Advanced

Screening protocols to monitor respiratory status in primary immunodeficiency disease : findings from a European survey and subclinical infection working group

Jolles, S.; Sánchez-Ramón, S.; Quinti, Isabella; Soler-Palacín, P.; Agostini, C.; Florkin, B.; Couderc, L. J.; Brodszki, N. LU ; Jones, A. and Longhurst, Hilary, et al. (2017) In Clinical and Experimental Immunology 190(2). p.226-234
Abstract

Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1–12 months for adult patients and every 3–6 months for paediatric patients. Patients diagnosed with lung diseases were more... (More)

Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1–12 months for adult patients and every 3–6 months for paediatric patients. Patients diagnosed with lung diseases were more likely to receive pharmaceutical therapies and received a wider range of therapies than patients without lung disease. Variation existed between centres in the frequency with which some clinical and laboratory monitoring tests are performed, including exercise tests, laboratory testing for IgG subclass levels and specific antibodies, and lung function tests such as spirometry. Some tests were carried out more frequently in adults than in children, probably due to difficulties conducting these tests in younger children. The percentage of patients seen regularly by a chest physician, or who had microbiology tests performed following chest and sinus exacerbations, also varied widely between centres. Our survey revealed a great deal of variation across Europe in how frequently patients with PID visit the clinic and how frequently some monitoring tests are carried out. These results highlight the urgent need for consensus guidelines on how to monitor lung complications in PID patients.

(Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
antibody deficiency, lung disease, monitoring, primary immunodeficiency disease, subclinical infection
in
Clinical and Experimental Immunology
volume
190
issue
2
pages
9 pages
publisher
British Society for Immunology
external identifiers
  • scopus:85028303819
  • wos:000412554100007
ISSN
0009-9104
DOI
10.1111/cei.13012
language
English
LU publication?
yes
id
088130d1-9113-4205-bd0b-d44e377ccc70
date added to LUP
2017-10-10 15:22:03
date last changed
2018-01-16 13:22:43
@article{088130d1-9113-4205-bd0b-d44e377ccc70,
  abstract     = {<p>Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1–12 months for adult patients and every 3–6 months for paediatric patients. Patients diagnosed with lung diseases were more likely to receive pharmaceutical therapies and received a wider range of therapies than patients without lung disease. Variation existed between centres in the frequency with which some clinical and laboratory monitoring tests are performed, including exercise tests, laboratory testing for IgG subclass levels and specific antibodies, and lung function tests such as spirometry. Some tests were carried out more frequently in adults than in children, probably due to difficulties conducting these tests in younger children. The percentage of patients seen regularly by a chest physician, or who had microbiology tests performed following chest and sinus exacerbations, also varied widely between centres. Our survey revealed a great deal of variation across Europe in how frequently patients with PID visit the clinic and how frequently some monitoring tests are carried out. These results highlight the urgent need for consensus guidelines on how to monitor lung complications in PID patients.</p>},
  author       = {Jolles, S. and Sánchez-Ramón, S. and Quinti, Isabella and Soler-Palacín, P. and Agostini, C. and Florkin, B. and Couderc, L. J. and Brodszki, N. and Jones, A. and Longhurst, Hilary and Warnatz, Klaus and Haerynck, F. and Matucci, A. and de Vries, Dick E.},
  issn         = {0009-9104},
  keyword      = {antibody deficiency,lung disease,monitoring,primary immunodeficiency disease,subclinical infection},
  language     = {eng},
  month        = {11},
  number       = {2},
  pages        = {226--234},
  publisher    = {British Society for Immunology},
  series       = {Clinical and Experimental Immunology},
  title        = {Screening protocols to monitor respiratory status in primary immunodeficiency disease : findings from a European survey and subclinical infection working group},
  url          = {http://dx.doi.org/10.1111/cei.13012},
  volume       = {190},
  year         = {2017},
}