New Kids on the Block in SSc-PAH: May We Futurely Nail It Additionally Down to Capillaroscopy? A Systematic Literature Review

open7siObjective Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Current screening algorithms are hampered by low positive predictive values. Outcome measures that could futurely add to performance characteristics would be very welcome. Against this background, we aim to evaluate the role of nailfold videocapillaroscopy (NVC) using standardized definitions, in SSc related PAH (SSc-PAH). Methods A systematic review to identify original research papers documenting an association between NVC and right heart catheterisation defined SSc-PAH was performed according to the PRISMA guidelines. Subsequently, NVC parameters were subdivided into quantitative (capillary density, dimension, morphology, and haemorrhages), semi-quantitative and qualitative assessment (NVC pattern), according to the definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases. Results The systematic search identified 316 unique search results, of which 5 were included in the final qualitative analysis. The occurrence of incident SSc-PAH unequivocally associated in 2 longitudinal studies with progressive capillary loss (p=0.04 and p=0.033) and the progression to a severe (active/late) NVC pattern (p=0.05/0.01 and HR=5.12, 95%CI: 1.23- 21.27). In 3 cross-sectional studies, SSc-PAH was found to be unequivocally inversely associated with capillary density (p=0.001 and p<0.05) and associated with the presence of a severe NVC pattern (p=0.03 and p<0.05). Conclusion This is the first systematic literature review investigating the role of NVC in SSc-PAH using standardized description. Unequivocal associations were found between (incident) SSc-PAH and capillary density and NVC pattern. Integration of NVC into current screening algorithms to boost their performance may be a future step.embargoed_20200816Smith V, Vanhaecke A, Vandecasteele E, Guerra M, Paolino S, Melsens K, Cutolo M.Smith, V; Vanhaecke, A; Vandecasteele, E; Guerra, M; Paolino, S; Melsens, K; Cutolo, M

Acute and critically ill peripartum cardiomyopathy and 'bridge to' therapeutic options: a single center experience with intra-aortic balloon pump, extra corporeal membrane oxygenation and continuous-flow left ventricular assist devices

Introduction: Peripartum cardiomyopathy (PPCM) patients refractory to medical therapy and intra-aortic balloon pump (IABP) counterpulsation or in whom weaning from these therapies is impossible, are candidates for a left ventricular assist device (LVAD) as a bridge to recovery or transplant. Continuous-flow LVADs are smaller, have a better long-term durability and are associated with better outcomes. Extra corporeal membrane oxygenation (ECMO) can be used as a temporary support in patients with refractory cardiogenic shock. The aim of this study was to evaluate the efficacy and safety of mechanical support in acute and critically ill PPCM patients. Methods: This was a retrospective search of the patient database of the Ghent University hospital (2000 to 2010). Results: Six PPCM-patients were treated with mechanical support. Three patients presented in the postpartum period and three patients at the end of pregnancy. All were treated with IABP, the duration of IABP support ranged from 1 to 13 days. An ECMO was inserted in one patient who presented with cardiogenic shock, multiple organ dysfunction syndrome and a stillborn baby. Two patients showed partial recovery and could be weaned off the IABP. Four patients were implanted with a continuous-flow LVAD (HeartMate II(R), Thoratec Inc.), including the ECMO-patient. Three LVAD patients were successfully transplanted 78, 126 and 360 days after LVAD implant; one patient is still on the transplant waiting list. We observed one peripheral thrombotic complication due to IABP and five early bleeding complications in three LVAD patients. One patient died suddenly two years after transplantation. Conclusions: In PPCM with refractory heart failure IABP was safe and efficient as a bridge to recovery or as a bridge to LVAD. ECMO provided temporary support as a bridge to LVAD, while the newer continuous-flow LVADs offered a safe bridge to transplant

Severe infection, sepsis and acute kidney injury

Both severe infection and acute kidney injury (AKI) have a high, and rising incidence in critically ill patients admitted to the intensive care unit (ICU), and are associated with increased in-hospital mortality. Septic AKI patients are more severely ill compared to non-septic AKI patients and have worse outcome. Severe infection is a major cause of AKI in ICU patients, while conversely, AKI patients are at increased risk for infection. The dogma from the past relates the development of AKI in sepsis patients to decreased renal blood flow. However, current data suggest that there is no impairment of renal blood flow inpatients with sepsis. The pathogenesis of AKI in sepsis is probably related to cytotoxic effects of inflammation, and impaired microcirculation. In addition, hyperglycaemia, and antimicrobial agent-induced drug nephrotoxicity may contribute to the development of AKI. On the other hand, AKI patients are at greater risk for infection as a result of volume overload, dialysis catheter insertion and secondary manipulation, inflammation of the kidneys leading to 'organ cross talk', and impaired host immunity

Grensoverschrijdend gedrag in de zorgrelatie: het perspectief van verpleegkundigen werkzaam op algemene ziekenhuisafdelingen

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Pulmonary hypertension in interstitial lung disease : an area of unmet clinical need

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

Researching atrial fibrillation in animal models or veterinary patients: major role for the anatomist

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