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

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift


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.


  • S. Jolles
  • S. Sánchez-Ramón
  • Isabella Quinti
  • P. Soler-Palacín
  • C. Agostini
  • B. Florkin
  • L. J. Couderc
  • N. Brodszki
  • A. Jones
  • Hilary Longhurst
  • Klaus Warnatz
  • F. Haerynck
  • A. Matucci
  • Dick E. de Vries
Enheter & grupper
Externa organisationer
  • University Hospital of Wales
  • Hospital Clinico San Carlos de Madrid
  • Sapienza University of Rome
  • Vall d'Hebron University Hospital
  • University of Padova
  • University of Liège Hospital
  • Versailles Saint-Quentin-en-Yvelines University
  • University College London
  • Queen Mary University
  • Albert-Ludwigs University Freiburg
  • Ghent University Hospital
  • University of Florence
  • Jeroen Bosch Hospital
  • Tilburg University
  • Skåne University Hospital

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Lungmedicin och allergi


Sidor (från-till)226-234
Antal sidor9
TidskriftClinical and Experimental Immunology
Utgåva nummer2
StatusPublished - 2017 nov 1
Peer review utfördJa