ArticleClinical and experimental immunology2017
Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group.
Article in Clinical and experimental immunology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 36 citations in OpenAlex.
- Pulmonary surveillance in inborn errors of immunity: current challenges and emerging approaches.Frontiers in immunology · 2026Review
- Pulmonary Manifestations of Inborn Errors of Immunity: Diagnostic and Therapeutic Insights.Life (Basel, Switzerland) · 2025Review
- Primary immunodeficiency diseases of adults: a review of pulmonary complication imaging findings.European radiology · 2024Review
- Subcutaneous immunoglobulin replacement therapy in patients with immunodeficiencies - impact of drug packaging and administration method on patient reported outcomes.BMC immunology · 2024Article
- Patients with indolent lymphomas are at high risk of infections: experience from a German outpatient clinic.BMC immunology · 2023Article
- Real-world experience with CLAIRYG® 50 mg/mL (intravenous immunoglobulin) in children under 12 years with primary immunodeficiency or immmune thrombocytopenia: a post-approval safety study.Frontiers in pediatrics · 2023Article
- Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022Article
- Monitoring patients with uncomplicated common variable immunodeficiency: a systematic review.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022Article
- Multiple Breath Washout for Early Assessment of Pulmonary Complications in Patients With Primary Antibody Deficiencies: An Observational Study in Pediatric Age.Frontiers in pediatrics · 2022Article
- Chronic Lung Disease in Primary Antibody Deficiency: Diagnosis and Management.Immunology and allergy clinics of North America · 2020Review
- Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry.Journal of clinical immunology · 2020Article
- The role of respiratory viruses in children with humoral immunodeficiency on immunoglobulin replacement therapy.Pediatric pulmonology · 2019Article
- Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management.Respiratory research · 2018Review
- The broad spectrum of lung diseases in primary antibody deficiencies.European respiratory review : an official journal of the European Respiratory Society · 2018Review
- Respiratory manifestations in LPS-responsive beige-like anchor (LRBA) protein-deficient patients.European journal of pediatrics · 2018Article
- The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation.Frontiers in immunology · 2018Review
Corrections and comments
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Authors and funding
14 authors at 14 institutions in 8 countries.
Funding
No grant is acknowledged in the PubMed record.
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 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.
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