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

• Main Authors: Shamez N. Ladhani, Helen Campbell, Jay Lucidarme, Steve Gray, Sydel Parikh, Laura Willerton, Stephen A. Clark, Aiswarya Lekshmi, Andrew Walker, Sima Patel, Xilian Bai, Mary Ramsay, Ray Borrow
• Format: Article
• Language: English
• Published: BMC 2019-06-01
• Series: BMC Infectious Diseases
• Subjects: Invasive meningococcal disease; Complement deficiency; Risk factors; Eculizumab
• Online Access: http://link.springer.com/article/10.1186/s12879-019-4146-5

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.