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Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017).

Ladhani, SN; Campbell, H; Lucidarme, J; Gray, S; Parikh, S; Willerton, L; Clark, SA; Lekshmi, A; Walker, A; Patel, S; et al. Ladhani, SN; Campbell, H; Lucidarme, J; Gray, S; Parikh, S; Willerton, L; Clark, SA; Lekshmi, A; Walker, A; Patel, S; Bai, X; Ramsay, M; Borrow, R (2019) Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017). BMC Infect Dis, 19 (1). p. 522. ISSN 1471-2334 https://doi.org/10.1186/s12879-019-4146-5
SGUL Authors: Ladhani, Shamez Nizarali

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Abstract

BACKGROUND: To describe patients with inherited and acquired complement deficiency who developed invasive meningococcal disease (IMD) in England over the last decade. METHODS: Public Health England conducts enhanced surveillance of IMD in England. We retrospectively identified patients with complement deficiency who developed IMD in England during 2008-2017 and retrieved information on their clinical presentation, vaccination status, medication history, recurrence of infection and outcomes, as well as characteristics of the infecting meningococcal strain. RESULTS: A total of 16 patients with 20 IMD episodes were identified, including four with two episodes. Six patients had inherited complement deficiencies, two had immune-mediated conditions associated with complement deficiency (glomerulonephritis and vasculitis), and eight others were on Eculizumab therapy, five for paroxysmal nocturnal haemoglobinuria and three for atypical haemolytic uraemic syndrome. Cultures were available for 7 of 11 episodes among those with inherited complement deficiencies/immune-mediated conditions and the predominant capsular group was Y (7/11), followed by B (3/11) and non-groupable (1/11) strains. Among patients receiving Eculizumab therapy, 3 of the 9 episodes were due to group B (3/9), three others were NG but genotypically group B, and one case each of groups E, W and Y. CONCLUSIONS: In England, complement deficiency is rare among IMD cases and includes inherited disorders of the late complement pathway, immune-mediated disorders associated with low complement levels and patients on Eculizumab therapy. IMD due to capsular group Y predominates in patient with inherited complement deficiency, whilst those on Eculizumab therapy develop IMD due to more diverse capsular groups including non-encapsulated strains.

Item Type: Article
Additional Information: © The Author(s). 2019Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Keywords: Complement deficiency, Eculizumab, Invasive meningococcal disease, Risk factors, Adolescent, Adult, Antibodies, Monoclonal, Humanized, Child, Child, Preschool, Complement System Proteins, England, Genotype, Humans, Immunologic Deficiency Syndromes, Meningococcal Infections, National Health Programs, Neisseria meningitidis, Polysaccharides, Bacterial, Retrospective Studies, Young Adult, Humans, Neisseria meningitidis, Meningococcal Infections, Immunologic Deficiency Syndromes, Polysaccharides, Bacterial, Retrospective Studies, Genotype, Adolescent, Adult, Child, Child, Preschool, National Health Programs, England, Complement System Proteins, Young Adult, Antibodies, Monoclonal, Humanized, Invasive meningococcal disease, Complement deficiency, Risk factors, Eculizumab, 0605 Microbiology, 1103 Clinical Sciences, 1108 Medical Microbiology, Microbiology
SGUL Research Institute / Research Centre: Academic Structure > Infection and Immunity Research Institute (INII)
Journal or Publication Title: BMC Infect Dis
ISSN: 1471-2334
Language: eng
Dates:
DateEvent
14 June 2019Published
30 May 2019Accepted
Publisher License: Creative Commons: Attribution 4.0
PubMed ID: 31200658
Web of Science ID: WOS:000471629000004
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/111134
Publisher's version: https://doi.org/10.1186/s12879-019-4146-5

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