Pediatrics Martinón-Torres F, Bernatowska E et. al.
Aug 2nd, 2018 - The capsular group B meningococcal vaccine (4CMenB) is recommended for children with complement deficiencies, asplenia, and splenic dysfunction; however, data on the immunogenicity of 4CMenB in these "at-risk" children are missing. Participants aged 2 to 17 years in Italy, Spain, Poland, the United Kingdom, and Russia with complement deficiencies, asplenia, or splenic dysfunction received 2 dos...
Clinical Microbiology and Infection : the Official Publication of the European Society of Clinical Microbiology and Infectious Diseases; Sanges S, Wallet F et. al.
Feb 13th, 2017 - Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults. Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in three centres during a 3-year period. Eighteen to 40-year-old patients wer...
American Journal of Hematology; Shih AR, Murali MR
Oct 6th, 2015 - The complement pathway is a cascade of proteases that is involved in immune surveillance and innate immunity, as well as adaptive immunity. Dysfunction of the complement cascade may be mediated by aberrations in the pathways of activation, complement regulatory proteins, or complement deficiencies, and has been linked to a number of hematologic disorders, including paroxysmal noctural hemoglobi...
Lupus Dutertre M, Tomasevic D et. al.
Oct 11th, 2013 - Gonorrhea is a common sexually transmitted infection, which can present as the 'arthritis-dermatitis syndrome'. Patients with systemic lupus erythematosus often develop disseminated neisserial infections, because of inherited and acquired complement deficiencies. Neisserial infection, and particularly gonococcemia, can mimic a lupus flare. We report one case of gonococcemia presenting as acral ...
Clinical Microbiology Reviews; Ram S, Lewis LA et. al.
Oct 11th, 2010 - The complement system comprises several fluid-phase and membrane-associated proteins. Under physiological conditions, activation of the fluid-phase components of complement is maintained under tight control and complement activation occurs primarily on surfaces recognized as "nonself" in an attempt to minimize damage to bystander host cells. Membrane complement components act to limit complemen...
Lupus Lipsker D, Hauptmann G
Aug 9th, 2010 - In this review we address the main cutaneous manifestations and diseases associated with deficiencies in components of the complement system. The first part is devoted to hereditary angioedema, in which acute, sometimes life-threatening recurrent attacks of acute swelling, usually associated with gastrointestinal symptoms, occur. It is related to a structural or functional deficiency of C1 este...
Clinical and Experimental Immunology; Gullstrand B, Mårtensson U et. al.
Mar 23rd, 2009 - Inherited deficiencies in components of the classical complement pathway are strong disease susceptibility factors for the development of systemic lupus erythematosus (SLE) and there is a hierarchy among deficiency states, the strongest association being with C1q deficiency. We investigated the relative importance of the different complement pathways regarding clearance of apoptotic cells. Phag...
Journal of Clinical Immunology; Brodszki N, Frazer-Abel A et. al.
Feb 16th, 2020 - This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within the complement syste...
Clinical and Experimental Immunology; Schein TN, Blackburn TE et. al.
Aug 28th, 2019 - The complement system is now a therapeutic target for the management of serious and life-threatening conditions such as paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, glomerulonephritis and other diseases caused by complement deficiencies or genetic variants. As complement therapeutics expand into more clinical conditions, monitoring complement activation is increasing...
Journal of Innate Immunity; van den Broek B, van der Flier M et. al.
Jul 3rd, 2019 - Streptococcus pneumoniae and Neisseria meningitidis are pathogens that frequently colonize the nasopharynx in an asymptomatic manner but are also a cause of invasive bacterial infections mainly in young children. The complement system plays a crucial role in humoral immunity, complementing the ability of antibodies to clear microbes, thereby protecting the host against bacterial infections, inc...
BMC Infectious Diseases; Ladhani SN, Campbell H et. al.
Jun 15th, 2019 - To describe patients with inherited and acquired complement deficiency who developed invasive meningococcal disease (IMD) in England over the last decade. 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, ...
Clinical Reviews in Allergy & Immunology; Bordron A, Bagacean C et. al.
May 28th, 2019 - Approved for the treatment of autoimmune diseases, hematological malignancies, and solid cancers, several monoclonal antibodies (mAb) make use of complement in their mechanism of action. Such an assessment is based on comprehensive investigations that used mouse models, in vitro studies, and analyses from patients at initiation (basal level to highlight deficiencies) and after treatment initiat...
Apr 1st, 2019 - Background The complement system is part of the innate immune system. The complement system plays an important part in defense against pyogenic organisms. It promotes the inflammatory response, eliminates pathogens, and enhances the immune response. Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. In addition to playing an important role in host defense agai...
The Pediatric Infectious Disease Journal; Marujo F, Costa LC et. al.
Mar 17th, 2019 - Although bacterial meningitis is a rare presentation of a congenital immunodeficiency, invasive meningococcal disease is classically associated with complement deficiencies. We report a patient from a consanguineous kindred presenting with an invasive meningococcal disease caused by serogroup B meningococcus that revealed an underlying C5 deficiency caused by a novel mutation in the C5 gene.
The Pediatric Infectious Disease Journal; Marujo F, Costa LC et. al.
Aug 1st, 2018 - Although bacterial meningitis is a rare presentation of a congenital immunodeficiency, invasive meningococcal disease (IMD) is classicaly associated with complement deficiencies. We report a patient from a consanguineous kindred presenting with an IMD caused by serogroup B meningococcus that revealed an underlying C5 deficiency caused by a novel mutation in the C5 gene.
May 15th, 2018 - Antibody deficiencies and complement deficiencies are the most frequent Primary immunodeficiencies (PIDs) in adults, and are associated with greatly increased susceptibility to recurrent and/or severe bacterial infections - especially upper and lower respiratory tract infections and meningitis. The literature data suggest that PIDs are under-diagnosed in adults. The current European and US guid...
Current Opinion in Immunology; Costa-Reis P, Sullivan KE
Nov 3rd, 2017 - Monogenic lupus is rare, but its study has contributed immensely to a better understanding of the pathogenesis of systemic lupus erythematosus. The first forms identified were inherited complement deficiencies, which predisposed to lupus due to impaired tolerance, and aberrant clearance of apoptotic bodies and immune complexes. In recent years, several new monogenic disorders with a lupus-like ...
Feb 14th, 2017 - The study aims at evaluating the safety and immunogenicity of rMenB+OMV NZ when administered to subjects from 2 to 17 years of age with increased risk of meningococcal disease because either of primary or secondary complement deficiencies or of asplenia or splenic dysfunction. A group of healthy age-matched subjects will be enrolled to serve as a descriptive control for immunogenicity and safety.
FP Essentials; Starr SP
Nov 21st, 2016 - There are 264 primary immunodeficiencies (PIDs), most of which are rare. They are caused by complement deficiencies, defects in phagocyte function, impaired T-cell function, and/or impaired B-cell function with antibody deficiencies. Most patients with PIDs will present, at varying ages, with frequent infections. These infections can be common respiratory tract infections such as otitis media o...
Current Rheumatology Reviews; Yang JW, Rich E et. al.
Aug 9th, 2016 - The causal link between inherited complement deficiencies and systemic lupus erythematosus (SLE) has been well established, although it remains a rare cause of the disease. We present the case of three biological sisters with hereditary heterozygous C2 deficiency, but who differ widely in their clinical and serological manifestations. Patient 1 is 25 years old and was diagnosed with SLE at the ...