ArticleRespiratory research2019
Primary immunodeficiency-related bronchiectasis in adults: comparison with bronchiectasis of other etiologies in a French reference center.
Article in Respiratory research, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 31 citations in OpenAlex.
- Bronchiectasis in Inborn Errors of Immunity: Prevalence, Predictors, and Cardiopulmonary Complications in a Genetically Characterized Cohort.Journal of clinical immunology · 2026Observational
- Distinct pulmonary pathophysiological mechanisms in COPD, bronchiectasis, and primary immunodeficiency: a multi-omics investigation of disease heterogeneity via sputum and blood profiling.Respiratory research · 2026Article
- Inhaled Corticosteroids and Risk ofJournal of clinical medicine · 2025Article
- Infections, autoimmunity and immunodeficiencies are the leading etiologies of non-cystic fibrosis bronchiectasis in adults from the southwest of Colombia.Biomedica : revista del Instituto Nacional de Salud · 2024Article
- Primary immunodeficiency diseases of adults: a review of pulmonary complication imaging findings.European radiology · 2024Review
- A comprehensive review ofFrontiers in medicine · 2024Review
- Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry.Journal of clinical immunology · 2023Article
- How and when to manage respiratory infections out of hospital.European respiratory review : an official journal of the European Respiratory Society · 2022Review
- Predictive Factors for and Complications of Bronchiectasis in Common Variable Immunodeficiency Disorders.Journal of clinical immunology · 2022Observational
- Comparison of different sets of immunological tests to identify treatable immunodeficiencies in adult bronchiectasis patients.ERJ open research · 2022Article
- Clinical and Radiological Phenotypes and Endotypes.Seminars in respiratory and critical care medicine · 2021Article
- Variable immunodeficiency score upfront analytical link (VISUAL), a proposal for combined prognostic score at diagnosis of common variable immunodeficiency.Scientific reports · 2021Article
- Chronic Lung Disease in Primary Antibody Deficiency: Diagnosis and Management.Immunology and allergy clinics of North America · 2020Review
Corrections and comments
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Authors and funding
16 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundBronchiectasis is a heterogeneous disease depending on etiology. It represents the most frequent non-infectious pulmonary complication of primary immunodeficiencies (PID). We investigated whether bronchiectasis associated with PID had a distinct course in comparison to bronchiectasis of other causes.
methodsRetrospective single-center study of adult patients diagnosed with non-cystic fibrosis bronchiectasis with more than 5 years of follow-up and at least 4 pulmonary functional tests available at one year apart. They were divided into three groups: PID- related bronchiectasis, idiopathic/post infectious-related bronchiectasis and other causes of bronchiectasis. Respiratory functional data and clinical outcomes were compared.
resultsOf 329 patients with bronchiectasis diagnosed in Foch Hospital (Suresnes, France), 98 patients fulfilled the selected criteria (20 PID-related cases, 39 idiopathic or post-infectious cases, and 39 cases with other causes). Median time of follow-up was 9.5 years. Groups were similar concerning initial characteristics (female 70.4%, never smokers 59.2%, mild severity bronchiectasis according to the FACED score and median FEV1 at diagnosis 73.5% predicted values [Q1-Q3: 53.75-90.5]), except PID patients who were younger (median age of 51.5 vs 62 years, p = 0.02). Eighty-five percent of PID patients received immunoglobulin substitution (median trough level was measured at 10.5 g/dl [10;10.92]). Global median FEV1 annual decline was 25.03 ml/year [8.16;43.9] and 19.82 ml/year [16.08;48.02] in the PID patients group. Forty-five percent of patients had bacterial colonization, pneumoniae occurred in 56% of patients and median exacerbation annual rate was 0.8 [0.3-1.4]. Hemoptysis occurred in 31.6% of patients. Global mortality rate was 11.2%. We did not record any significant difference for all clinical and functional outcomes between patients with PID and other etiologies. The median decline in FEV1 was similar in the three groups.
conclusionsThe course of PID-related bronchiectasis was similar to bronchiectasis of other causes. Provided that patients receive immunoglobulin replacement, the course of PID-related bronchiectasis seems to be independent of the underlying immune disorder.
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