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Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients
Complement system is a part of innate immunity, its main function is to protect human from bacterial infection. As genetic disorders, complement deficiencies are often diagnosed in pediatric population. However, complement deficiencies can also be revealed in adults but have been poorly investigated...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902496/ https://www.ncbi.nlm.nih.gov/pubmed/27175654 http://dx.doi.org/10.1097/MD.0000000000003548 |
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author | Audemard-Verger, A. Descloux, E. Ponard, D. Deroux, A. Fantin, B. Fieschi, C. John, M. Bouldouyre, A. Karkowsi, L. Moulis, G. Auvinet, H. Valla, F. Lechiche, C. Davido, B. Martinot, M. Biron, C. Lucht, F. Asseray, N. Froissart, A. Buzelé, R. Perlat, A. Boutboul, D. Fremeaux-Bacchi, V. Isnard, S. Bienvenu, B. |
author_facet | Audemard-Verger, A. Descloux, E. Ponard, D. Deroux, A. Fantin, B. Fieschi, C. John, M. Bouldouyre, A. Karkowsi, L. Moulis, G. Auvinet, H. Valla, F. Lechiche, C. Davido, B. Martinot, M. Biron, C. Lucht, F. Asseray, N. Froissart, A. Buzelé, R. Perlat, A. Boutboul, D. Fremeaux-Bacchi, V. Isnard, S. Bienvenu, B. |
author_sort | Audemard-Verger, A. |
collection | PubMed |
description | Complement system is a part of innate immunity, its main function is to protect human from bacterial infection. As genetic disorders, complement deficiencies are often diagnosed in pediatric population. However, complement deficiencies can also be revealed in adults but have been poorly investigated. Herein, we describe a case series of infections revealing complement deficiency in adults to study clinical spectrum and management of complement deficiencies. A nationwide retrospective study was conducted in French university and general hospitals in departments of internal medicine, infectious diseases enrolling patients older than 15 years old who had presented at least one infection leading to a complement deficiency diagnosis. Forty-one patients included between 2002 and 2015 in 19 different departments were enrolled in this study. The male-to-female ratio was 1.3 and the mean age at diagnosis was 28 ± 14 (15–67) years. The main clinical feature was Neisseria meningitidis meningitis 75% (n = 31/41) often involving rare serotype: Y (n = 9) and W 135 (n = 7). The main complement deficiency observed was the common final pathway deficiency 83% (n = 34/41). Half of the cohort displayed severe sepsis or septic shock at diagnosis (n = 22/41) but no patient died. No patient had family history of complement deficiency. The mean follow-up was 1.15 ± 1.95 (0.1–10) years. Half of the patients had already suffered from at least one infection before diagnosis of complement deficiency: meningitis (n = 13), pneumonia (n = 4), fulminans purpura (n = 1), or recurrent otitis (n = 1). Near one-third (n = 10/39) had received prophylactic antibiotics (cotrimoxazole or penicillin) after diagnosis of complement deficiency. The vaccination coverage rate, at the end of the follow-up, for N meningitidis, Streptococcus pneumonia, and Haemophilius influenzae were, respectively, 90% (n = 33/37), 47% (n = 17/36), and 35% (n = 14/34). This large study emphasizes that complement deficiencies can be revealed in adults by infectious episodes. Most of them were meningococcal infections revealing common final pathway deficiency. To avoid undiagnosis or late diagnosis, adult displaying first episode of N meningitidis infection should be tested for complement deficiency. |
format | Online Article Text |
id | pubmed-4902496 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-49024962016-06-27 Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients Audemard-Verger, A. Descloux, E. Ponard, D. Deroux, A. Fantin, B. Fieschi, C. John, M. Bouldouyre, A. Karkowsi, L. Moulis, G. Auvinet, H. Valla, F. Lechiche, C. Davido, B. Martinot, M. Biron, C. Lucht, F. Asseray, N. Froissart, A. Buzelé, R. Perlat, A. Boutboul, D. Fremeaux-Bacchi, V. Isnard, S. Bienvenu, B. Medicine (Baltimore) 3600 Complement system is a part of innate immunity, its main function is to protect human from bacterial infection. As genetic disorders, complement deficiencies are often diagnosed in pediatric population. However, complement deficiencies can also be revealed in adults but have been poorly investigated. Herein, we describe a case series of infections revealing complement deficiency in adults to study clinical spectrum and management of complement deficiencies. A nationwide retrospective study was conducted in French university and general hospitals in departments of internal medicine, infectious diseases enrolling patients older than 15 years old who had presented at least one infection leading to a complement deficiency diagnosis. Forty-one patients included between 2002 and 2015 in 19 different departments were enrolled in this study. The male-to-female ratio was 1.3 and the mean age at diagnosis was 28 ± 14 (15–67) years. The main clinical feature was Neisseria meningitidis meningitis 75% (n = 31/41) often involving rare serotype: Y (n = 9) and W 135 (n = 7). The main complement deficiency observed was the common final pathway deficiency 83% (n = 34/41). Half of the cohort displayed severe sepsis or septic shock at diagnosis (n = 22/41) but no patient died. No patient had family history of complement deficiency. The mean follow-up was 1.15 ± 1.95 (0.1–10) years. Half of the patients had already suffered from at least one infection before diagnosis of complement deficiency: meningitis (n = 13), pneumonia (n = 4), fulminans purpura (n = 1), or recurrent otitis (n = 1). Near one-third (n = 10/39) had received prophylactic antibiotics (cotrimoxazole or penicillin) after diagnosis of complement deficiency. The vaccination coverage rate, at the end of the follow-up, for N meningitidis, Streptococcus pneumonia, and Haemophilius influenzae were, respectively, 90% (n = 33/37), 47% (n = 17/36), and 35% (n = 14/34). This large study emphasizes that complement deficiencies can be revealed in adults by infectious episodes. Most of them were meningococcal infections revealing common final pathway deficiency. To avoid undiagnosis or late diagnosis, adult displaying first episode of N meningitidis infection should be tested for complement deficiency. Wolters Kluwer Health 2016-05-13 /pmc/articles/PMC4902496/ /pubmed/27175654 http://dx.doi.org/10.1097/MD.0000000000003548 Text en Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by-nc-nd/4.0 This is an open access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0, where it is permissible to download, share and reproduce the work in any medium, provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/4.0 |
spellingShingle | 3600 Audemard-Verger, A. Descloux, E. Ponard, D. Deroux, A. Fantin, B. Fieschi, C. John, M. Bouldouyre, A. Karkowsi, L. Moulis, G. Auvinet, H. Valla, F. Lechiche, C. Davido, B. Martinot, M. Biron, C. Lucht, F. Asseray, N. Froissart, A. Buzelé, R. Perlat, A. Boutboul, D. Fremeaux-Bacchi, V. Isnard, S. Bienvenu, B. Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title | Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title_full | Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title_fullStr | Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title_full_unstemmed | Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title_short | Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients |
title_sort | infections revealing complement deficiency in adults: a french nationwide study enrolling 41 patients |
topic | 3600 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902496/ https://www.ncbi.nlm.nih.gov/pubmed/27175654 http://dx.doi.org/10.1097/MD.0000000000003548 |
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