The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

Phenotype, penetrance, and treatment of 133 cytotoxic T-lymphocyte antigen 4–insufficient subjects

(2018) Charlotte Schwab et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Prevalence and clinical challenges among adults with primary immunodeficiency and recombination-activating gene deficiency

(2018) Dylan Lawless et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Respiratory Infections and Antibiotic Usage in Common Variable Immunodeficiency

(2018) Johannes M. Sperlich et al.   Journal of Allergy and Clinical Immunology-In Practice

Measurement of Health-Related Quality of Life in Primary Antibody-Deficient Patients

(2017) Bahar Ataeinia et al.   IMMUNOLOGICAL INVESTIGATIONS

Erratum to: Low IgA and IgM is Associated with a Higher Prevalence of Bronchiectasis in Primary Antibody Deficiency

(2017) John P. Hodkinson et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Low IgA and IgM Is Associated with a Higher Prevalence of Bronchiectasis in Primary Antibody Deficiency

(2017) John P. Hodkinson et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Recurrent rhinovirus infections in a child with inherited MDA5 deficiency

(2017) Ian T. Lamborn et al.   JOURNAL OF EXPERIMENTAL MEDICINE

British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders

(2017) John R. Hurst et al.   Journal of Allergy and Clinical Immunology-In Practice

FDG PET-CT imaging of therapeutic response in granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID)

(2016) S. Jolles et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

The regulation of host defences to infection by the microbiota

(2016) Rebecca L. Brown et al.   IMMUNOLOGY

Abatacept alleviates severe autoimmune symptoms in a patient carrying a de novo variant in CTLA-4

(2016) Sangmoon Lee et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Defects of B-cell terminal differentiation in patients with type-1 Kabuki syndrome

(2016) Andrew W. Lindsley et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Quantitative Evidence of Wear-Off Effect at the End of the Intravenous IgG (IVIG) Dosing Cycle in Primary Immunodeficiency

(2016) Mikhail A. Rojavin et al.   JOURNAL OF CLINICAL IMMUNOLOGY

A nationwide survey of common viral infections in childhood among patients with primary immunodeficiency diseases

(2016) Etsuro Nanishi et al.   JOURNAL OF INFECTION

Genes associated with common variable immunodeficiency: one diagnosis to rule them all?

(2016) Delfien J A Bogaert et al.   JOURNAL OF MEDICAL GENETICS

Altered gut microbiota profile in common variable immunodeficiency associates with levels of lipopolysaccharide and markers of systemic immune activation

(2016) S F Jørgensen et al.   Mucosal Immunology

Cellular and humoral influenza-specific immune response upon vaccination in patients with common variable immunodeficiency and unclassified antibody deficiency

(2016) Leif G. Hanitsch et al.   VACCINE

Genetic Diagnosis Using Whole Exome Sequencing in Common Variable Immunodeficiency

(2016) Patrick Maffucci et al.   Frontiers in Immunology

Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency—histologic and immunohistochemical analyses of 16 cases

(2015) Nagarjun Rao et al.   HUMAN PATHOLOGY

Lung Magnetic Resonance Imaging with Diffusion Weighted Imaging Provides Regional Structural as well as Functional Information Without Radiation Exposure in Primary Antibody Deficiencies

(2015) Cinzia Milito et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Use of ibuprofen to assess inflammatory biomarkers in induced sputum: Implications for clinical trials in cystic fibrosis

(2015) James F. Chmiel et al.   Journal of Cystic Fibrosis

Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy

(2015) B. Lo et al.   SCIENCE

Lung structure-function correlation in patients with primary ciliary dyskinesia

(2015) M. Boon et al.   THORAX

European protocols for the diagnosis and initial treatment of interstitial lung disease in children

(2015) Andrew Bush et al.   THORAX

Lung disease in primary antibody deficiency

(2015) Nisha Verma et al.   Lancet Respiratory Medicine

Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings

(2015) Paul J. Maglione et al.   Journal of Allergy and Clinical Immunology-In Practice

Common variable immunodeficiency and autoimmunity – an inconvenient truth

(2014) Xiao Xiao et al.   AUTOIMMUNITY REVIEWS

Role of the Microbiota in Immunity and Inflammation

(2014) Yasmine Belkaid et al.   CELL

Subclinical infection and dosing in primary immunodeficiencies

(2014) S. Jolles   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Treatment of Persistent Rhinovirus Infection With Pegylated Interferon α2a and Ribavirin in Patients With Hypogammaglobulinemia

(2014) Olli Ruuskanen et al.   CLINICAL INFECTIOUS DISEASES

The efficacy of video-assisted thoracoscopic surgery lung biopsies in patients with interstitial lung disease: a retrospective study of 66 patients

(2014) Dominic Morris et al.   Journal of Cardiothoracic Surgery

Infliximab for Treatment of Granulomatous Disease in Patients with Common Variable Immunodeficiency

(2014) Timothy J. Franxman et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Quality of Life in Children with Primary Antibody Deficiency

(2014) P. Titman et al.   JOURNAL OF CLINICAL IMMUNOLOGY

CT Screening for Pulmonary Pathology in Common Variable Immunodeficiency Disorders and the Correlation with Clinical and Immunological Parameters

(2014) L. J. Maarschalk-Ellerbroek et al.   JOURNAL OF CLINICAL IMMUNOLOGY

ICON: The Early Diagnosis of Congenital Immunodeficiencies

(2014) John Routes et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Identification of Patients with RAG Mutations Previously Diagnosed with Common Variable Immunodeficiency Disorders

(2014) David Buchbinder et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: A double-blind placebo-controlled cross-over trial

(2014) Jochen G. Mainz et al.   Journal of Cystic Fibrosis

Autosomal dominant immune dysregulation syndrome in humans with CTLA4 mutations

(2014) Desirée Schubert et al.   NATURE MEDICINE

Lung clearance index in adults with non-cystic fibrosis bronchiectasis

(2014) Sherif Gonem et al.   RESPIRATORY RESEARCH

Immune dysregulation in human subjects with heterozygous germline mutations in CTLA4

(2014) H. S. Kuehn et al.   SCIENCE

Vaccine use in primary immunodeficiency disorders

(2014) Nicola Principi et al.   VACCINE

Sinonasal inhalation of tobramycin vibrating aerosol in cystic fibrosis patients with upper airway Pseudomonas aeruginosa colonization: results of a randomized, double-blind, placebo-controlled pilot study

(2014) Jochen G. Mainz et al.   Drug Design Development and Therapy

Outgrowth of the Bacterial Airway Microbiome after Rhinovirus Exacerbation of Chronic Obstructive Pulmonary Disease

(2013) Philip L. Molyneaux et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

The Role of the Lung Microbiome in Health and Disease. A National Heart, Lung, and Blood Institute Workshop Report

(2013) Yvonne J. Huang et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Common Variable Immunodeficiency

(2013) Jonathan S. Tam et al.   American Journal of Rhinology & Allergy

Evaluation of CD4+CD25+FOXP3+ regulatory T cells function in patients with common variable immunodeficiency

(2013) Nargess Arandi et al.   CELLULAR IMMUNOLOGY

New diagnostic criteria for Common Variable Immune Deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous Immunoglobulin

(2013) Rohan Ameratunga et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Virus shedding after human rhinovirus infection in children, adults and patients with hypogammaglobulinaemia

(2013) V. Peltola et al.   CLINICAL MICROBIOLOGY AND INFECTION

Model-Based Iterative Reconstruction Technique for Ultralow-Dose Chest CT

(2013) Masaki Katsura et al.   INVESTIGATIVE RADIOLOGY

Tertiary lymphoid neogenesis is a component of pulmonary lymphoid hyperplasia in patients with common variable immunodeficiency

(2013) Paul J. Maglione et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Respiratory viruses are associated with common respiratory pathogens in cystic fibrosis

(2013) Charles R. Esther et al.   PEDIATRIC PULMONOLOGY

Non-cystic fibrosis bronchiectasis

(2013) M. J. McDonnell et al.   QJM-AN INTERNATIONAL JOURNAL OF MEDICINE

Lung disease, T-cells and inflammation in common variable immunodeficiency disorders

(2013) Stina Gregersen et al.   SCANDINAVIAN JOURNAL OF CLINICAL & LABORATORY INVESTIGATION

Clinical measures of disease in adult non-CF bronchiectasis correlate with airway microbiota composition

(2013) Geraint B Rogers et al.   THORAX

The role of surgical lung biopsy in the management of interstitial lung disease: experience from a single institution in the UK

(2013) Vivienne Blackhall et al.   Interactive Cardiovascular and Thoracic Surgery

Deleterious Mutations in LRBA Are Associated with a Syndrome of Immune Deficiency and Autoimmunity

(2012) Gabriela Lopez-Herrera et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Short- and Long-Term Antibiotic Treatment Reduces Airway and Systemic Inflammation in Non–Cystic Fibrosis Bronchiectasis

(2012) James D. Chalmers et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Immune mechanisms and the impact of the disrupted lung microbiome in chronic bacterial lung infection and bronchiectasis

(2012) R. J. Boyton et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function

(2012) Hilario Nunes et al.   EUROPEAN RESPIRATORY JOURNAL

Use of Combination Chemotherapy for Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Patients with Common Variable Immunodeficiency (CVID)

(2012) Nicole M. Chase et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Immunoglobulin Responses at the Mucosal Interface

(2011) Andrea Cerutti et al.   Annual Review of Immunology

Morbidity and mortality in common variable immune deficiency over 4 decades

(2011) E. S. Resnick et al.   BLOOD

Airway and interstitial lung disease are distinct entities in paediatric common variable immunodeficiency

(2011) A. A. J. M. van de Ven et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Longitudinal decline in lung function in patients with primary immunoglobulin deficiencies

(2011) Yan Chen et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Protective effect of IgM against colonization of the respiratory tract by nontypeable Haemophilus influenzae in patients with hypogammaglobulinemia

(2011) Romain Micol et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Effectiveness of Immunoglobulin Replacement Therapy on Clinical Outcome in Patients with Primary Antibody Deficiencies: Results from a Multicenter Prospective Cohort Study

(2011) Isabella Quinti et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Transient TLR Activation Restores Inflammatory Response and Ability To Control Pulmonary Bacterial Infection in Germfree Mice

(2011) C. T. Fagundes et al.   JOURNAL OF IMMUNOLOGY

Microbiota regulates immune defense against respiratory tract influenza A virus infection

(2011) T. Ichinohe et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis

(2011) Fiona Kellett et al.   RESPIRATORY MEDICINE

Commensal Microflora Contribute to Host Defense Against Escherichia Coli Pneumonia Through Toll-Like Receptors

(2011) Lee-Wei Chen et al.   SHOCK

A CT Scan Score for the Assessment of Lung Disease in Children With Common Variable Immunodeficiency Disorders

(2010) Annick A.J.M. van de Ven et al.   CHEST

Clinical potential of pulsating aerosol for sinus drug delivery

(2010) Winfried Möller et al.   Expert Opinion on Drug Delivery

Infection outcomes in patients with common variable immunodeficiency disorders: Relationship to immunoglobulin therapy over 22 years

(2010) Mary Lucas et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Recurrent and persistent respiratory tract viral infections in patients with primary hypogammaglobulinemia

(2010) Leena Kainulainen et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

A Persistent and Diverse Airway Microbiota Present during Chronic Obstructive Pulmonary Disease Exacerbations

(2010) Yvonne J. Huang et al.   OMICS-A JOURNAL OF INTEGRATIVE BIOLOGY

Pulsating aerosols for drug delivery to the sinuses in healthy volunteers

(2010) Winfried Möller et al.   OTOLARYNGOLOGY-HEAD AND NECK SURGERY

Pathogenesis of bronchiectasis

(2010) Paul King   Paediatric Respiratory Reviews

Disordered Microbial Communities in Asthmatic Airways

(2010) Markus Hilty et al.   PLoS One

Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

(2010) R. Amin et al.   THORAX

Bronchiectasis

(2010) Cylen Javidan-Nejad et al.   Thoracic Surgery Clinics

The Natural History of Chronic Airflow Obstruction Revisited

(2009) Robab Kohansal et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Diagnostic Surgical Lung Biopsies for Suspected Interstitial Lung Diseases: A Retrospective Study

(2009) Martin I. Sigurdsson et al.   ANNALS OF THORACIC SURGERY

Current understanding and future directions

(2009) S. Jolles et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Regulatory T cell dysfunction in subjects with common variable immunodeficiency complicated by autoimmune disease

(2009) Grace P. Yu et al.   CLINICAL IMMUNOLOGY

Role of B cells in common variable immune deficiency

(2009) Sam Ahn et al.   Expert Review of Clinical Immunology

What are the consequences of the disappearing human microbiota?

(2009) Martin J. Blaser et al.   NATURE REVIEWS MICROBIOLOGY

Detection of pulmonary complications in common variable immunodeficiency

(2009) Catharina M. L. Touw et al.   PEDIATRIC ALLERGY AND IMMUNOLOGY

Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis

(2009) J G Mainz et al.   THORAX

Common variable immunodeficiency disorders: division into distinct clinical phenotypes

(2008) H. Chapel et al.   BLOOD

Memory B cells in common variable immunodeficiency: Clinical associations and sex differences

(2008) Silvia Sánchez-Ramón et al.   CLINICAL IMMUNOLOGY

Chromosomal radiosensitivity in patients with common variable immunodeficiency

(2008) Asghar Aghamohammadi et al.   IMMUNOBIOLOGY