Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present study provides a comprehensive literature review of the burden of illness of CIDP. Methods Systematic literature search of PubMed, Embase, and key conferences in May 2019. Search terms identified studies on the epidemiology, humanistic burden, current treatment, and economic burden of CIDP published since 2009 in English. Results Forty-five full texts and nineteen conference proceedings were identified on the epidemiology (n = 9), humanistic burden (n = 7), current treatment (n = 40), and economic burden (n = 8) of CIDP. Epidemiological studies showed incidence and prevalence of 0.2-1.6 and 0.8-8.9 per 100,000, respectively, depending on geography and diagnostic criteria. Humanistic burden studies revealed that patients experienced physical and psychosocial burden, including impaired physical function, pain and depression. Publications on current treatments reported on six main types of therapy: intravenous immunoglobulins, subcutaneous immunoglobulins, corticosteroids, plasma exchange, immunosuppressants, and immunomodulators. Treatments may be burdensome, due to adverse events and reduced independence caused by treatment administration setting. In Germany, UK, France, and the US, CIDP economic burden was driven by direct costs of treatment and hospitalisation. CIDP was associated with indirect costs driven by impaired productivity. Conclusions This first systematic review of CIDP burden of illness demonstrates the high physical and psychosocial burden of this rare disease. Future research is required to fully characterise the burden of CIDP, and to understand how appropriate treatment can mitigate burden for patients and healthcare systems.

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Positive chronotropic and inotropic responses of guinea-pig isolated spontaneously beating right atria and paced left atria to beta-adrenoceptor agonists were recorded. Cumulative concentration-response curves for the rate and tension responses to isoprenaline, orciprenaline, terbutaline and fenoterol were obtained before incubation of the tissues with isoprenaline (10(-6) M), the tissues were then washed during 1 h to remove isoprenaline before constructing a post-incubation curve to the same agonist. Incubation with isoprenaline for 4 h caused parallel rightwards shifts to the curves, but extending the incubation to 8 h caused additional depression of the rate (85.2 +/- 5.4%) and tension (68.9 +/- 2.3%) maxima. Incubation with isoprenaline for 4 h only shifted the curves for orciprenaline to the right but depressed the post-incubation maximum rate and tension responses to terbutaline (74.8 +/- 1.5 and 33.8 +/- 2.5%) and fenoterol (85.6 +/- 5.5 and 76.0 +/- 5.1%). Thus incubation with isoprenaline for 4 h induced desensitization of the cardiac beta-adrenoceptor which was revealed as a loss in sensitivity to both isoprenaline and other beta-adrenoceptor agonists. The reduced maxima were attributed to a loss of beta-adrenoceptors and exhaustion of the receptor reserve. Those experiments displaying the reduced maxima were used for calculation of dissociation constants (KA) values by the method of Furchgott (1966) for irreversible antagonism. The values for the right atria were 57 (19-170)nM, 29 (8.4-100)microM and 13 (1.9-8.4)microM and for the left atria, 89 (15-510)nM, 25 (9.9-64)microM and 18 (7.7-42)microM for isoprenaline (8 h incubation), terbutaline and fenoterol respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

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Background There is emerging evidence that particularly high rates of major adverse cardiovascular events (MACE) occur in the 90-day period after acute myocardial infarction (AMI). Post-AMI MACE are associated with additional resource utilization and economic burden for healthcare systems, with multivessel disease (MVD) conferring a 3- to 4-fold increase in risk of recurrent MACE in the first year post AMI vs. single vessel disease. Thus, characterizing the prevalence and outcomes of patients with MVD and various comorbidities is crucial to predict and mitigate clinical risk and associated costs. Purpose To describe post-AMI readmissions and economic burden in MVD patients at 30, 90, and 365 days. Methods This was a retrospective cohort study (1st January, 2008 to 31st December, 2018). Patients with MVD were identified by a confirmed diagnosis code from a longitudinal, representative UK population health dataset comprising primary care data from the Clinical Practice Research Datalink (CPRD), which was linked to secondary care data from the Hospital Episode Statistics (HES) and Office of National Statistics (ONS) databases. Patients who experienced at least one AMI after their first recorded MVD diagnosis were defined as MVD+. A MACE was defined as any one of non-fatal MI, non-fatal stroke, or cardiovascular death. Readmissions were defined as non-elective admissions with a MACE outcome diagnosis occurring up to 28 days from a previous admission with the same treatment speciality. Results The study population included 78,128 MVD+ patients (4.2% of the total patient population). In the MVD+ cohort, 3,753 (4.8%) patients were readmitted with at least one MACE event or other non-elective admission within the cardiovascular specialty: 2,394 (63.8%), 2,562 (68.3%), and 2,830 (75.4%) readmissions occurred within 30, 90, and 365 days, respectively. The mean length of hospital stay per patient, per readmission for MACE within 30, 90, and 365 days was 7.22, 7.46, and 7.74 days, respectively. The mean cost per MACE admission within 90 days of index AMI was £3,926.52. Non-fatal MI was the most common reason for readmission at all timepoints. Percutaneous transluminal balloon angioplasty and saphenous vein graft procedures (coronary artery bypass graft, CABG) were among the most common cardiac procedures in MVD+ patients, at 70,276 (22.7%) and 15,418 (5.0%), respectively. Conclusions In patients with MVD who suffer an AMI, there is high resource utilization and cost, particularly in the first 90-days post AMI; therefore, the 90-day post-AMI period represents significant economic costs to healthcare systems. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): CSL Behring

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combination treatment, in Spain this proportion is only 15%. Conclusions: This study provided a robust quantification of MF-CTCL patients treated in France and Spain, it described differences in care pathways that may impact on patient access to treatment.