ENCAPSID Study : ENCApsulated Bacterial Infection and Primary, Secondary ImmunoDeficiency
ENCAPSID Study : ENCApsulated Bacterial Infection and Primary, Secondary ImmunoDeficiency
Background : The occurrence of Primary immune deficiencies (PID) is rare in adults. Antibody
 deficiencies were the first PID to be diagnosed in adulthood and are mainly represented by
 common variable immune deficiency. The main manifestation of these PID are encapsulated
 bacterial infection which used to be recurrent and/or invasive, lead to hospitalization and
 have high rates of morbidity and mortality. Diagnosis of PID in adulthood may be supported by
 six warning signs from the European Society of Immunodeficiencies (ESID). However, their
 guidelines do not comprehensively describe symptoms of PID, even for patients with
 infections. The guidelines recommend screening adults for PID after at least two severe
 bacterial infections. The aim of this study is to screen for PID adult admitted to our
 hospital for encapsulated infection without any predisposal factor.

 Material and methods :

 -  Monocentric study. Inclusion between September 218 and September 2021

 -  Inclusion criteria :

 1. Age 18 to 65 years old

 2. Invasive encapsulated infection (Streptococcus pneumoniae, Streptococcus pyogènes,
 Haemophilus influenzae, Neisseiria meningitidis or Neisseria gonorrhoeae )

 -  Exclusion criteria :

 1. Medical history of PID

 2. Medical history of Secondary immune deficiency (SID)

 3. Local-regional factor that could predispose them to infection

 4. Hospital-acquired infection

 -  PID screening included the following: complete blood count, blood smear, immunoglobulin
 (Ig) isotype (IgA,M, G) and IgG subclass levels, total hemolytic complement and
 complement fractions (C) 3 and 4, alternative complement pathway (AP50) in case of
 Neisseria meningitidis (NM) infection, quantitative immunophenotyping of T, B and
 natural killer cells, specific antibody response to diphtheria, tetanus and pneumococcal
 vaccine and HIV serology. The clinical and laboratory diagnostic criteria used to
 identify PID were based on guidelines from the ESID and the Pan-American Group for
 Immunodeficiency (PAGID)

 -  A consultation in the infectious Diseases Department or Internal Medicine will be
 scheduled the hospitalization 3 months later to include patients and perform PID
 screening.
Background : Primary immune deficiencies (PID) are characterized by a failure of the immune
 system that is not explained by any infectious, neoplastic, or iatrogenic causes. By 2017,
 more than 350 different inherited rare PID disorders had been described. The occurrence of
 PID is rare in adults, with a prevalence of approximately 6.7 per 100,000 inhabitants in
 France. Infections are the most common manifestations of PID .Theses Infections are usually
 recurrent and/or invasive, lead to hospitalization and have high rates of morbidity and
 mortality. Antibody deficiencies were the first PID to be diagnosed in adulthood and are
 mainly represented by common variable immune deficiency. The main manifestation of these PID
 are encapsulated bacterial infection. Diagnosis of PID in adulthood may be supported by six
 warning signs from the European Society of Immunodeficiencies (ESID). However, their
 guidelines do not comprehensively describe symptoms of PID, even for patients with
 infections. The guidelines recommend screening adults for PID after at least two severe
 bacterial infections. The aim of this study is to screen for PID adult admitted to our
 hospital for encapsulated infection without any predisposal factor.

 Material and methods :

 -  Monocentric study. Inclusion between September 218 and September 2021

 -  Every months, micriobiologists will transfer to infectious diseases doctors and internal
 medicine doctors the name of patients who had a sample (cerebral fluid, articular fluid,
 blood culture, urine culture...) positive for Streptococcus pneumoniae, Streptococcus
 pyogenes, Haemophilus influenzae, Neisseiria meningitidis or Neisseria gonorrhoeae.

 -  Doctors will look at their medical records to see if they are eligible.

 -  Inclusion criteria :

 1. Age 18 to 65 years old

 2. Invasive encapsulated infection (Streptococcus pneumoniae, Streptococcus pyogènes,
 Haemophilus influenzae, Neisseiria meningitidis or Neisseria gonorrhoeae )

 -  Exclusion criteria :

 1. Medical history of PID

 2. Medical history of Secondary immune deficiency (SID)

 3. Local-regional factor that could predispose them to infection

 4. Hospital-acquired infection

 If patients are eligible, a consultation in the infectious Diseases Department or
 Internal Médicine Department will be scheduled the hospitalization 3 months later
 to include patients and perform PID screening.

 -  PID screening included the following: complete blood count, blood smear, immunoglobulin
 (Ig) isotype (IgA,M, G) and IgG subclass levels, total hemolytic complement and
 complement fractions (C) 3 and 4, alternative complement pathway (AP50) in case of
 Neisseria meningitidis (NM) infection, quantitative immunophenotyping of T, B and
 natural killer cells, specific antibody response to diphtheria, tetanus and pneumococcal
 vaccine and HIV serology. The clinical and laboratory diagnostic criteria used to
 identify PID were based on guidelines from the ESID and the Pan-American Group for
 Immunodeficiency (PAGID)
Inclusion Criteria:

 -  Age :18 to 65 years old

 -  Admitted for an invasive encapsulated infection (Streptococcus pneumoniae,
 Streptococcus pyogènes, Haemophilus influenzae, Neisseiria meningitidis or Neisseria
 gonorrhoeae )

 Exclusion criteria :

 -  Medical history of PID

 -  Medical history of Secondary immune deficiency (SID)

 -  A local-regional factor that could predispose them to infection (chronic pulmonary
 failure, cystic fibrosis, base scull fracture....)

 -  An hospital-acquired infection