Thrombotic antiphospholipid syndrome (APS) is characterised by venous, arterial or microvascular thrombosis in the context of persistently positive antiphospholipid antibodies (aPL). The diagnosis and management of thrombotic APS continues to prove challenging for clinicians. We provide a practical guide to the diagnosis of APS including who to test for aPL and which tests to do. We also consider clinical practice points on the management of venous, arterial and small vessel thrombosis, in the context of first and recurrent thrombotic events. Non-criteria manifestations of APS are reviewed. An approach to recurrent thrombosis and anticoagulant-refractory APS is discussed, with options including increasing the anticoagulation intensity of vitamin K antagonists, switching to low-molecular-weight-heparin, the use of fondaparinux and/or the addition of antiplatelet treatment. The use of adjunctive options such as vitamin D, hydroxychloroquine and statins are also addressed.
Arterial and venous thromboses occur in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein level, and skin changes) syndrome at a previously reported rate of 20%. We reviewed the University College London Hospitals (UCLH) POEMS Registry to determine the rate of venous thromboembolism (VTE), arterial events, and risk factors. This registry, established in 1999 and comprising 103 patients at the time of this study, is the largest single-center cohort in Europe. Of the 83 assessable patients, median age at presentation was 52 years (range, 31-84). Twenty-five patients experienced clinically apparent arterial or venous events, and 2 had concurrent arterial and venous thromboses. Eleven patients had VTEs, including deep vein thrombosis (DVT; 3 of 11), pulmonary embolism (4 of 11), and peripherally inserted central catheter–associated DVT, which occurred during autologous stem cell transplantation (3 of 11). Sixteen patients experienced arterial events: stroke (7 of 16), peripheral arterial occlusion (5 of 16), myocardial infarction (3 of 16), and microvascular disease (1 of 16), with no discernible relationship with thrombocytosis or polycythemia. Thirty percent of POEMS patients have arterial and venous thromboses, higher than previously reported. There were more arterial than venous events, and most occurred during active disease, before the start of chemotherapy, indicating the need for a preemptive approach to thromboprophylaxis.
Background and Objectives: Multidisciplinary care (MDC) is known to improve the management of chronic diseases. In this study, we investigated whether MDC improves outcomes in patients with advanced chronic kidney disease. Design, Setting, Participants and Measurements: In this retrospective case-control study we have compared the outcomes at the point of starting dialysis and beyond between a cohort of MDC patients (n = 171) and a cohort of nephrology patients (n = 194). The groups were well-matched demographically and were seen in the clinic for at least 3 months before starting dialysis. Dialysis access, blood pressure, haemoglobin, various biochemical parameters, hospital admissions, and survival were compared between the 2 groups. Results: In the MDC group, 68.4% started dialysis with permanent access compared with 58.8% in the nephrologist group (p = 0.04). The mean haemoglobin in the MDC group was 10.28 ± 1.86 versus 9.81 ± 1.76 g/dl in the nephrology group (p = 0.02). There was no difference between the groups in terms of blood pressure control or serum calcium, phosphate, or albumin levels. There were fewer hospital admissions in the MDC cohort (1.42 vs. 2.52 admissions per patient per year, p = 0.005). Kaplan-Meier survival analysis showed that patient survival was significantly better in the MDC group (p = 0.033). Conclusions: This study demonstrates that patients attending a multidisciplinary clinic are better prepared for dialysis treatment, have fewer hospital admissions after start of dialysis, and have a higher patient survival compared to those attending a traditional nephrology clinic.
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