA] p < 0.0001), which is a decrease of 51% at month 24 vs. baseline. DAS showed significant decrease over the total study period, with a score of 4.1 (SD 1.7) at baseline to 1.5 (SD 1.8) at month 24 (MMA p < 0.0001). Additionally, this was significant at all time points vs. baseline, both for MRSS and DAS. Internal organ status remained clinically stable throughout the study period. No statistically significant differences compared to baseline were found at the follow-up time points. Seven serious adverse events took place, all except for one, considered unrelated to study medication. Conclusions: This is the first multicentre Belgian collaboration investigating potential efficacy of rituximab in early dcSSc. Rituximab appears to be safe and tolerable and it may have beneficial effects on skin involvement, on overall disease activity and on stabilization of internal organ status in early dcSSc., SCOPUS: ar.j, info:eu-repo/semantics/published

Aims: The relationship between the predictive performance of the TIMI risk score for STEMI and gender has not been evaluated in the setting of primary PCI (pPCI). Here, we compared in-hospital mortality and predictive performance of the TIMI risk score between Belgian women and men undergoing pPCI. Methods and results: In-hospital mortality was analysed in 8,073 (1,920 [23.8%] female and 6,153 [76.2%] male patients) consecutive pPCI-treated STEMI patients, included in the prospective, observational Belgian STEMI registry (January 2007 to February 2011). A multivariable logistic regression model, including TIMI risk score variables and gender, evaluated differences in in-hospital mortality between men and women. The predictive performance of the TIMI risk score according to gender was evaluated in terms of discrimination and calibration. Mortality rates for TIMI scores in women and men were compared. Female patients were older, had more comorbidities and longer ischaemic times. Crude in-hospital mortality was 10.1% in women vs. 4.9% in men (OR 2.2; 95% CI: 1.82-2.66, p<0.001). When adjusting for TIMI risk score variables, mortality remained higher in women (OR 1.47, 95% CI: 1.15-1.87, p=0.002). The TIMI risk score provided a good predictive discrimination and calibration in women as well as in men (c-statistic=0.84 [95% CI: 0.809-0.866], goodness-of-fit p=0.53 and c-statistic=0.89 [95% CI: 0.873-0.907], goodness-of-fit p=0.13, respectively), but mortality prediction for TIMI scores was better in men (p=0.02 for TIMI score x gender interaction). Conclusions: In the Belgian STEMI registry, pPCI-treated women had a higher in-hospital mortality rate even after correcting for TIMI risk score variables. The TIMI risk score was effective in predicting in-hospital mortality but performed slightly better in men. The database was registered with clinicaltrials.gov (NCT00727623). © Europa Digital & Publishing 2014. All rights reserved.

Aims: The relationship between the predictive performance of the TIMI risk score for STEMI and gender has not been evaluated in the setting of primary PCI (pPCI). Here, we compared in-hospital mortality and predictive performance of the TIMI risk score between Belgian women and men undergoing pPCI. Methods and results: In-hospital mortality was analysed in 8,073 (1,920 [23.8%] female and 6,153 [76.2%] male patients) consecutive pPCI-treated STEMI patients, included in the prospective, observational Belgian STEMI registry (January 2007 to February 2011). A multivariable logistic regression model, including TIMI risk score variables and gender, evaluated differences in in-hospital mortality between men and women. The predictive performance of the TIMI risk score according to gender was evaluated in terms of discrimination and calibration. Mortality rates for TIMI scores in women and men were compared. Female patients were older, had more comorbidities and longer ischaemic times. Crude in-hospital mortality was 10.1% in women vs. 4.9% in men (OR 2.2; 95% CI: 1.82-2.66, p<0.001). When adjusting for TIMI risk score variables, mortality remained higher in women (OR 1.47, 95% CI: 1.15-1.87, p=0.002). The TIMI risk score provided a good predictive discrimination and calibration in women as well as in men (c-statistic=0.84 [95% CI: 0.809-0.866], goodness-of-fit p=0.53 and c-statistic=0.89 [95% CI: 0.873-0.907], goodness-of-fit p=0.13, respectively), but mortality prediction for TIMI scores was better in men (p=0.02 for TIMI score x gender interaction). Conclusions: In the Belgian STEMI registry, pPCI-treated women had a higher in-hospital mortality rate even after correcting for TIMI risk score variables. The TIMI risk score was effective in predicting in-hospital mortality but performed slightly better in men. The database was registered with clinicaltrials.gov (NCT00727623). © Europa Digital & Publishing 2014. All rights reserved., SCOPUS: ar.j, info:eu-repo/semantics/published

Background: Treatment strategies and outcome of ST-elevation myocardial infarction (STEMI) have been mainly studied in middle-aged patients. With increasing lifetime expectancy, the proportion of octogenarians will substantially increase. We aimed to evaluate whether the benefit of currently recommended reperfusion strategies is maintained in octogenarians. Methods: Reperfusion therapy and in-hospital mortality were evaluated in 1,092 octogenarians and compared with 7,984 STEMI patients <80 years old based on data from the prospective Belgian STEMI registry. Results: The octogenarian STEMI group had more cardiovascular comorbidities, contained more female patients and presented more frequently with cardiac failure (Killip class >1, 40 vs. 20 %) compared with their younger counterparts (all p < 0.05). Although the rate of thrombolysis was similar (9.2 vs. 9.9 %) between both groups, a conservative approach was chosen more frequently (13.8 vs. 4.7 %), while PCI was performed less frequently (76.9 vs. 85.4 %) in octogenarians (p < 0.001). Moreover, ischemic time and door-to-needle/balloon time were longer for octogenarians. In-hospital mortality for octogenarians was 17.8 vs. 5.5 % in the younger group [adjusted OR 2.43(1.92-3.08)]. In haemodynamically stable octogenarians, PCI seemed to improve outcome compared with thrombolysis or conservative treatment (5.7 vs. 12.7 vs. 8.5 %, p = 0.09). In octogenarians with cardiac failure, in-hospital mortality was extremely high independent of the chosen reperfusion therapy (34.6 vs. 31.6 vs. 36.3 %, p = 0.88). Conclusions: In-hospital mortality in octogenarian STEMI patients was high and related to a high prevalence of cardiac failure. Less PCI was performed in the octogenarian group compared with the younger patients, although mortality benefit of PCI was maintained in haemodynamically stable octogenarians. © 2013 Springer-Verlag Berlin Heidelberg.

Background: Treatment strategies and outcome of ST-elevation myocardial infarction (STEMI) have been mainly studied in middle-aged patients. With increasing lifetime expectancy, the proportion of octogenarians will substantially increase. We aimed to evaluate whether the benefit of currently recommended reperfusion strategies is maintained in octogenarians. Methods: Reperfusion therapy and in-hospital mortality were evaluated in 1,092 octogenarians and compared with 7,984 STEMI patients <80 years old based on data from the prospective Belgian STEMI registry. Results: The octogenarian STEMI group had more cardiovascular comorbidities, contained more female patients and presented more frequently with cardiac failure (Killip class >1, 40 vs. 20 %) compared with their younger counterparts (all p < 0.05). Although the rate of thrombolysis was similar (9.2 vs. 9.9 %) between both groups, a conservative approach was chosen more frequently (13.8 vs. 4.7 %), while PCI was performed less frequently (76.9 vs. 85.4 %) in octogenarians (p < 0.001). Moreover, ischemic time and door-to-needle/balloon time were longer for octogenarians. In-hospital mortality for octogenarians was 17.8 vs. 5.5 % in the younger group [adjusted OR 2.43(1.92-3.08)]. In haemodynamically stable octogenarians, PCI seemed to improve outcome compared with thrombolysis or conservative treatment (5.7 vs. 12.7 vs. 8.5 %, p = 0.09). In octogenarians with cardiac failure, in-hospital mortality was extremely high independent of the chosen reperfusion therapy (34.6 vs. 31.6 vs. 36.3 %, p = 0.88). Conclusions: In-hospital mortality in octogenarian STEMI patients was high and related to a high prevalence of cardiac failure. Less PCI was performed in the octogenarian group compared with the younger patients, although mortality benefit of PCI was maintained in haemodynamically stable octogenarians. © 2013 Springer-Verlag Berlin Heidelberg., SCOPUS: ar.j, info:eu-repo/semantics/published