Epidemiology of chronic inflammatory demyelinating polyradiculoneuropathy in The Netherlands

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare but disabling disorder that often requires long‐term immunomodulatory treatment. Background incidence rates and prevalence and risk factors for developing CIDP are still poorly defined. In the current study, we used a longitudinal population‐based cohort study in The Netherlands to assess these rates and demographic factors and comorbidity associated with CIDP. We determined the incidence rate and prevalence of CIDP between 2008 and 2017 and the occurrence of potential risk factors in a retrospective Dutch cohort study using the Integrated Primary Care Information (IPCI) database. Cases were defined as CIDP if the diagnosis of CIDP was described in the electronic medical file. In a source population of 928 030 persons with a contributing follow‐up of 3 525 686 person‐years, we identified 65 patients diagnosed with CIDP. The overall incidence rate was 0.68 per 100 000 person‐years (95% CI 0.45‐0.99). The overall prevalence was 7.00 per 100 000 individuals (95% CI 5.41‐8.93). The overall incidence rate was higher in men compared to woman (IRR 3.00, 95% CI 1.27‐7.11), and higher in elderly of 50 years or older compared with people <50 years of age (IRR 17 95% CI 4‐73). Twenty percent of CIDP cases had DM and 9% a co‐existing other auto‐immune disease. These background rates are important to monitor changes in the frequency of CIDP following infectious disease outbreaks, identify potential risk factors, and to estimate the social and economic burden of CIDP

Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis

Abstract Background: Prevalence and incidence rates of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are required to determine the impact of CIDP on society. We aimed to estimate the prevalence and incidence of CIDP worldwide and to determine the effect of diagnostic criteria on prevalence and incidence. Method: A systematic review was conducted for all published incidence and prevalence studies on CIDP until May 18, 2017. Methodological quality was assessed using the Methodological Evaluation of Observational Research checklist. We performed a random effect meta-analysis to estimate pooled prevalence and incidence rates. Results: Of the 907 studies, 11 were included in the systematic review, 5 in the meta-analysis of incidence (818 cases; 220,513,514 person-years) and 9 in the meta-analysis of prevalence (3,160 cases; 160,765,325 population). These studies had a moderate quality. The pooled crude incidence rate was 0.33 per 100,000 person-years (95% CI 0.21–0.53; I2 = 95.7%) and the pooled prevalence rate was 2.81 per 100,000 (95% CI 1.58–4.39; I 2 = 99.1%). Substantial heterogeneity in incidence and prevalence across studies seems to be partly explained by using different diagnostic criteria. Conclusion: These findings provide a starting point to estimate the social burden of CIDP and demonstrate the need to reach consensus on diagnostic criteria for CIDP

Evaluation of management and guideline adherence in children with mild traumatic brain injury

Aim: To evaluate the management and guideline adherence in children with mild traumatic brain injury (MTBI) in emergency departments (ED) in the Netherlands. Methods: A multicentre cohort study was conducted, including children younger than 18 years with MTBI who presented within 24 hours after trauma in the ED of hospitals in the southwest region of the Netherlands, in 2014. Primary outcome measures for management were percentages of performed computed tomography (CT) scans and hospital admissions. Guideline adherence was defined as percentages of correctly following the guideline. Secondary outcome measures were differences in management and guideline adherence between hospitals. Results: About 563 patients were analysed. Hospital admission was the most frequently performed management type (49.2% hospital admission vs. 30.9% CT). In only 49.7% of patients, the guideline was followed correctly. A substantial overuse of hospital admission (35%) and underuse of CT (40.1%) were found. Percentages of hospital admission and CT varied between 39.4–55.6% and 23.3–44.1%, respectively, across hospitals. Percentages of correctly following the guideline varied between 39.2–64.9% across hospitals. Conclusion: These findings suggest that physicians in the participating hospitals prefer hospital admission of children with MTBI instead of CT despite the current recommendations of the national MTBI guideline in the Netherlands

Genetic biomarkers for intravenous immunoglobulin response in chronic inflammatory demyelinating polyradiculoneuropathy

Background and purpose Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinical and electrophysiological heterogeneous immune-mediated polyneuropathy. Intravenous immunoglobulin (IVIg), corticosteroids, and plasma exchange are proven effective treatments for CIDP. The clinical response to IVIg is variable between patients and currently unexplained. Finding biomarkers related to treatment response can help to understand the diversity of CIDP and personalise treatment choice.Methods We investigated whether genetic variation between patients may explain some of these differences in treatment response. Based on previous publications, we selected six candidate genes that might affect immune and axonal functions, IVIg metabolism, and treatment response in CIDP. Genetic variants were assessed in 172 CIDP patients treated with at least one course of IVIg (2 g/kg). A response to IVIg was defined by >= 1 grade improvement on the modified Rankin Scale. Blood samples were tested for variations in CNTN2, PRF1, FCGRT, FCGR2B, GJB1, and SH2D2A genes.Results In univariate analysis, patients with the FCGR2B promoter variant 2B.4/2B.1 responded more often to IVIg than patients with the 2B.1/2B.1 variant (odds ratio [OR] = 6.9, 95% confidence interval [CI] = 1.6-30; p = 0.003). Patients with the p.(Ala91Val) variant of PRF1 were less often IVIg responsive (OR = 0.34, 95% CI = 0.13-0.91; p = 0.038). In multivariate analysis, both PRF1 and FCGR2B showed discriminative ability to predict the chance of IVIg response (area under the curve = 0.67).Conclusions Variations in PRF1 and the promoter region of FCGR2B are associated with the response to IVIg in CIDP. These findings, which require validation, are a first step towards the understanding of the heterogeneity in the treatment response in CIDP.Genetics of disease, diagnosis and treatmen

Genetic biomarkers for intravenous immunoglobulin response in chronic inflammatory demyelinating polyradiculoneuropathy

Background and purpose: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinical and electrophysiological heterogeneous immune-mediated polyneuropathy. In

Guideline summary: Intensive care admission, treatment and discharge of critically ill haemato-oncological patients

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Samenvatting Richtlijn Intensive-care-opname, behandeling en ontslag van de kritisch zieke hemato-oncologische patiënt

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Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study.

Contains fulltext : 81439.pdf (publisher's version ) (Closed access)The prevalence and incidence of systemic sclerosis (SSc) in The Netherlands is unknown. The same holds true for its leading causes of death: pulmonary fibrosis and pulmonary arterial hypertension (PAH), for which effective treatment options have recently become available. OBJECTIVE: To establish the prevalence and incidence of SSc and its pulmonary complications. METHODS: Detailed information on patients in the POEMAS registry, "Pulmonary Hypertension Screening, a Multidisciplinary Approach in Scleroderma", consisting of 819 patients, was combined with a nationwide questionnaire. RESULTS: By combining the two sources the prevalence of SSc was found to be 8.9 per 100 000 adults. The incidence was 0.77 patients per 100 000 per year. PAH was diagnosed in 9.9% of SSc patients. The prevalence of interstitial lung disease in SSc varied from 19% to 47% depending on the definition used. CONCLUSION: This study clarifies the epidemiology of SSc in The Netherlands and confirms the frequent occurrence of pulmonary complications, based on 654 cases. This can and will be studied further in the ongoing POEMAS study