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Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients.

A Audemard-Verger1, E Descloux, D Ponard

  • 1From the CHU de Caen, Department of Internal Medicine, Caen (AA-V, BB), Department of Infectious Diseases, Nouvelle Calédonie University Hospital, Nouvelle Calédonie (ED, MJ), Laboratory of Immunology, Grenoble University Hospital, Grenoble (DP, AD), Department of Internal Medicine, Grenoble University Hospital, Grenoble, Department of Internal Medicine, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris (BF), Department of Clinical Immunology, Saint-Louis University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris (CF, DB), Department of Internal Medicine, Robert Ballanger General Hospital, Aulnay sous Bois (MAB), Department of Internal Medicine, Hôpital d'Instruction des Armées, Metz (LK), Department of Internal Medicine, Toulouse University Hospital, UMR 1027 INSERM University of Toulouse; CIC 1436, Toulouse (GM), Department of Internal Medicine, Brest University Hospital, Brest (HA), Department of Paediatric Intensive Care Unit, Lyon University Hospital, Lyon (FV), Department of Infectious Diseases, Nîmes University Hospital, Nîmes (CL), Department of Infectious Diseases, Raymond Poincaré University Hospital, Garches (BD), Department of Medicine, Colmar General Hospital, Colmar (MM), Department of Infectious Diseases, Nantes University Hospital, Nantes (CB, NA), Department of Infectious Diseases, Saint Etienne University Hospital, Saint Etienne (FL), Department of Internal Medicine, Créteil Hospital, Créteil (AF), Department of Infectious Diseases, Tours University Hospital, Tour (RB), Department of Internal Medicine, Rennes University Hospital, Rennes (AP), Laboratory of Immunology, Team Dentritic Cells Physiology, Cochin Institute (SI); and Laboratory of Immunology, Européen Georges Pompidou University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris (VF-B), France.

Medicine
|May 14, 2016
PubMed
Summary
This summary is machine-generated.

Complement deficiencies, often seen in children, can manifest in adults through infections like meningococcal meningitis. Early testing for complement deficiency is crucial in adults experiencing their first N. meningitidis infection to prevent delayed diagnosis.

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Area of Science:

  • Immunology
  • Infectious Diseases
  • Genetics

Background:

  • The complement system is vital for innate immunity against bacterial infections.
  • Complement deficiencies are typically diagnosed in pediatric populations, with adult cases being under-investigated.
  • This study focuses on infections revealing complement deficiency in adults to understand clinical spectrum and management.

Purpose of the Study:

  • To investigate the clinical spectrum and management of complement deficiencies in adults.
  • To highlight the role of infections as indicators of complement deficiency in the adult population.
  • To emphasize the need for timely diagnosis and appropriate management strategies.

Main Methods:

  • A nationwide retrospective study of adult patients (older than 15) diagnosed with complement deficiency due to infection.
  • Data collected from French university and general hospitals between 2002 and 2015.
  • Analysis of clinical features, complement deficiency types, infection history, and management outcomes.

Main Results:

  • Forty-one adult patients were included, with a mean age of 28 years.
  • Neisseria meningitidis meningitis was the predominant infection (75%), often involving rare serotypes.
  • Common final pathway deficiency was the most frequent type (83%), and no patient died despite severe sepsis in half the cohort.

Conclusions:

  • Infectious episodes, particularly meningococcal infections, can reveal complement deficiencies in adults.
  • Common final pathway deficiency is frequently associated with these infections.
  • Testing adults with a first episode of N. meningitidis infection for complement deficiency is recommended to avoid underdiagnosis.